Quiz Questions Flashcards

Question

Two weeks after a holiday in the Far East, a 30 year old lady presented with anorexia, fever and joint pains. Jaundice appeared a week later and on examination her liver and spleen were both enlarged and very tender. ``` A. Toxoplasmosis B. Syphilis C. Enteric fever D. CMV (cytomegalovirus) E. Tetanus F. Tuberculosis G. Giardiasis H. Malaria I. Herpes zoster (shingles) J. HIV K. Influenza L. Rabies M. Viral hepatitis N. Glandular fever O. Cholera P. Polio ```

Answer 1

M. Viral hepatitis - This is likely hepatitis A which is primarily transmitted via the faecal-oral route. After the virus is consumed and absorbed, it replicates in the liver and is excreted in the bile (to be re-transmitted). Transmission usually precedes symptoms by about 2 weeks and patients are non-infectious one week after onset of jaundice. The history can reveal risk factors such as living in an endemic area, contact with an infected person, homosexual sex or a known food-borne outbreak. This is classically, in EMQs, associated with shellfish which is harvested from sewage contaminated water. The clinical course of HAV consists of a pre-icteric phase, lasting 5-7 days, consisting characteristically of N&V, abdominal pain, fever, malaise and headache. Rarer symptoms may be present such as arthralgias and even severe thrombocytopenia and signs that may be found include splenomegaly, RUQ tenderness and tender hepatomegaly as well as bradycardia. The icteric phase is characterised by dark urine, pale stools, jaundice and pruritis. When jaundice comes on, the pre-icteric phase symptoms usually diminish, and jaundice typically peaks at 2 weeks. However, a fulminant course runs in

Answer 2

C. Enteric fever - Typhoid (or enteric fever) is a faecal-oral illness caused by Salmonella enterica, serotype S typhi, Salmonella enterica and S paratyphi. There are over 2500 serovars for S enterica. In this country, it is mainly from people who have returned from a country where it is endemic. The water supplies in Brazil may not treated and sanitary conditions may be dire which prompts transmission of this infection. Humans are the only known reservoir. The vaccine only offers moderate protection and does not protect against paratyphoid infection. This person has a fever which is a hallmark of infection. The fever of typhoid classically increases incrementally until a persistent fever with temperature 39-41 is established. There are also typically flu like symptoms after onset of fever and cough. Remember that characteristic findings such as bradycardia and rose spots may not be present and indeed rose spots may not be easy to spot in those with dark skin. Rose spots are blanching red lesions reported in 5-30% of cases usually occuring on the chest or abdomen. This patient needs antibiotics though the temperature will fall over about week. A third generation cephalosporin is indicated. If the sensitivity panel returns and shows that this organism is sensitive to all antibiotics then ciprofloxacin should be given.

Answer 3

F. HIV - The aim here is to establish the patient’s prognosis and begin the patient on HAART (highly active antiretroviral therapy). This patient needs to have a CD4 count, screen for HBV and HCV, VDRL, tuberculin skin test and a CXR. HIV viral load is also assessed at baseline. Generalised lymphadenopathy is common in HIV and present as painless enlarged nodes, usually in 2 or more non-contiguous sites, of >1cm for > 3months. Fevers and night sweats are also commonly seen, which may also indicate TB, which needs to be excluded (and malaria if from an endemic area). Unexplained diarrhoea of >1 month duration with no pathogen diagnosed is also seen in HIV. The oral candidiasis is also indicative of HIV and is an opportunitistic infection in an immunocompromised host. This patient has WHO stage 3 disease and would be placed in CDC category B, or symptomatic non-AIDS conditions. Oropharyngeal candidiasis is not AIDS indicating, but if this patient has candidiasis of the bronchi, trachea or lungs, or oesophagus then this would indicate an AIDS diagnosis (CDC category C – once the patient reaches category C, they stay in this category). I would not worry too much about clinical staging which is done according to the WHO or CDC criteria. Other category C conditions, which indicate AIDS, include CMV retinitis, Kaposi’s sarcoma, Mycobacterium avium complex/tuberculosis/other, PCP and toxoplasmosis. Key risk factors for HIV include needle sharing with IVDU, unprotected homo- and heterosexual intercourse (a higher risk if the receptive partner, particular with receptive anal intercourse), HIV infected blood transfusion, needle stick injury and high maternal viral load (mother to child transmission).

Answer 4

L. Syphilis - Syphilis is a common STI which is caused by the spirochete bacterium Treponema pallidum (subspecies pallidum). This history is textbook syphilis. People at high risk include those who have had sexual contact with an infected person, men who have sex with men, those with HIV and other STDs, those with multiple partners, commercial sex workers and those who use illicit drugs. The presentation is often asymptomatic but can manifest in many different ways. There is a ulcer of primary syphilis here noticed 6 weeks ago. Initial infection is local and leads to development of a macule. This develops into a papule which then ulcerates to form a chancre (typically appears 9 to 90 days after exposure, usually between 2-3 weeks) and may not be noticed by the patient (as it is painless). It typically develops in the anogenital area or cervix. There may also be (discrete, painless, rubbery) regional lymphadenopathy. This ulcer heals spontaneously. This patient has then gone on to develop secondary syphilis 6 weeks later (typically 4-8 weeks). Here there is disseminated treponemal infection which has multi-system manifestations and a diverse presentation. There are constitutional symptoms here as well as generalised lymphadenopathy. There may also, like this case, be this typical generalised symmetrical macular, papular or maculopapular diffuse rash which typically affects the palms of the hands and the plantar aspects of the feet. This rash may also occur on the trunk and scalp and may ulcerate. There may also be flesh-coloured wart-like lesions in the genital area which is known as condylomata lata. The treatment is with penicillin. Untreated syphilis facilitates the transmission of HIV and can cause considerable problems such as cardiovascular and neurological disease, as well as a congenital syndrome in the newborn. In some parts of the developing world, syphilis in pregnancy is a major cause of miscarriage and stillbirth. Pregnant women with syphilis are at risk of transmitting the infection across the placenta to the fetus.

Answer 5

M. Tetanus - This is very obvious tetanus which is caused by the exotoxin of the bacterium Clostridium tetani. This should have been prevented by appropriate management of the initial tetanus-prone wound and with complete active immunisation, with passive immunisation given when required. There is trismus here which has resulted in a grimace which is described as ‘risus sardonicus’ or sardonic smile. During a generalised tetanic spasm, the patient classically arches their back and extends their legs, flexes their arms in abduction and clenches their fists. Apnoea may also be a feature of these spasms. Intermittent tonic contractions of skeletal muscles often occurs which causes intensely painful spasms which may last for minutes – these are often triggered by stimuli such as noise, light and physical contact. Tetanic spasms can also produce opisthotonus, board like abdominal wall rigidity, dysphagia and apnoeic periods. Management of clinical tetanus involves supportive care (airway management is crucial here as spasms may compromise ventilation – without mechanical ventilation facilities such as in the third world, asphyxia is the most common cause of death due to muscle spasm), wound debridement, antimicrobials, passive and active immunisation, control of spasms and the management of autonomic dysfunction. Case fatality rate is 12-53%.

Answer 6

I. Cholera - This is a secretory diarrhoea caused by the bacterial organism Vibrio cholerae. This organism releases a toxin which stimulates adenylate cyclase. It is classically a disease of poverty but is also well described in returning travellers. Vomiting is a common early feature. The most striking and characteristic feature of cholera is the loose rice-water stools – a high volume diarrhoea which remains pale and loose, without blood. The presentation tends to be with litres of this rice-water stools. Culture of the organism provides a definitive diagnosis and rapid dipstick tests are currently available. Most patients will recover if the effects of the profound volume depletion are corrected with either oral or IV rehydration. Antibiotics do shorten the duration and severity of disease but the rising rate of bacterial resistance is becoming a problem. Note that about 70-80% of those infected with Vibrio cholerae do remain asymptomatic. What is interesting is that blood group O appears to lead to more severe disease, but may be protective against initial infection. Many infectious diseases indeed do show a relationship between blood group and disease susceptibility

Answer 7

O. Rabies - This 20 year old man has rabies and is going to die. Symptomatic rabies is 100% fatal and there is no curative medical therapy. Palliative care is needed in this situation and given that spasms in rabies (hydrophobia and aerophobia) are very much stimulus driven, the recommendation is seclusion, room darkening and restraint. Rabies is one horrible way to die. Current recommendations are also to use haloperidol. (Prog Palliat Care. 2006;14:62-67.). Opioid analgesics may also be of use here as well as anticonvulsants for seizures and neuromuscular blockers. If interested, you can look up the Milwaukee protocol, the use of which has led to only 6 patients who have been known to survive symptomatic rabies without receiving the vaccine. This involves putting the patient into a drug induced coma and then giving them antiviral drugs

Answer 8

A. Giardiasis - Giardiasis (caused by Giardia lamblia) is an intestinal infection with a flagellated protozoan parasite which is transmitted faecal-orally (transmission is by ingestion of water or food contaminated with cysts, and by person to person spread). Symptoms vary from the asymptomatic passage of cysts to acute self limiting diarrhoea or chronic diarrhoea. Presenting symptoms aside from diarrhoea include abdominal bloating with cramps, frequent belching with a sulphuric smell, nausea, anorexia and fatigue. The diagnosis is made on the detection of cysts or trophozoites (the two morphological forms of Giardia which exist) in a stool sample. The first line treatment is with metronidazole or tinidazole.

Answer 9

B. Polio - Poliovirus infection is usually asymptomatic and when symptomatic the most common presentation is with a minor GI illness. There is no cure for poliovirus infection and treatment is primarily supportive. This patient has acute flaccid paralysis (AFP), or paralytic poliomyelitis, which is the hallmark of major illness. This can rarely progress to bulbar paralysis and respiratory compromise. Paralytic poliomyelitis presents with decreased tone and motor function, as well as reduced tendon reflexes and muscle atrophy of the affected limb. Lack of vaccination is a strong risk factor. Remember that there are two main types of polio vaccine – Sabin, which is the oral weakened strain in disease endemic regions, and Salk, which is inactivated poliovirus in the rest of the world.

Answer 10

1. Chlamydia - This is genital tract chlamydia infection which is one of the most common STDs in the world. Remember that many infected individuals are asymptomatic. Women may present with cervical inflammation or yellow, cloudy discharge from the cervical os. A friable cervix is often also found on examination – the cervix bleeds easily with friction from a Dacron swab.

Answer 11

4. Varicella zoster - This is VZV infection (chickenpox) which typically presents with a fever, malaise and a widespread vesicular and pruritic rash which primarily affects the torso and face. Most countries in Europe do not immunise children against varicella.

Answer 12

1. Cytomegalovirus - This is a presentation of CMV retinitis, which is the most common manifestation of CMV disease in AIDS, the second most common being colitis. However do remember that virtually any organ can be affected by CMV and it can cause a range of conditions from encephalitis to pneumonitis. In CMV retinitis, fundoscopy will reveal areas of infarction, haemorrhage, perivascular sheathing and retinal opacification. These findings here are of chorioretinitis.

Answer 13

1. Dengue fever - Dengue in endemic in over 100 countries, especially SE Asia, Western Pacific and the Americas. It is an arbovirus which is transmitted by the Aedes aegypti mosquito found in the tropical and subtropical parts of the world. Clinical features include fever, headache, myalgia/arthralgia, skin flush and leucopenia, thrombocytopenia and elevated LFTs. Viral antigen or nucleic acid detection and serology are confirmatory tests to perform.

Answer 14

4. Kaposi sarcoma - This is Kaposi sarcoma which is a low-grade neoplasm caused by human herpesvirus-8 (or KSHV). It is associated with the acquired immunodeficiency associated with HIV infection. Oral KS can frequently affect the hard palate, gums and dorsum of the tongue. Cutaneous lesions are purple in colour and usually painless and non-pruritic. Histopathology of these vascular lesions will show characteristic atypical spindle-shape cells.

Answer 15

3. Oral hairy leukoplakia - This is oral hairy leukoplakia which presents as a painless white plaque found along the lateral tongue borders. There is history here which suggests immunosuppression. In situ hybridisation here has demonstrated the presence of EBV in the tissue.

Answer 16

3. Molluscum contagiosum - This is molluscum contagiosum which is sexually transmitted in adulthood. Lesions appear as the umbilicated pearly and smooth papules mentioned. About a third of patients will also develop symptoms of local redness, swelling or pruritis. Adults should be treated for this STD.

Answer 17

4. Syphilis - The chance of primary syphilis is usually a solitary, painless and clean ulcer with an indurated base. This is a common sexually transmitted infection caused by the spirochete Treponema pallidum. Treatment is with penicillin – untreated infection can cause considerable morbidity and facilitates HIV transmission.

Answer 18

4. Psittacosis - Chlamydia psittaci causes a community-acquired atypical pneumonia. It is often acquired from domesticated or commercially raised birds or exotic imported birds. The presentation can be similar to Mycoplasma and Chlamydophila pneumoniae. Tetracyclines are the preferred treatment. Hepatomegaly can occur in this condition with pain on palpation but is uncommon, as can splenomegaly. Both organs, if enlarged, are diffusely so.

Answer 19

4. Haemolytic uraemic syndrome - This is haemolytic uraemic syndrome which is characterised by MAHA, thrombocytopenia and nephropathy. Most cases are in children and related to gastroenteritis caused by verotoxin producing E. coli (O157:H7). The presence of schistocytes establishes the presence of thrombotic microangiopathy. The anaemia, thrombocytopenia and renal dysfunction characterised by creatinine rise mark this diagnosis.

Answer 20

1. Rabies - This is rabies, which can be difficult to diagnose if there is the absence of identifiable rabies exposure. There is non-specific prodrome of fever, chills, malaise, sore throat, vomiting, headaches and paraesthesias. The most specific signs of the disease are displayed here with hydrophobia and aerophobia (elicited by the placement of nasal cannulae). This patient is going to die and palliative care is needed. Rabies is considered 100% fatal.

Answer 21

1. Streptococcus pyogenes - This girl has rheumatic fever which is caused by an autoimmune process following infection with group A streptococci. The 5 major manifestations of acute rheumatic fever is something you need to be aware of. They are carditis, polyarthritis, chorea, erythema marginatum and SC nodules. Various other signs can also be seen such as spooning sign and pronator sign.

Answer 22

This is caused by a fluke which is acquired through exposure of the skin to contaminated freshwater (it is a snail-borne parasite). Patients can present acutely with bloody diarrhoea and hepatosplenomegaly is a common finding too. Diagnosis is made on microscopic visualisation of eggs in stools or urine. The preferred treatment for schistosome infections is with a drug called praziquantel.

Answer 23

1. Tetanus - This is caused by the exotoxin of Clostridium tetani and there is trismus here (lock jaw) which has resulted in risus sardonicus. The intermittent tonic contractions are also characteristic and the spasms can be triggered by both external and internal stimuli.

Answer 24

F. Neisseria meningitidis - This patient has meningitis. Schools and universities are common sites of outbreaks due to crowding. Commonly there will be a headache, fever and nuchal rigidity. There may also be an altered mental status, confusion, photophobia and vomiting. Kernig’s sign is uncommon but is positive when attempts to extend the leg are met with resistance when the patient is supine with the thigh flexed to 90 degrees. Another uncommon sign is Brudzinski’s sign and a petechial/purpuric rash, typically associated with meningococcal meningitis. CT head should be considered before LP if there is any evidence of raised ICP. An LP will confirm the diagnosis with bacterial meningitis showing a low CSF glucose, elevated CSF protein and positive CSF culture/gram stain or meningococcal antigen.

Answer 25

G. Mycobacterium tuberculosis - This patient has had pulmonary TB in Ethiopa but has not undergone a full course of anti-TB treatment. Subsequently, she has now developed extrapulmonary involvement. There is skeletal TB here with pain of the lower back. The onset of pain is usually gradual occuring over weeks to months and diagnosis is therefore frequently delayed. Local swelling and limitation of movement may be present, along with cold abscesses which may form if this is untreated. They are called cold abscesses as they are neither tender nor erythematous. These are also more common in HIV positive patients. MRI or a CT scan of the involved area is indicated here. Vertebral disease usually starts in the subchondral cancellous bone where it then spreads into the cortex and on to the disc. Destruction is more extensive on the ventral aspect than the dorsal aspect which leads to anterior wedging. As a result patients may present with kyphosis. There may also still be abnormalities on CXR consistent with TB. Note that normally TB initial therapy is a 4 drug regimen of isoniazid, rifampicin, pyrazinamide and ethambutol lasting for at least 6 months.

Answer 26

H. Campylobacter jejuni - This patient has Guillain-Barre syndrome. This condition is a demyelinating polyneuropathy. Classic neurology is a progressive symmetrical muscle weakness affecting lower extremities before upper extremities, and proximal muscles before distal muscles, accompanied by paraesthesias in the hands and feet which often precedes onset of weakness. The paralysis is typically flaccid with areflexia and progresses acutely over days, with an ensuing plateau phase followed by recovery. Two thirds of patients have a history of either prior influenza-like illness or gastroenteritis. This patient gives a history of gastroenteritis, the cause of which is likely Campylobacter. Studies have shown that 60-70% of acute cases are preceded by Campylobacter jejuni infection. Additionally, Campylobacter-associated GBS appears to have a worse prognosis with slower recovery and higher residual neurological disability. A study in Sweden has shown that the risk of developing GBS after Campylobacter jejuni infection is roughly 100 fold higher than after other infections. Other weak risks include immunisation, cancer and lymphoma, older age, HIV infection and male gender. Up to 30% will develop respiratory muscle weakness requiring ventilation so spirometry should be carried out at 6 hour intervals initially (and may show reduced vital capacity). AST and ALT may be elevated though the cause is unclear. LP is useful and the classic finding is of elevated CSF protein with normal cell count (known as albuminocytological dissociation). Treatment is with supportive and disease modifying treatment (plasma exchange or high dose Ig).

Answer 27

D. Legionella pneumophila - Legionella is a gram negative rod. Legionella infecting the lungs is legionnaires’ disease or Legionella pneumonia whereas non-lung infection is known as Pontiac fever. This bacteria is found in aqueous environments such as lakes and almost all cases are from contaminated water systems, which relates to the risk factors of getting Legionella (this patient is a plumber). Smoking is also a risk factor. It can cause confusion as well as hyponatraemia, abdominal pain, diarrhoea and bradycardia. Legionella does not grow on routine culture media and diagnosis relies on urine antigen detection (hence the request for a urine sample), serology or culture on special media.

Answer 28

A. Pseudomonas aeroginosa - The green pigment here is pyoverdine which is produced by Pseudomonas. Pseudomonas has virulence factors for lung colonisation and is noted for its type III injection apparatus, which you may remember from your microbiology lectures. It is a gram negative bacilli causing hospital acquired pneumonia and UTI. It particularly affects immunocompromised hosts such as those on chemotherapy, those with cystic fibrosis (this patient), burns and wounds. Biofilms are antibiotic resistant. Pseudomonas is also a special organism which is not sensitive to many antibiotics originally used for gram negatives. You need to prescribe a drug here which has specific anti-pseudomonal activity such as ceftazidime (a third generation cephalosporin), tazocin, ciprofloxacin, imipenem or gentamicin (usually used with one of the others). This organism acquires resistance genes very quickly so two antibiotics are given. It is, as mentioned, inherently resistant to many drug classes. Some are even resistant to all antibiotics.

Answer 29

G. FBC - Pallor and exertional SOB are suggestive of anaemia here. A FBC should be done here which would expect to find a low Hb. The World Health Organisation defines anaemia as

Answer 30

C. LP - This patient has meningitis. Universities are common sites of outbreaks due to crowding. Commonly there will be a headache, fever and nuchal rigidity. There may also be an altered mental status, confusion, photophobia and vomiting. An LP will confirm the diagnosis with bacterial meningitis showing a low CSF glucose, elevated CSF protein and positive CSF culture/gram stain or meningococcal antigen. A CT head scan should be considered before LP if there is reason to suspect raised ICP such as focal neurology, seizures, papilloedema on fundoscopy or altered mental state. The seizure here would be an indication to do a CT scan before attempting LP.

Answer 31

D. CXR and sputum sample - This sounds like pulmonary TB although it could well also be bronchial carcinoma. Remember that adenocarcinomas are usually located peripherally in the lung and are more common in non-smokers although most cases are still associated with smoking. Which ever the diagnosis is, a CXR will be useful with a sputum sample being obtained to check for TB. This patient should be placed in isolation due to suspected TB and 3 sputum samples cultured for AFB being the gold standard for diagnosis. Culture takes several weeks so sputum smears will be done before culture results are known. Interferon-gamma release assays (IGRAs) are now used by some hospitals to rapidly determine a patient’s TB status. All patients who have TB should be tested for HIV within 2 months of diagnosis.

Answer 32

I. Wound swab and culture - This is a straightforward question. A wound swab and culture is needed to see what the infection is. This will guide treatment.

Answer 33

L. CT head - This patient obviously has meningitis. A CT head scan needs to be done here before a diagnostic LP as there is reason to suspect raised ICP with bilateral papilloedema on fundoscopy. This can exclude a brain abscess or generalised cerebral oedema. Meningitis associated complications may also be identified such as hydrocephalus and brain infarction.

Answer 34

F. Streptococcus pneumoniae - Classic lobar pneumonia with no signs and symptoms to suggest an atypical organism is most likely to due to pneumococcus. Streptococcus pneumoniae is, as mentioned in the question, an alpha haemolytic Gram positive cocci (also Streptococcus viridans but it does not present in this way). A CXR is the most specific and sensitive test available and antibiotics are indicated. The rusty coloured sputum is hinting at a pneumococcal pneumonia too.The patient has presented with common symptoms of fever and a productive cough. There is additionally pleuritic chest pain. Initial treatment of a CAP is empirical with antibiotics. Often diagnosis is made solely on history and examination findings. Management is guided by the patient’s CURB-65 score.

Answer 35

True - It is the shortest cranial nerve and enters the skull at the cribriform plate, then becoming the olfactory tract.

Answer 36

True - Pituitary tumours and craniopharyngiomas can compress the optic chiasm, sparing the outer fibres responsible for the nasal field. This causes the characteristic bitemporal hemianopia.

Answer 37

True - Because the actions of the trochlear and abducens nerve are unopposed now.

Answer 38

False - The facial nerve carries taste sensation and the trigeminal carries touch/pain/temperature sensation from the anterior two-thirds of the tongue.

Answer 39

False - It ABducts the eye.

Answer 40

False - Muscles of mastication (masseter, temporalis, medial pterygoid, lateral pterygoid) are supplied by the mandibular branch of the trigeminal nerve.

Answer 41

True. The vestibular part of the nerve transmits positional and rotational information from the semicircular canals, saccule and utricle.

Answer 42

False - It is the glossopharyngeal nerve.

Answer 43

False - The parasympathetic system generally induces gastrointestinal motility and secretions. Acetylcholine stimulates HCl production so cutting the vagus will reduce this.

Answer 44

True - It supplies trapezius, which is the muscle responsible for shrugging shoulders!

Answer 45

False - The tongue will deviate towards the weaker side.

Answer 46

False - It is really UMN sign.

Answer 47

A. Extradural haemorrhage - Here we have the ‘lucid interval’ classically associated with an extradural haematoma. There is blood buildup this time between the dura mater and the skull. Compressive signs may also be present such as the down and out pupil due to CN III compression. The bleed here is usually from arteries, under high pressure, causing raised intracranial pressure. In this case, there is a chance the brain stem has been compressed causing his LOC. The bleed here is possibly from the anterior branch of the middle meningeal artery which runs beneath the pterion and is vulnerable at this point where the skull is thin. Remember that the pterion marks the junction of four bones: the parietal bone, temporal bone, sphenoid bone and frontal bone.

Answer 48

F. Tension headache - A tension headache is commonly triggered by stress and mental tension (also, fatigure and missing meals), hence the name. It is more common in females and those in middle age, and there is a link with lower socioeconomic status, although this does not necessarily represent causation. Symptoms include a dull, non-pulsatile and constricting bilateral pain, which is often described as a band across the patient’s head. It is not severe or disabling but classically worsens as the day progresses. This headache normally responds well to simple analgesics.

Answer 49

E. Migranes - Migraine is a chronic condition, with genetic determinants, which usually presents in early to mid life. The typical migraine aura this patient describes (which can be visual, sensory or speech symptoms) which can occur during or before the headache, is pathognomic, but is not seen in the majority of patients. The aura can be positive phenomena (for example this patient seeing flashing lights) or negative phenomena (for example visual loss). Nausea, photophobia and disability (the headache gets in the way with the patient’s ability to function) accompanying a headache also suggest a migraine diagnosis. The headache of a migraine tends to be prolonged if untreated, and tends to be unilateral and pounding (but does not have to be). Tests aim to rule out other differentials, although if the history is compatible and neurological examination is unremarkable, further testing is not needed.

Answer 50

B. Cluster headache - Cluster headache is characterised by attacks of severe pain localised to the unilateral orbital, supraorbital and/or temporal areas which lasts from 15 minutes to 3 hours, and occurs with a frequency ranging from once every other day to 8 times a day. These attacks can occur at the same time period of many weeks (known as the cluster period) accompanied by ipsilateral autonomic signs. The cause is hypothalamic activation with secondary trigeminal and autonomic activation (for instance, lacrimation, rhinorrhoea, nasal congestion, conjunctival injection and partial Horner’s i.e. ptosis and miosis). Cluster period attacks can be triggered by things like alcohol. Greater occipital nerve blockade often provides immediate relief until preventative medications take effect.

Answer 51

C. Trigeminal neuralgia - Trigeminal neuralgia occurs as episodes of severe unilateral pain in the distribution of CNV, usually lasting seconds, with no pain occuring between these episodes. Examination is often unremarkable. The pain is described as sharp, intense, stabbing or burning. It can be triggered commonly by actions such as eating, tooth brushing, cold and touch. Shaving and eating seem to be common in EMQs. Most people are asymptomatic between attacks although the severity of the pain makes these patients live in constant fear. TN is more common in MS and incidence increases with age. Post-herpetic TN is also possible. The mainstay of treatment is medical, with antiepileptics such as carbamazepine (which is the only medicine which is proven in RCTs and is therefore typically first line). If medical treatment fails, surgical options do exist such as microvascular decompression.

Answer 52

D. Subarachnoid haemorrhage - SAH (bleeding into the subarachnoid space) presents with sudden severe headache patients will often describe as the worst headache of their life, and can often be so bad that they feel like they’ve been kicked in the back of the back. Half of all patients lose consciousness and eye pain with exposure to light can also be seen. Altered mental status is common. SAH occurs most commonly in the 50-55 age group and affects women and black people more than men and white people. The most common cause of non-traumatic SAH is an aneurysm which ruptures. Conditions which predispose to aneurysm formation and SAH include adult PKD, Marfan’s, NF1 and Ehlers-Danlos. Cerebral aneurysms arise around the circle of Willis. A CT scan is indicated, and if unrevealing, this should be followed by an LP. Cerebral angiography can confirm the presence of aneurysms. The patient should be stabilised and this followed by surgical clipping or endovascular coil embolisation, the choice is subject to much current controversy sparked by relatively recent research. Complications can commonly occur and include rebleeding, hydrocephalus and vasospasm.

Answer 53

C. Stokes-Adams attack - Stokes-Adams attacks are episodes of transient LOC due to sudden decreased cardiac output. The previous heart attacks and later palpitations towards an arrhythmia such as heart bock, which caused the attack. Pallor prior to the attack and facial flushing due to reactive hyperemia after the attack is characteristic of a Stokes-Adams attack. Definitive treatment is with surgical insertion of a pacemaker.

Answer 54

A. Hypoglycaemia - In DM, hypoglycaemia is usually secondary to insulin or oral hypoglycaemics. Symptoms of hypoglycaemia are present when glucose drops

Answer 55

B. Anaemia - This man is anaemic and as a result he has fainted. This is IDA from a UGI bleed. His faint can also be attributed to hypovolaemia from his blood loss, from a presumed peptic ulcer (which accounts for his abdominal pain and coffee ground vomit). Whilst he may also display postural hypotension due to his hypovolaemia, this is not what this question is looking for.

Answer 56

E. Postural hypotension - This patient has postural hypotension which is demonstrated by a fall of >20 in systolic blood pressure and >10 diastolic within 3 minutes of standing upright. It is a side effect of anti-hypertensives and is a common problem in the elderly. A good history should be enough to diagnose this.

Answer 57

D. Opioid overdose - Opiate OD signs include CNS depression, miosis and apnoea. Finding small constricted pupils in someone who is unconscious is highly indicative. Naloxone is indicated both therapeutically and diagnostically. If there is a response, then it is diagnostic. Another diagnosis should be sought if the patient is unresponsive. IV is the preferred route of administration although naloxone can be given IM or SC if IV access cannot be established. Ventilatory support is key with 100% oxygen. You can check out Toxbase for a full database on poisons and treatments.

Answer 58

D. Meningitis - This 20 year old student has meningitis. Meningitis can present with a host of symptoms and commonly presents with seizures (children who have seizures are more likely to be infected by Streptococcus pneumoniae and Haemophilus influenza than meningococcus). Universities are common sites of outbreaks due to crowding. Commonly there will be a headache, fever and nuchal rigidity. There may also be an altered mental status, confusion, photophobia and vomiting. Kernig’s sign is uncommon but is positive when attempts to extend the leg are met with resistance when the patient is supine with the thigh flexed to 90 degrees. Another uncommon sign is Brudzinski’s sign and a petechial/purpuric rash, typically associated with meningococcal meningitis. CT head should be considered before LP if there is any evidence of raised ICP. An LP will confirm the diagnosis with bacterial meningitis showing a low CSF glucose, elevated CSF protein and positive CSF culture/gram stain or meningococcal antigen.

Answer 59

I. Partial seizure - The feeling this woman felt in her stomach before (aura), the description of the arm, incontinence and feeling "worn out" afterwards make this a seizure. Seizures can happen in anyone but epilepsy is the tendency to have repeated, unprovoked seizures (but this is still the best answer option from the available list). Epileptic seizures can be split into focal (partial/localised) and generalised (involving both hemispheres). Generalised seizures can be further divided into abscence (which can be typical or atypical), myoclonic, clonic, tonic, tonic-clonic and atonic types. Be sure to exclude other causes, such as a space occupying lesion with a CT or MRI scan. There may also be tongue biting. Always think of "before, during and after" when taking the history. History taking is the most important aspect of diagnosis. Antiepileptic monotherapy is the preferred treatment.

Answer 60

A. Right sided stroke - Weakness on one side and the difficulty swallowing makes this likely to be a stroke. If you have a think about the motor pathways you will realise that this is a right sided stroke. It is important is perform a CT head to exclude a haemorrhagic aetiology and consider thrombolysis with tPA if within the 4.5 hour window and there are no contraindications. Thrombolysis is done with alteplase at 10% bolus, 90% infusion at a dose of 0.9 mg/kg. Presentation after the 4.5 hour window is managed with aspirin. The Bamford/Oxford Stroke Classification subtypes ischaemic stroke according to vascular territory of infarction. After initial management, stroke care involves the ethos of an MDT environment with rehabilitation.

Answer 61

B. Myasthenia gravis - This lady has myasthenia gravis, which is an autoimmune condition with antibodies affecting the NMJ, mostly the nAChR at the post-synaptic muscle membrane. Although some have antibodies against MuSK, and there are other proteins involved. MG is characterised by muscle weakness which increases with exercise (fatigue, unlike Lambert-Eaton myasthenic syndrome). Commonly, presentations include diplopia and drooping eyelids like this patient, and there may also be SOB, proximal limb weakness, facial paresis and oropharyngeal weakness. MG is associated with thymic hyperplasia in 70% or thymoma in 10%, and these associations can also crop up in EMQs. There will be elevated serum AChR receptor antibody titres or MuSK antibodies. Electrophysiology will demonstrate a decremental response on repetitive nerve stimulation. Treatment includes anticholinesterases (pyridostigmine, and immunotherapy. Patients may also require a thymectomy. Some 15-20% may experience a myasthenic crisis (which needs mechanical ventilation). Do you know what the Tensilon test is and why edrophonium is given in this test?

Answer 62

J. Parkinsons disease - Parkinson’s is characterised by a resting tremor, rigidity, bradykinesia and postural instability. The resting tremor occurs at 4-6 Hz at rest which dissipates with the use of limbs, with generally asymmetrical onset. There is often also cogwheeling, especially if there is a superimposed tremor. The patient may have other signs like a mask like face due to the loss of spontaneous facial movement, hypophonia and micrographia, and may walk around in a shuffling gait with a stooped posture. The diagnosis is clinical. Treatment is symptomatic in an MDT setting. Medical therapy includes MAO-B inhibitors and DA agonists, for example rasagiline and carbidopa/levodopa. There are other medical therapies depending on the specific symptoms the patient presents with.

Answer 63

K. Multiple Sclerosis - Multiple sclerosis is a demyelinating CNS condition which is characterised by 2 or more episodes of neurological dysfunction which are separated in both time and space. MS classically presents in white women aged 20-40 with temporary visual/sensory loss although any presentation can occur. MRI is a sensitive test but less specific than spinal MRI, however, spinal MRI is abnormal in fewer cases. Treatment aims at treating the attack, preventing future attacks and symptomatic treatment of problems like bladder dysfunction, pain and fatigue.

Answer 64

C. Transient Ischaemic Attack - A TIA is colloquially called a ‘mini stroke’ with symptoms typically lasting under an hour. Here there is a description of focal neurological deficit unilaterally in the form of amaurosis fugax. There may also be other signs like unilateral weakness or sensory loss, hemianopsia, aphasia and cranial nerve defects, to name but a few. An antiplatelet drug such as aspirin is effective secondary prevention if the patient is not already anticoagulated. The patient will be anticoagulated if they have a likely or known cardioembolic source such as AF. Clopidogrel is an alternative in those who do not tolerate aspirin.

Answer 65

A. Diabetic Neuropathy - Diabetic neuropathy has lead to the painless ulcer most likely developing over pressure points in the foot such as on the ball of the foot. This may have resulted from an object becoming lodged in the shoe and eroding through the skin with walking. This is why it is important for diabetics to check their feet regularly and to wear specialised footwear. Diabetic neuropathy is a microvascular complication of DM and is characterised by peripheral nerve dysfunction. Pain is a common complaint such as the burning sensation this patient describes. Patient’s may also describe the pain as prickling or sticking. Complications range from the painless neuropathic ulcer described, at areas of the foot where there is weight loading (particularly the metatarsal heads), to the Charcot foot with severe architectural destruction of the foot. Foot ulceration is a common precusor to amputation. Foot care is crucial in DM. Examination should include peripheral pulses, reflexes and sensation to light touch with a 10g monofilament, vibration (128Hz tuning fork), pinprick and proprioception. The pain may be treated with medications like pregabalin and gabapentin.

Answer 66

B. Carpal tunnel syndrome - Carpal tunnel syndrome is the most common nerve entrapment and women just past middle age are at the highest risk. Symptoms include numbness/tingling of the thumb and radial fingers, an aching wrist and clumsiness (especially with fine motor tasks). The symptoms are of gradual onset and often wake the patient up at night, and is relieved by shaking the wrist. Numbness is normally on the palmar aspect of the thumb, index and middle fingers (but not the little finger). When the patient wakes up, there may be difficulty flexing or extending fingers. Symptoms in the day tend to be associated with activity. The most sensitive and specific test for diagnosis is EMG and can confirm damage to the median nerve in the carpal tunnel and categorise the severity of the damage. There are specific tests for CTS such as Tinel’s test and Phalen’s test, though clinically these are not particularly useful due to sensitivity and specificity. CTS is caused by anything that causes a reduction in the size of the carpal tunnel – from inflammation, arthritis and tenosynovitis to old fractures. In CTS there is preserved sensation of the palm as the palmar cutaneous branch comes off a few cm above the carpal tunnel.

Answer 67

C. Charcot-Marie-Tooth disease - Charcot-Marie-Tooth disease comprises of a group of hereditary peripheral neuropathies with different genetic abnormalities, hence the FH here (most patients have a FHx). Pes cavus (high foot arches), hammer toes and distal atrophy of the hands and legs are characteristic (resulting in this inverted champagne bottle appearance). Pes cavus can be associated with areflexia. Patients may have to lift their legs up excessively to clear the toes. Rehabilitative and orthotic treatments can help to keep patients functional. There are no other known risk factors for this condition aside from FH. The risk of passing the condition on to the next generation varies depending on the subtype of CMT, which have different inheritance patterns. Most CMT1 and CMT2 are autosomal dominant and CMT 4 is autosomal recessive. There are also X-linked forms.

Answer 68

A. UTI - Confusion and a fever is not an uncommon presentation of UTI in the elderly where the presentation is often non-specific. The lower abdominal suprapubic pain here is another clue. 10 percent of women over the age of 70 have UTIs. Post-menopause (absence of oestrogen) is a strong risk factor for UTI in women. In women of this age group, sexual activity is less strongly associated with UTI. Treatment can be started on the symptoms here as well as dipstick result, but urine culture and sensitivity confirms the diagnosis and guides the appropriate use of antibiotics. A positive nitrite and leucocyte esterase in the urine indicates a likely UTI diagnosis. A midstream clean-catch urine specimen should be sent for culture (looking for uropathogenic organisms) if symptoms are atypical or do not respond to treatment. The AMTS is used to rapidly assess mental function and a score of 6 or less suggests either delirium or dementia (the former is acute decline).

Answer 69

G. Thiamine/B1 deficiency - This patient has Wernicke’s encephalopathy. Wernicke’s is due to acute thiamine deficiency, which is a problem in alcoholics (Dupytren’s contracture here is a clue to alcohol use). Others at risk include those with AIDS, cancer and treatment with chemotherapy, malnutrition and GIT surgery, especially bariatric procedures. It is a clinically under-diagnosed condition. The classic EMQ triad is of mental change, ophthalmoplegia and gait dysfunction, which is actually only seen in 10% of cases. In reality, the manifestations are varied and a high index of suspicion is needed. Despite there, the manifestiations typically include altered consciousness, gait disorders and eye movement abnormalities. This is an emergency and treatment is with parenteral replacement of thiamine. This avoids permanent neurological damage including later development of Korsakoff’s psychosis, which is irreversible. Note that thiamine should be given before dextrose! Magnesium deficiency also needs to be corrected as it is a co-factor in the functioning of thiamine dependent enzymes.

Answer 70

F. Hypoglycaemia - This patient has symptoms of hypoglycaemia, present when glucose drops

Answer 71

H. U&E imbalance - This patient has become confused and there is an acute change in mental status. Dehydration (volume depletion) can be a relatively common cause. (The most common causes of volume depletion are haemorrhage, vomiting, diarrhoea, diuresis or third space losses). FBC in this case may reveal a raised haematocrit and high Hb. Serum electrolyte analysis may reveal derangements such as hyper or hypokalaemia and hyponatraemia. Urine specific gravity can be expected to be high and serum urea to creatinine ratio >20. Examination of this dehydrated patient may reveal signs like dry mucosal membranes, postural hypotension, postural tachycardia and possibly even shock depending on severity. The patient may complain of symptoms such as thirst, tiredness, muscle cramps, pain in the chest and/or abdomen as well as confusion. The confusion may result from U&E abnormalities (the best option from this list) but is non-specific and may indeed reflect poor cerebral perfusion or uraemia in the setting of impaired renal function, which are equally if not more likely in this patient.

Answer 72

K. CVA - Atrial fibrillation is strongly implicated in the risk of cardioembolic stroke. Confusion is common, especially in the elderly who have had past strokes and in those with cognitive dysfunction. An extensor plantar response is known as Babinski’s sign and is a sign of an UMN lesion. The normal response is flexion where the big toe moves downwards. It is important is perform a CT head exclude a haemorrhagic aetiology and consider thrombolysis with tPA if within the 4.5 hour window and there are no contraindications. Thrombolysis is done with alteplase at 10% bolus, 90% infusion at a dose of 0.9 mg/kg. Presentation after the 4.5 hour window is managed with aspirin. The Bamford/Oxford Stroke Classification subtypes ischaemic stroke according to vascular territory of infarction. After initial management, stroke care involves the ethos of an MDT environment with rehabilitation.

Answer 73

E. Stroke - If you have a think about the motor pathways you will realise that this is a right sided stroke. Cardiac conditions are potential risks for cardioembolism. The previous MI could, for instance, lead to regional wall motion abnormalities or decreased LV ejection fraction which can be risks. Other cardiac conditions include valve disease, PFO, mitral prolapse, prosthetic heart valve and cardiomyopathy. Other major risk factors include AF, DM, smoking, hypertension and FH. It is important is perform a CT head exclude a haemorrhagic aetiology and consider thrombolysis with tPA if within the 4.5 hour window and there are no contraindications. Obviously this patient has presented to late for this but thrombolysis is usually done with alteplase at 10% bolus, 90% infusion at a dose of 0.9 mg/kg. Presentation after the 4.5 hour window will be managed with aspirin. The Bamford/Oxford Stroke Classification subtypes ischaemic stroke according to vascular territory of infarction. After initial management, stroke care involves the ethos of an MDT environment with rehabilitation. This is not a TIA which resolves within 24 hours of symptom onset, although in practice this is often difficult to distinguish due to interventional measures which are rapidly enacted.

Answer 74

J. Aortic Stenosis - Aortic stenosis can present with chest pain, dyspnoea and syncope. It is characterised by a harsh ejection systolic murmur heard loudest at the right upper sternal edge at end expiration, which radiates up towards the carotids. The pulse pressure is narrow and there may be an associated slow-rising and plateau pulse. Doppler echo is vital for diagnosis and shows a pressure gradient across the narrowed valve orifice. 20% of cases are due to a congenital bicuspid valve. The most common cause of aortic stenosis in adults is calcification of normal trileaflet valves. Clinically stable patients may be considered for surgical repair or TAVR.

Answer 75

B. Vasovagal syncope - This is vasovagal syncope which is also known as the ‘common faint’. It has many manifestations and many triggers such as emotional upset, unpleasant sights, fear and pain. These tend to recur and patients needs to be warned of this and that they are almost never life-threatening. The cause of the faint is a transient insufficiency of cerebral flow. LOC inevitably leads to loss of postural tone and the patient falling over.

Answer 76

D. Cardiac arrhythmias - Stokes-Adams attacks are episodes of transient LOC due to sudden decreased cardiac output. Pallor prior to the attack and facial flushing due to reactive hyperemia after the attack is characteristic of a Stokes-Adams attack. The underlying cause is a cardiac arrhythmia such as complete heart block.

Answer 77

F. Postural hypotension - Postural hypotension is a side effect of anti-hypertensives and is a common problem in the elderly. Diabetic autonomic neuropathy may also be a cause here. A good history should be enough to diagnose this.

Answer 78

D. Stroke - This is a left sided stroke which may have some frontal lobe involvement consistent with his personality changes. It is important is perform a CT head exclude a haemorrhagic aetiology and consider thrombolysis with tPA if within the 4.5 hour window and there are no contraindications. Thrombolysis is done with alteplase at 10% bolus, 90% infusion at a dose of 0.9 mg/kg. Presentation after the 4.5 hour window is managed with aspirin. The Bamford/Oxford Stroke Classification subtypes ischaemic stroke according to vascular territory of infarction. After initial management, stroke care involves the ethos of an MDT environment with rehabilitation.

Answer 79

F. Myasthenia gravis - Myasthenia gravis is an autoimmune condition with antibodies affecting the NMJ, mostly the nAChR at the post-synaptic muscle membrane. Although some have antibodies against MuSK, and there are other proteins involved. MG is characterised by muscle weakness which increases with exercise (fatigue, unlike Lambert-Eaton myasthenic syndrome). Commonly, presentations include diplopia and drooping eyelids like this patient, and there may also be SOB, proximal limb weakness, facial paresis and oropharyngeal weakness. MG is associated with thymic hyperplasia in 70% or thymoma in 10%, and these associations can also crop up in EMQs. There will be elevated serum AChR receptor antibody titres or MuSK antibodies. Electrophysiology will demonstrate a decremental response on repetitive nerve stimulation. Treatment includes anticholinesterases (pyridostigmine, and immunotherapy. Patients may also require a thymectomy. Some 15-20% may experience a myasthenic crisis (which needs mechanical ventilation). Do you know what the Tensilon test is and why edrophonium is given in this test?

Answer 80

B. Space occupying lesion - Intracranial space-occupying lesions are mostly caused by primary and metastatic tumours. The progressive headache associated with N&V, visual disturbances and focal neurology indicate a slowly growing space-occupying mass. This could be a brain tumour, which may be primary (originates within the cranium) or secondary (from a metastatic tumour found elsewhere). The signs of raised intracranial pressure are seen here, and include headache, altered mental status, nausear and/or vomiting and gait abnormality. Tumours may be benign or malignant and range from meningiomas and medulloblastomas to craniopharyngiomas and astrocytic brain tumours. Further investigation is warranted in this case.

Answer 81

J. Peripheral neuropathy - This is by definition peripheral neuropathy. This is a case of diabetic neuropathy with sensory and motor deficits. This is a microvascular complication of DM and is characterised by peripheral nerve dysfunction. Pain is a common complaint such as the burning and shooting sensation this patient describes. Patient’s may also describe the pain as prickling or sticking. Examination should include peripheral pulses, reflexes and sensation to light touch with a 10g monofilament, vibration (128Hz tuning fork), pinprick and proprioception. The pain may be treated with medications like pregabalin (VGCC modulator) and duloxetine (serotonin-NA re-uptake inhibitor).

Answer 82

I. Multiple sclerosis - Multiple sclerosis is a demyelinating CNS condition which is characterised by 2 or more episodes of neurological dysfunction which are separated in both time and space. MS classically presents in white women aged 20-40 with temporary visual/sensory loss although any presentation can occur. MRI is a sensitive test but less specific than spinal MRI, however, spinal MRI is abnormal in fewer cases. Treatment aims at treating the attack, preventing future attacks and symptomatic treatment of problems like bladder dysfunction, pain and fatigue.

Answer 83

6. Hyponatraemia - Hyponatraemia is defined by serum sodium

Answer 84

4. Encaphalitis - Encephalitis (brain parenchyma inflammation) is not to be confused with meningitis where the meninges is inflammed (although meningoencephalitis can exist). In encephalitis, there is an altered state of consciousness (this patient is confused) and may there also be focal neurology. There is also a fever (exceptions, however, are subacute sclerosing panencephalitis, VZV and HCV). Other typical features include headache and seizures. It is a medical emergency and people are the extremes of age are more at risk. The list of possible causes is massive. HSV encephalitis is characterised by temporal lobe changes. Hypodense lesions and mass effect can be seen in the temporal lobes on CT. An MRI is a better test for encephalitis although it is less available when compared to CT. If you suspect this diagnosis, empiral IV acyclovir should be started before the results of any investigations are known. A large proportion are caused by HSV and empirical therapy is backed up by RCTs which show improved mortality. You can change the treatment if the cause is known, for instance, ganciclovir for CMV. You should look for a rash which may give a clue to the aetiology. For example, vesicular patterns are seen in VZV, HSV and enteroviruses, EBV causes a maculopapular pattern after ampicillin, Lyme disease pathognomically gives erythema migrans whereas erythema nodosum might make you think of TB. The patient may also have animal/insect bites and a careful and thorough history is important.

Answer 85

7. Hypocalcaemia - Hypocalcaemia can develop as a complication of thyroid surgery due to the loss of parathyroid glands which produce PTH. This is the cause of this lady’s seizure. Trousseau’s sign can be seen which is carpal spasm when a blood pressure cuff is used for several minutes. Carpopedal spasm that occurs with hypocalcaemia is a painful spasm and could also be the presenting sign. Chvostek’s sign is twitching of the perioral muscles in response to tapping over the facial nerve at the ear. If urgent replacement is necessary, calcium gluconate can be given IV. It is preferred over calcium chloride as it causes less tissue necrosis if it leaks out. It is worth noting that digoxin may be ineffective until serum calcium is restored to normal.

Answer 86

10. Tonic-clonic seizures - This is a tonic-clonic, generalised seizure. It is characterised by LOC and widespread motor tonic contractions followed by clonic jerking movements. There will characteristically be a suppressed level of arousal following the event. This may either reflect a primary generalised episode or a focal seizure with secondary generalisation. The main aim of acute treatment is to terminate the seizure and to protect the airway. Management always starts with basic life-support (like every acute emergency) and your ABCs. IV access needs to be established (bloods sent to the lab too and serum glucose measured to test for reversable causes of seizure activity – thiamine should also be given to the patient if there is any concern about deficiency and hypoglycaemia, for instance in alcohol abuse). The following are needed: ECG, pulse oximetry, ABG. IV lorazepam is the preferred initial therapy, though rectal diazepam can be used if there is no IV access. If BZDs fail to stop the seizure then phenytoin or fosphenytoin can be tried. After the episode, MRI and EEG are essential in diagnosing an epilepsy syndrome. During the episode of generalised tonic-clonic activity, the EEG will show bilateral synchrony in the epileptiform activity. If this is a one-off seizure in which a provoking factor, such as electrolyte disturbance or hypoglycaemia, has been identified then there is no need for therapy for epilepsy. In unprovoked cases, this depends on history, examination, EEG and MRI. Treatment may not be needed the first time but after a second unprovoked instance, therapy is generally recommended.

Answer 87

1. Abscence seizures - A typical absence seizure is characterised by behavioural arrest or staring, lasting 5-10 seconds, interrupting otherwise normal activity. It can be induced by hyperventilation. Absence seizures can also be atypical, where it is less clear when it begins and ends and is not usually precipitated by hyperventilation. The definitive test here is to do an EEG to determine the exact nature of the seizure. This will ensure appropriate treatment. Most are medically responsive and childhood absence epilepsy (CAE) tends to remit by adulthood. First line treatment is with ethosuximide, valproate or lamotrigine. Atypical seizures though, tend to be refractory to medical treatment and associated with mental retardation.

Answer 88

B. Occipital stroke - This is cortical blindness, which is the loss of vision in a normal eye which results from damage to the visual area in the occipital cortex. The lack of insight that they have lost vision is a phenomenon known as Anton’s syndrome. They are ‘cortically blind’ but claim, often confabulate and adamantly despite obvious evidence, claim that they are capable of seeing. The pupillary reflex to light is intact as it does not involve the cortex and fundscopy, if done, would also be normal.

Answer 89

G. Parkinson's disease - Parkinson’s is characterised by a resting tremor, rigidity, bradykinesia and postural instability. This patient has difficulty walking, a resting tremor (4-6 Hz at rest which dissipates with the use of limbs, with generally asymmetrical onset) and limbs oppose movement. This last point demonstrates rigidity, which shows as resistance to passive movement about a joint. There is often also cogwheeling, especially if there is a superimposed tremor. The patient may have other signs like a mask like face due to the loss of spontaneous facial movement, hypophonia and micrographia, and may walk around in a shuffling gait with a stooped posture. The diagnosis is clinical. Treatment is symptomatic in an MDT setting. Medical therapy includes MAO-B inhibitors and DA agonists, for example rasagiline and carbidopa/levodopa. There are other medical therapies depending on the specific symptoms the patient presents with.

Answer 90

I. Alzheimers dementia - This is Alzheimer’s dementia which is a progressive irreversible disorder characterised by memory loss, loss of social function and dimished executive function. The MMSE is the most widely used screening test for cognitive function and a score

Answer 91

H. Picks disease - Pick’s disease (not to be confused with Niemann-Pick disease) is one cause of frontotemporal degeneration. If you are not aware of this then this question would have been difficult. In any case, you should have realised that this is frontotemporal dementia. This type of dementia presents primarily with disruption in personality and social conduct, or as a primary language disorder. Essentially, the patient will present with components of the dysexecutive syndrome. Almost 50% will also display parkinsonism and a subset also have MND. Treatment is supportive combining medications ranging from BZDs to antidepressants depending on symptoms, with community services and carer guidance.

Answer 92

E. Vascular dementia - Vascular dementia is a chronic and progressive dementia with loss of brain parenchyma mainly due to causes such as infarction and small vessel changes. It is classically assumed to be a stepwise progression in symptoms, although a gradual course can also be seen. This patient is an arteriopath. It is the second most common cause of dementia in older people and there is a large overlap with Alzheimer’s with many patients having a mixed form. Treatment is of limited use and the best course of action is to target vascular risk factors at as early a stage as possible.

Answer 93

1. Functional - Functional MRI's can highlight an area of the brain that is being used in certain tasks.

Answer 94

True - It is a medical emergency, treated with IV or PR diazepam initially.

Answer 95

2. Complex partial - The above are all features of a temporal lobe seizure, which is often complex but may be simple (ie. no impairment of consciousness).

Answer 96

2. Substantia nigra - The substantia nigra is the main area affected in Parkinson's disease.

Answer 97

3. Hemiplegia - Hemiplegia is seen in cerebrovascular disease or lesions affecting the motor cortex and pyramidal tracts.

Answer 98

False - Dopamine does not cross the blood-brain barrier. Therefore levodopa (L-dopa), its precursor is given, because this can cross the barrier. It is then processed to make dopamine.

Answer 99

False - MS only affects the CNS.

Answer 100

True - MS is characterised by inflammation and demyelination.

Answer 101

1. MRI - MRI scans of the brain or spine are the most useful imaging investigations.

Answer 102

2. Left anterior circulation stroke - Anterior circulation strokes are commonly associated with aphasia and loss of strength in the face and upper and/or lower extremities. The inability to raise the right arm points to a left sided lesion. Remember that UMNs innervate contralateral LMNs, which innervate ipsilateral muscles.

Answer 103

4. Wernicke's encephalopathy - Wernicke’s encephalopathy is caused by the acute deficiency of thiamine in a susceptible host. It is clinically underdiagnosed. The classic triad is of mental status changes, ophthalmoplegia and gait dysfunction, though this is present in only 10% of cases.

Answer 104

4. Absence seizure - This description is of a typical absence seizure which interrupts otherwise normal activity. This can be hyperventilation-induced. EEG is the definitive test and most cases are medically responsive.

Answer 105

2. Charcot-Marie-Tooth disease - This history here points to Charcot-Marie-Tooth disease. There is pes cavus and distal atrophy of the legs here which are characteristic features. The progressive history from childhood suggests this genetic condition as the cause.

Answer 106

2. Migrane - This is a classic migraine history with an aura of a tingling sensation, which is pathognomic of migraine. The pain tends to be described as throbbing or pulsing and tends to be disabling, and can be associated with N&V and photophobia.

Answer 107

1. Benign positional paroxysmal vertigo - The history of vertigo on rollong over in bed which lasts for a few seconds is suggestive here. Dix-Hallpike test would be diagnostic in this case and will demonstrate nystagmus and symptoms delayed by about 15s, peak in 20-30s and then decay with complete resolution of the episode of vertigo.

Answer 108

2. Subdural haematoma - There is a history of falls here and confusion. A subdural haematoma occurs due to extra-axial blood collection between the dura and arachnoid layers surrounding the brain. Surgical treatment will likely be necessary in this case with neurological deficit.

Answer 109

2. Poliovirus infection - This is paralytic poliovirus infection. The mother here only believes in herbal remedies so this child has not been immunised. The gastrointestinal prodrome is common although non-specific. Examination findings here are consistent. While GBS may be preceded by gastroenteritis, the limb weakness is usually symmetrical and there may be autonomic dysfunction. There is no cure for poliovirus infection.

Answer 110

1. Myasthenia gravis - MG is characterised by muscle weakness that increases with exercise and improves on rest. It is a chronic autoimmune disorder of the post-synaptic membrane at the NMJ in skeletal muscle.

Answer 111

4. Multiple sclerosis - MS is a demyelinating CNS condition clinically defined by 2 episodes of neurological dysfunction which are separated in space and time. Smoking is a weak risk factor.

Answer 112

1. Spinal stenosis - This is neurogenic claudication due to spinal stenosis. This is relieved by sitting or bending over, which widens the size of the spinal canal. This is the reason for the stooped posture when walking, which is classically described as the ‘shopping cart sign’ where patients lean forwards like onto a shopping trolley to flex the spine.

Answer 113

B. Exercise ECG - This patient has presented with stable angina. Resting ECG is often normal however during exercise stress ECG (most often the Bruce Protocol) there will be ST segment depression during exercise indicative of ischaemia. Those unable to exercise to an adequate level may need stress myocardial perfusion imaging or stress echocardiography. 1st line treatment involves lifestyle changes and antiplatelet therapy with aspirin. Anti-anginal theray will also be given, first line being beta-blockade. Statin therapy, blood sugar control in diabetics and BP control with antihypertensives may also be necessary. Those with LMS disease, 3 vessel disease or a reduced EF may benefit from CABG. Single vessel disease may benefit from PCI.

Answer 114

I. Nifedipine - First line anti-anginal therapy for stable angina is a beta blocker such as metoprolol. However, this patient has COPD and beta blockers are relatively contraindicated due to bronchospasm (even those considered to be cardioselective). 2nd line treatment is with a CCB such as nifedipine. Long acting nitrates can be used as additional therapy or in patients where beta blockers and CCBs are contraindicated.

Answer 115

F. Long acting nitrates - Long acting nitrates such as isosorbide mononitrate or transdermal GTN is indicated as the patient is still symptomatic on beta blockers and CCBs. Appropriate nitrate-free periods will be needed to avoid tolerance. Severe hypotension may occur if combined with a phosphodiesterase-5 inhibitor.

Answer 116

G. Angioplasty - Those with LMS disease, 3 vessel disease or a reduced EF may benefit from CABG. Most single vessel disease can be adequately managed with PCI.

Answer 117

B. Angiotensin converting enzyme inhibitor therapy - This patient has worsening LV function in line with heart failure. First line treatment is with an ACE inhibitor which reduces morbidity and mortality associated with the condition. All patients with LV dysfunction should receive ACE inhibitors, whether symptomatic or not. Caution should be taken if the patient has renal impairment, cardiogenic shock or hyperkalaemia. All patients with chronic heart failure will also receive a beta blocker such as carvedilol.

Answer 118

A. Weight reduction and increased physical activity - This woman is obese (BMI greater than or equal to 30) and needs to lose weight. Central obesity has a greater correlation with co-morbidities than peripheral obesity so arguably waist circumference is a better indicator of risk than body mass index. The mainstay of treatment is with diet and exercise. This patient is obese and drug therapy can be considered as an adjunct. This is primarily with orlistat which inhibits fat absorption by inhibiting lipases. For weight loss, the recommended intake is 1200-1500 kcal/day for men and 1000-1200kcal/day for women, producing a 500-1000kcal/day deficit.

Answer 119

G. Aspirin therapy - A TIA is colloquially called a ‘mini stroke’ with symptoms typically lasting under an hour. An antiplatelet drug such as aspirin is effective secondary prevention if the patient is not already anticoagulated. The patient will be anticoagulated if they have a likely or known cardioembolic source such as AF. Clopidogrel is an alternative in those who do not tolerate aspirin.

Answer 120

C. Weight reduction and metformin therapy - This patient has DM. Symptomatic patients need a single random blood glucose of >11.1 or single fasting glucose of >7. Asymptomatic patients need two separate elevated readings for a diagnosis. Alternatively if there are borderline results, an OGTT can be conducted to see if plasma glucose is raised >11.1 two hours after an oral glucose load of 75g. A patient is said to have impaired fasting glucose if fasting glucose falls between 6.1-6.9. Impaired glucose tolerance is present if plasma glucose 2 hours after oral glucose load in OGTT falls between 7.8-11.0. First line intervention in this situation is diet and lifestyle advice and changes. Metformin will be added if there is no adequate response. In terms of this question, this option will reduce cardiovascular risk the most for this patient. Metformin is a biguanide and suppresses hepatic glucose production.

Answer 121

F. Innocent Murmur - This is a functional murmur which is not caused by a structural cardiac defect. Functional murmurs tend to be systolic, occuring in an otherwise healthy individual with no symptoms. They are also characteristically position dependent and soft in nature. Ones that occur in children tend to disappear as the child grows. Benign paediatric murmurs include Still’s murmur.

Answer 122

D. Mitral stenosis - rheumatic - Sydenham's chorea (St Vitus Dance) are dancelike movements seen in rheumatic fever. The major criteria for rheumatic fever can be remember by CASES: carditis, arthritis, Sydenham’s chorea, erythema marginatum and subcutaneous nodules. Practically every single case of mitral stenosis is caused by rheumatic heart disease. The process tends to also cause regurgitation. This is characteristically a grade 1-2 low pitch murmur heard in mid-diastole which has a rumbling nature and there is no radiation. There can be an associated malar flush, tapping apex beat and a diastolic thrill palpable at the apex, in the 5th intercostal space in the MCL. The first heart sound is also characteristically loud and often this is the most striking feature on ascultation. It is a difficult murmur to pick up so if you are ever asked at this stage to spot this murmur, it will most likely be based on the loud S1.

Answer 123

E. Infective endocarditis - Any patient presenting with fever and a new murmur should always make you think of bacterial endocarditis. The classic new or worsening murmur is actually rare. As are splinter haemorrhages, which this patient has. Other uncommon signs you may find include Janeway lesions (painless macular haemorrhagic plaques on the palms and soles) and Osler nodes (painful nodules on the pads of the fingers and toes). Roth spots may also be seen on fundoscopy. Three sets of bood cultures are required and this patient will have to go for an echocardiogram.The Duke criteria is used for diagnosis.

Answer 124

G. Aortic stenosis - Aortic stenosis can present with chest pain, dyspnoea and syncope. It is characterised by a harsh ejection systolic murmur heard loudest at the right upper sternal edge at end expiration, which radiates up towards the carotids. The pulse pressure is narrow and there may be an associated slow-rising and plateau pulse. Doppler echo is vital for diagnosis and shows a pressure gradient across the narrowed valve orifice. 20% of cases are due to a congenital bicuspid valve. The most common cause of aortic stenosis in adults is calcification of normal trileaflet valves. Clinically stable patients may be considered for surgical repair or TAVR.

Answer 125

B. Mitral incompetence - MR is loudest at the apex and radiates to the axilla and tends to be around grade 4. It is associated with a systolic thrill at the apex. TTE is the investigation of choice for diagnosis. Chronic MR is associated with a laterally displaced apex beat with LV dilatation. Mitral valve prolapse is a strong risk factor for development of MR.

Answer 126

E. Autonomic neuropathy - Autonomic neuropathy is a complication of diabetic neuropathy. Symptoms of autonomic neuropathy include... resting tachycardia (late findings due to vagal impairment), impaired HR variation, erectile dysfunction (affects many diabetic men though is not solely due to autonomic neuropathy), decreased libido and dyspareunia, orthostatic hypotension (measure BP supine and then standing after 1, 2, 3 and sometimes 5 minutes – an abnormal drop when standing is indicative) and urinary symptoms of frequency, urgency, incontinence, nocturia, weak stream and retention. Other symptoms include constipation, faecal incontinence and sweating dysfunction. Fludrocortisone may be helpful in this woman.

Answer 127

D. Volume depletion - Volume depletion is a reduction in ECF volume due to salt and fluid losses which exceed intake. Causes include vomiting, bleeding, diarrhoea, diuresis and third space losses. Symptoms do not occur until large losses have alrady occured. Cool peripheries are a sign of peripheral shut down. Confusion may reflect poor cerebral flow or uraemia.Volume depletion has led to the low BP. Other symptoms include postural hypotension and tachycardia, weight loss and signs of shock. Serum urea and creatinine is elevated (you need to eyeball the patient when looking at creatinine – a very big body builder will have a much higher creatinine), indicating poor renal blood flow. This patient needs IV saline fluid replacement.

Answer 128

A. Addison's disease - Hyperpigmentation in the palmar creases points towards Addison’s disease. Hyperpigmentation due to excess ACTH production can be mucosal or cutaneous and is more pronounced in the palms, knuckles and around scars. MSH is a byproduct of the production of ACTH from the cleavage of POMC. Sodium is low and potassium elevated. Vomiting is present in 75% of patients and nausea is a common finding. Additionally, postural hypotension may be present. The presence of other autoimmune diseases is a risk factor for the development of Addison’s.

Answer 129

I. Cardiogenic shock - Cardiogenic shock is pump dysfunction. This may occur, like in this case, after MI (shock complicates just under 10% of MIs) or may be due to cardiomyopathy, valve dysfunction or arrhythmias. This cause of shock in this patient is obviously apparent. Clinical signs of shock include stress responses of tachycardia and tachypnoea, hypotension (

Answer 130

C. Drug induced - Opioid OD symptoms include CNS depression (drowsiness, sleepiness), respiratory depression and relative bradycardia. This patient needs ventilation prior to the administration of naloxone, titrated to patient response.

Answer 131

C. ECG - This patient’s crushing central chest pain sounds like an MI. Chest pain is classically severe and heavy in nature, located centrally with possible radiation to the left arm or jaw and lasts for >20 minutes. SOB due to pulmonary congestion and sweating due to high sympathetic output are common symptoms. Risk factors incorporate the standard set of cardiovascular risks such as smoking, high BP, DM, obesity and dyslipidaemia. An ECG is indicated. If ECG shows STEMI, new LBBB or confirmed posterior MI then PCI/thrombolysis is indicated. It is worth noting that RV infarction is present in 40% of inferior infarcts so if ST elevation is seen in II, III and aVF, right sided ECG leads should be obtained.

Answer 132

E. Transthoracic echo - This patient has likely HOCM. The patient’s young age makes it unlikely to be atherosclerotic coronary artery disease (unstable angina). HOCM is the most likely cardiomyopathy and the most frequent cause of sudden cardiac death in younger people. The FH of sudden death is suggestive of this diagnosis. This has a benign prognosis is most people though symptomatic patients are treated medically with beta blockade, CCBs or disopyramide. Chest pain on exertion (playing squash) is a common presentation in those who are symptomatic, as is dyspnoea on exertion, palpitations (such as due to AF) and a history of either pre-syncope or syncope (due to LV outflow obstruction). Inheritance is autosomal dominant with a variable penetrance. Examination findings may be normal or may reveal an ejection systolic murmur which is positionally responsive and a double carotid or apex pulsation due to the transient interruption of CO. A fourth heart sound may also be heard due to hypertrophy. Echocardiography must be performed to establish a diagnosis, though ECG and CXR will also be done and may also show changes. Echo will show septal hypertrophy. There may also be MR.

Answer 133

A. Upper GI endoscopy - This patient has GORD characterised by heartburn and regurgitation of acid. It is more severe at night when the patient is lying flat and also when the patient is bending over. Risk factors include obesity and hiatus hernia. Diagnosis is generally clinical and can also be achieved by a diagnostic trial of a PPI. Normally an upper GI endoscopy is reserved for complications such as strictures, Barrett’s or cancer, or for atypical features. An OGD may show oesophagitis or Barrett’s (red velvety), however OGD may be normal. Manometry and pH monitoring may also be performed, but in this case, this patient will probably just have a therapeutic and diagnostic trial of a PPI instead of an OGD.

Answer 134

F. CT scan abdomen - This patient has acute pancreatitis. He has vomited and is describing mid-epigastric pain radiating around to the back which is relieved in the fetal position and is worse with movement. He is an alcoholic and alcoholic pancreatitis is seen more frequently in men usually after an average of 4-8 years of alcohol intake. Binge drinking also increases the risk. This patient also has nausea and may describe vomiting too, with agitation and confusion. The examination findings described here allude to a pleural effusion which is seen in half of patients with acute pancreatitis. Complicated haemorrhagic pancreatitis may exhibit Cullen’s sign, Grey-Turner’s sign and Fox’s sign. Make sure you know what these are and you are familiar with the causes of acute pancreatitis. Those caused by hypocalcaemia may display Chvostek’s sign and Trousseau’s sign. Key to diagnosis is serum amylase or lipase levels which are massively elevated. Prognostic criteria are outlined in Ranson’s criteria applied on admission and after 48 hours, or the modified Glasgow score which you can find in your Oxford Handbook. An abdominal CT is however the most sensitive and specific study and findings may include enlargement of the pancreas with irregular contours, necrosis, pseudocysts and peripancreatic fat obliteration. For interest, urinary trypsinogen-2 is now considered a better screening test than amylase but is not currently clinically used.

Answer 135

B. Thoracic spine x-ray - This patient likely has osteoporotic vertebral collapse/fracture which is compressing the intercostal nerve and causing her sudden pain in the back of her chest. The pain tends not to be related to traumatic activities and can occue on standing up or bending forwards, even coughing and sneezing. The history of corticosteroid use is associated with osteoporosis through multiple mechanisms. The thoracic spine plain x-ray would show loss of height in one of the thoracic vertebrae. Osteoporotic changes may also be seen such as osteopenia. Treatment depends on the extent of spinal involvement and the severity of the pain and whether it is complicated by spinal deformity such as kyphosis. For the diagnosis of osteoporosis, a DEXA scan is needed indicating a T score of less than or equal to -2.5.

Answer 136

E. Congestive cardiac failure - The key manifestation is dyspnoea and tiredness. CCF is a term used for patients who are breathless with oedema (signs of LVF and RVF). Elevated JVP, hepatomegaly and peripheral oedema are all signs of RVF. Initial investigations should include ECG, CXR, TTE and bloods including BNP levels. First line treatment is with an ACE inhibitor which reduces morbidity and mortality. Salt and fluid restriction is also beneficial. All patients with chronic heart failure will also receive a beta blocker such as carvedilol. Other adjuncts include spironolactone, diuretics, hydralazine and a nitrate, and digoxin. The NYHA classification criteria can be used based on symptoms to describe functional limitations and ranges from Class I to Class IV with symptoms occuring at rest. Many patients are asymptomatic for long periods of time because mild cardiac impairment is balanced by compensation.

Answer 137

D. Left ventricular failure - This patient has no signs of RVF mentioned above. You should know the distinction between LVF and RVF. RVF leads to a backlog of blood and congestion of the systemic capillaries. This causes peripheral oedema and ascites and hepatomegaly may develop. Nocturia may be a symptom as fluid returns from the legs when the patient lies down flat. LVF causes congestion in the pulmonary circulation so the symptoms are respiratory. As seen in this patient, there is SOB on exertion and orthopnoea. This is why you can ask patients in a cardiac history how many pillows they sleep with. PND can also occur as well as ‘cardiac asthma'.

Answer 138

F. Myocardial infarction - This diabetic is having a silent MI without chest pain. Silent MIs are more common in the elderly and those with DM probably due to autonomic neuropathy. Tachycardia is a common feature of MI especially anterior wall MI. Chest pain of MI is classically severe and heavy in nature, located centrally with possible radiation to the left arm or jaw and lasts for >20 minutes. SOB due to pulmonary congestion and sweating due to high sympathetic output are common symptoms. This patient also has pallor which is due to a high sympathetic output. Risk factors incorporate the standard set of cardiovascular risks such as smoking, high BP, DM, obesity and dyslipidaemia. Patients with DM are at increased risk of CAD by a variety of mechanisms which are not fully known. If ECG shows STEMI, new LBBB or confirmed posterior MI then PCI/thrombolysis is indicated. It is worth noting that RV infarction is present in 40% of inferior infarcts so if ST elevation is seen in II, III and aVF, right sided ECG leads should be obtained. Cardiac biomarkers include CK-MB and troponin.

Answer 139

G. Decubitus angina - Usually as a complication of heart failure. This patient has chest pain which occurs on lying down, which is decubitus angina by definition.

Answer 140

A. Atrial fibrillation - This patient has hyperthyroidism. More specifically, Graves’ disease (peripheral manifestations such as ophthalmopathy do not occur with other causes of hyperthyroidism). Treatment of Graves’ aims to normalise thyroid function and is achieved by radioactive iodine, antithyroid medications or with surgery. They are all effective and relatively safe options. Symptomatic therapy is given with beta blockers such as propranolol. This patient has AF which has occured as a result of his hyperthyroid state which affects around 10% of untreated patients. Irregular HR is the hallmark feature of AF. Have a think about what the ECG would show.

Answer 141

B. Angina - This patient has presented with stable angina. Resting ECG is often normal however during exercise stress ECG (most often the Bruce Protocol) there will be ST segment depression during exercise indicative of ischaemia. Those unable to exercise to an adequate level may need stress myocardial perfusion imaging or stress echocardiography. 1st line treatment involves lifestyle changes and antiplatelet therapy with aspirin. Anti-anginal theray will also be given, first line being beta-blockade. 2nd line is with a CCB. Long acting nitrates can be used as additional therapy or in patients where beta blockers and CCBs are contraindicated. Statin therapy, blood sugar control in diabetics and BP control with antihypertensives may also be necessary. Those with LMS disease, 3 vessel disease or a reduced EF may benefit from CABG. Single vessel disease may benefit from PCI.

Answer 142

I. MI - Chest pain of MI is classically severe and heavy in nature, located centrally with possible radiation to the left arm or jaw and lasts for >20 minutes. SOB due to pulmonary congestion and sweating due to high sympathetic output are common symptoms. N&V was believed to be associated with inferior wall infarction but this has been disputed. If ECG shows STEMI, new LBBB or confirmed posterior MI then PCI/thrombolysis is indicated. Risk factors incorporate the standard set of cardiovascular risks such as smoking, high BP, DM, obesity and dyslipidaemia.

Answer 143

E. Infective endocarditis - Any patient presenting with fever and a new murmur should always make you think of bacterial endocarditis. The classic new or worsening murmur is actually rare. Other uncommon signs you may find include splinter haemorrhages, Janeway lesions (painless macular haemorrhagic plaques on the palms and soles) and Osler nodes (painful nodules on the pads of the fingers and toes). Roth spots may also be seen on fundoscopy. Septic embolic are common in IE and urinalysis may show active sediment. Three sets of bood cultures are required and this patient will have to go for an echocardiogram.The Duke criteria is used for diagnosis.

Answer 144

F. HOCM - This patient has HOCM. The patient’s young age makes it unlikely to be atherosclerotic coronary artery disease (unstable angina). HOCM is the most likely cardiomyopathy and the most frequent cause of sudden cardiac death in younger people. This has a benign prognosis in most people though symptomatic patients are treated medically with beta blockade, CCBs or disopyramide. Chest pain on exertion (playing squash) is a common presentation in those who are symptomatic, as is dyspnoea on exertion, palpitations (such as due to AF) and a history of either pre-syncope or syncope (due to LV outflow obstruction). Inheritance is autosomal dominant with a variable penetrance and there may be a FH of sudden death. Examination findings may be normal or may reveal an ejection systolic murmur which is positionally responsive and a double carotid or apex pulsation due to the transient interruption of CO. The arterial pulse is described as ‘jerky’. A fourth heart sound may also be heard due to hypertrophy. Echocardiography must be performed to establish a diagnosis, though ECG and CXR will also be done and may also show changes. Echocardiography will show septal hypertrophy. There may also be MR.

Answer 145

A. Pericarditis - This patient has presented with pericarditis. Symptoms include a sharp and severe chest pain retrosternally which is worse on inspiration and when supine, relieved by sitting forwards. The classical finding on examination is a friction rub which is said to sound like ‘walking on snow’. There may be diffuse ST elevations on ECG, an effusion on echocardiography and blood results suggesting inflammation. Complications include tamponade and constrictive pericarditis. The prior viral infection is a risk factor with the most common pericardial infection being viral. Bacterial purulent pericarditis also occurs. The inflammation is due either to direct viral attack or immune mediated damage. Other risk factors include male gender, post-MI (both ‘early’ and Dressler’s), post-pericardiotomy syndrome, neoplasm from local tumour invasion, uraemia and autoimmune conditions such as RA and SLE.

Answer 146

K. Venous ulcer - Venous ulcers occur on a background of deep venous insufficiency. There is oedema and a brown skin discolouration due to leaching of pigments and haemosiderin deposition. In addition there may be lipodermatosclerosis and an inflammatory response, which is seen as an eczema-like thickening and hardening of the skin. The skin can also be drawn tightly around the ankle. Ulceration usually follows trauma and is usually on the medial gaiter region. The base has granulation tissue and is sloughy in nature and there is a sloping edge to the ulcer. The shape is often irregular. Look up some photos to help you remember. Once significant arterial disease is excluded (ulcers can have mixed components), the mainstay of treatment is with compression bandaging, appropriate dressings and treatment of any infection with antibiotics. Maggots can also be used and varicose veins should be treated where possible to reduce recurrence. If the ulcer is not healing, a biopsy should be considered (Marjolin’s ulcer).

Answer 147

E. Cardiac failure - This sounds like a venous ulcer due to right-sided heart failure. Venous ulcers are mainly caused by either venous insufficiency or heart failure. RVF will lead to peripheral oedema which may be complicated by a venous ulcer. Initial investigations should include ECG, CXR, TTE and bloods including BNP levels. First line treatment is with an ACE inhibitor which reduces morbidity and mortality. Salt and fluid restriction is also beneficial. All patients with chronic heart failure will also receive a beta blocker such as carvedilol. Other adjuncts include spironolactone, diuretics, hydralazine and a nitrate, and digoxin.

Answer 148

D. Neuropathic ulcer - This is a case of diabetic neuropathy. This is a microvascular complication of DM and is characterised by peripheral nerve dysfunction. Pain is a common complaint such as the burning sensation this patient describes. Patient’s may also describe the pain as prickling or sticking. Complications range from the painless neuropathic ulcer described, at areas of the foot where there is weight loading (particularly the metatarsal heads), to the Charcot foot with severe architectural destruction of the foot. Foot ulceration is a common precusor to amputation. Foot care is crucial in DM. Examination should include peripheral pulses, reflexes and sensation to light touch with a 10g monofilament, vibration (128Hz tuning fork), pinprick and proprioception. The pain may be treated with medications like pregabalin and gabapentin.

Answer 149

B. Arterial ulcer - Arterial ulcers are deep and painful with a well defined edge, usually found on the shin or foot. There may be local changes such as cold peripheries, loss of hair, dusky cyanosis and toenail dystrophy. On examination, peripheral pulses may be absent or reduced. An angiogram with contrast will define the lesion and determine whether it can be improved by surgical intervention. Pain often increases when your legs are at rest and elevated. They can occur between the webs of toes so it is important to always check these in your peripheral vascular examination.

Answer 150

J. Pyoderma gangrenosum - Pyoderma gangrenosum is mainly associated with IBD (UC more so than CD), RA and the myeloid blood dyscrasias. It causes necrotic tissue leading to deep ulcers, often found on the legs. There are dark red borders.

Answer 151

C. MI - NSTEMI is a common complication of surgical procedures and is often detected as a rise in cardiac markers in the days following surgery. Chest pain of MI is classically severe and heavy in nature, located centrally with possible radiation to the left arm or jaw and lasts for >20 minutes. SOB due to pulmonary congestion and sweating due to high sympathetic output are common symptoms. If ECG shows STEMI, new LBBB or confirmed posterior MI then PCI/thrombolysis is indicated. Risk factors incorporate the standard set of cardiovascular risks such as smoking, high BP, DM, obesity and dyslipidaemia, though there are variations in risk factors attributed to STEMIs and NSTEMIs.

Answer 152

H. GORD - This is a common condition and the patient here is complaining of heartburn and acid regurgitation. The diagnosis is easily made clinically as the symptoms the patient describes are worse on lying down or bending over. Typically a patient will describe a burning sensation after meals which is not exertional. Reflux of acid into the mouth can leave a sour taste. Aside from obesity, other strong risk factors include advanced age, family history and hiatus hernia. This patient will need a trial of a PPI which will both be therapeutic and diagnostic. Complications of GORD include stricture formation, Barrett’s and oesophageal carcinoma.

Answer 153

F. Unstable angina - This is UA characterised by chest pain at rest. ECG will typically show ST depression and T wave inversion. Acute management includes antiplatelets and antithrombotics to reduce damage and complications. Long term management aims at reducing risk factors. Key risk factors include obesity, hypertension, smoking, hyperlipidaemia, FH, DM and positive FH. People with diabetes may again present with atypical symptoms. Cardiac biomarkers will not be elevated although in a patient who has had an acute MI days earlier, troponin may remain elevated (remains elevated up to 10-14 days after release). All patients with presumed cardiac chest pain should in the first instance get oxygen, morphine and GTN with antiplatelet therapy in the absence of contraindications.

Answer 154

A. Variant angina - Variant angina (Prinzmetal) is angina caused by coronary artery vasospasm rather than atherosclerosis. It occurs at rest and in cycles. Many patients will also have some degree of atherosclerosis although not in proportion to the severity of the chest pain experienced. ECG changes are of ST elevation (rather than depression) when the patient is experiencing an attack and a stress ECG will be negative. Patients with Prinzmetal angina are often treated for ACS and indeed, cardiac biomarkers may be raised as vasospasm can cause damage to the myocardium. The gold standard investigation is with coronary angiography and the injection of agents to try to provoke a spasm.

Answer 155

G. Stable angina - This patient has presented with stable angina. Resting ECG is often normal and the patient is asymptomatic. However during exercise stress ECG (most often the Bruce Protocol) there will be ST segment depression during exercise indicative of ischaemia and the patient will complain of chest pain. Those unable to exercise to an adequate level may need stress myocardial perfusion imaging or stress echocardiography. 1st line treatment involves lifestyle changes and antiplatelet therapy with aspirin. Anti-anginal theray will also be given, first line being beta-blockade. Statin therapy, blood sugar control in diabetics and BP control with antihypertensives may also be necessary. Those with LMS disease, 3 vessel disease or a reduced EF may benefit from CABG. Single vessel disease may benefit from PCI.

Answer 156

B. Pulmonary embolism - Patients with a high clinical suspicion of PE should be anticoagulated while waiting a definitive diagnosis unless contraindicated. The underlying pathophysiology is based on Virchow’s triad. SOB and chest pain are common symptoms and there may also be haemoptysis. This patient has recent air travel, which is actually a weak risk factor but seems to crop up a lot on EMQs. Strong risk factors include DVT, obesity, surgery in the past 2 months, prolonged bed rest, malignancy, previous VTE, pregnancy and the thrombophilias such as factor V Leiden. ECG may be normal, or may show tachycardia, new RAD, new RBBB or the classical S wave in I, Q wave with T inversion in III. Various clinical probability scores exist for PE and D-dimer can be used to exclude PE as a diagnosis.

Answer 157

E. Anxiety - The presence of anxiety does not exclude a cardiac cause and appropriate investigations are required even if the patient obviously has anxiety.The absence of an attributable cause and the sudden onset and spontaneous remission with no cardiac risk factors make this likely to be due to anxiety.

Answer 158

J. Dissecting aortic aneurysm - The tearing chest pain suggests aortic dissection. There may also be interscapular pain with dissection of the descending aorta. Dissecting aneurysms are either type A, which involves the ascending aorta, or type B. Type A dissections require urgent surgery whereas type B can be managed medically if it is not complicated by end organ ischaemia. BP differential between the 2 arms is a hallmark feature. Pulse differences may also be present in the lower limbs. There may also be the diastolic murmur of AR in proximal dissections.

Answer 159

C. MI - Chest pain of MI is classically severe and heavy in nature, located centrally with possible radiation to the left arm or jaw and lasts for >20 minutes. SOB due to pulmonary congestion and sweating due to high sympathetic output are common symptoms. This patient also has pallor which is due to a high sympathetic output. Risk factors incorporate the standard set of cardiovascular risks such as smoking, high BP, DM, obesity and dyslipidaemia. If ECG shows STEMI, new LBBB or confirmed posterior MI then PCI/thrombolysis is indicated. It is worth noting that RV infarction is present in 40% of inferior infarcts so if ST elevation is seen in II, III and aVF, right sided ECG leads should be obtained. Cardiac biomarkers include CK-MB and troponin. Troponins rise 4-6 hrs after onset of infarction and peak at 18-24 hours and may persist for 7-10 days.

Answer 160

A. Pericarditis - This patient has presented with pericarditis. Symptoms include a sharp and severe chest pain retrosternally which is worse on inspiration and when supine, relieved by sitting forwards. The classical finding on examination is a friction rub which is said to sound like ‘walking on snow’. There may be diffuse (saddle-shaped) ST elevations on ECG, an effusion on echocardiography and blood results suggesting inflammation. Complications include tamponade and constrictive pericarditis. The prior viral infection is a risk factor with the most common pericardial infection being viral. Bacterial purulent pericarditis also occurs. The inflammation is due either to direct viral attack or immune mediated damage. Other risk factors include male gender, post-MI (both ‘early’ and Dressler’s), post-pericardiotomy syndrome, neoplasm from local tumour invasion, uraemia and autoimmune conditions such as RA and SLE.

Answer 161

H. Tricuspid regurgitation - The murmur of TR is a lower left parasternal systolic murmur (pansystolic, or less, depending on severity). The murmur commonly increases on inspiration (Carvallo’s sign). The apex is not displaced in TR whereas it may be displaced in MR. Key risk factors for TR include LVF, rheumatic heart disease, endocarditis and the presence of a permanent pacemaker. Patients typically present with SOB, periperal oedema and tiredness. There may also be abdominal distension. An enlarged and pulsatile liver is normally seen in severe TR and caused by a reversal in blood flow during systole. It is worth noting that mild to moderate TR is not necessarily abnormal and is present in many asymptomatic young adults.

Answer 162

I. Mitral valve prolapse - This is mitral valve prolapse characterised by the mid-systolic click and the late systolic murmur, the combination of which is specific for mitral prolapse but not sensitive enough for diagnosis. It occurs when one or more leaflets of the mitral valve prolapse into the LA in systole. Dynamic manoevres such as Valsalva or squatting change the timing of the murmur and click. Patients also commonly suffer from palpitations. Diagnosis can be confirmed with an echocardiogram. Connective tissue diseases such as Marfan’s are key risk factors. The exact cause of MVP is unknown but genetic links have been identified and there is histological myxomatous degeneration in MVP. MR is a complication of MVP.

Answer 163

D. Aortic stenosis - Aortic stenosis can present with chest pain, dyspnoea and syncope. It is characterised by a harsh ejection systolic murmur heard loudest at the apex at end expiration, which radiates up towards the carotids. The pulse pressure is narrow and there may be an associated slow-rising and plateau pulse. Carotid parvus et tardus may also be present. Doppler echo is vital for diagnosis and shows a pressure gradient across the narrowed valve orifice. 20% of cases are due to a congenital bicuspid valve which experience abnormal shear stress. Bicuspid valves are more common in those with aortic co-arctation and Turner’s. The most common cause of aortic stenosis in adults is calcification of normal trileaflet valves. Clinically stable patients may be considered for surgical repair or TAVR. The murmur of aortic sclerosis is usually less than grade 2 and describes leaflet thickening without obstruction. The pulse character is normal in sclerosis as blood flow is normal. It is the beginning of a spectrum of calcific aortic disease with severe stenosis at the end of the spectrum.

Answer 164

J. HOCM - This patient has HOCM which is the most frequent cause of sudden cardiac death in younger people. Examination findings may be normal or may reveal an ejection systolic murmur which is positionally responsive and a double carotid or apex pulsation due to the transient interruption of CO. A fourth heart sound may also be heard due to hypertrophy. There may also be MR. The arterial pulse is described as ‘jerky’ and this patient is also in AF which warrants anticoagulation and anti-arrhythmics. Echocardiography must be performed to establish a diagnosis, though ECG and CXR will also be done and may also show changes. Echo will show septal hypertrophy. This has a benign prognosis in most people though symptomatic patients are treated medically with beta blockade, CCBs or disopyramide. Chest pain on exertion is a common presentation in those who are symptomatic, as is dyspnoea on exertion, palpitations (such as due to AF) and a history of either pre-syncope or syncope (due to LV outflow obstruction). Inheritance is autosomal dominant with a variable penetrance. It is worth noting that ventricular hypertrophy causes concentric hypertrophy i.e. the wall of the ventricle gets thicker inwards. Hence the apex beat is not displaced unlike in DCM.

Answer 165

C. Patent ductus arteriosus - The ductus arteriosus is a fetal structure which normally closes within 2 days of birth. Persistence can result in heart failure and increased pressures in the pulmonary vasculature as blood is shunted from the aorta into the pulmonary artery. The classic murmur is known as a Gibson murmur or machinery murmur and is best heard in the left infraclavicular area, usually peaking in late systole and continuing into diastole. Maternal rubella infection in the first trimester is a predisposing risk factor for PDA. The definitive diagnostic test is an echocardiogram.

Answer 166

C. Aortic stenosis - Aortic stenosis is characterised by a harsh ejection systolic murmur heard loudest at the right upper sternal border at end expiration, which radiates up towards the carotids. The pulse pressure is narrow and there may be an associated slow-rising and plateau pulse. Doppler echo is vital for diagnosis and shows a pressure gradient across the narrowed valve orifice. 20% of cases are due to a congenital bicuspid valve. The most common cause of AS in adults is calcification of normal trileaflet valves. Clinically stable patients may be considered for surgical repair or TAVR. Presentation includes chest pain, dyspnoea and syncope.

Answer 167

E. Aortic regurgitation - Aortic regurgitation is the leakage of blood back into the LV in diastole. The collapsing pulse is also known as a water hammer or Corrigan’s pulse and describes the rapid rise and quick ‘collapse’ of the arterial pulse resulting in a wide pulse pressure. The murmur in AR is early diastolic in mild cases and increases to pansystolic in severe cases. Risk factors include a bicuspid valve, rheumatic fever, endocarditis, anklylosing spondylitis and Marfan’s. Other commonly seen signs in EMQs, although uncommon in clinical practice include Traube’s, Quincke’s, Duroziez’s and de Musset’s sign. Occasionally although uncommon, an Austin Flint murmur may be heard which is a rumbling mid-diastolic murmur best heard at the apex, produced by the regurgitant jet hitting the LV endocardium. Its presence indicates severe AR and the absence of a loud S1 or an opening snap distinguishes this from the murmur of mitral stenosis.

Answer 168

H. Mitral valve prolapse - This is mitral valve prolapse characterised by the mid-systolic click and the late systolic murmur, the combination of which is specific for mitral prolapse but not sensitive enough for diagnosis. It occurs when one or more leaflets of the mitral valve prolapse into the LA in systole. Dynamic manoevres such as Valsalva or squatting change the timing of the murmur and click. Patients also commonly suffer from palpitations. Diagnosis can be confirmed with an echocardiogram. Connective tissue diseases such as Marfan’s are key risk factors. The exact cause of MVP is unknown but genetic links have been identified and there is histological myxomatous degeneration in MVP.

Answer 169

F. Systemic hypertension - Fundoscopy clearly demonstrates changes associated with hypertensive retinopathy. There are 4 grades: Grade 1: ‘Silver wiring’ and tortuous vessels, Grade 2: Plus ‘AV nipping’, Grade 3: Plus cotton wool spots (previously called soft exudates but they are not exudates) and flame haemorrhages, Grade 4: Plus papilloedema

Answer 170

J. Phaeochromocytoma - Phaeochromocytomas presents with paroxysmal episodes of palpitations, anxiety, excessive sweating, pallor and hypertension. The postural dizziness is thought to be due to a reduction in volume secondary to alpha stimulation. Episodic panic attacks are seen commonly in adrenaline producing phaeochromocytomas. Headaches occur in up to 90% of those symptomatic. It can be inherited in MEN2, von Hippel-Lindau syndrome and NF1. Diagnosis is based on raised urinary and serum catecholamines, metanephrines and normetanephrines. 24 hour urinary VMA will be elevated. CT is used to localise the tumour. Treatment includes medical with the use of phenoxybenzamine, phentolamine and surgical options. Surgical excision is carried out under alpha and beta blockade to protect against the release of catecholamines into circulation when the tumour is being manipulated. The 10% rule is often quoted: 10% are bilateral, 10% malignant, 10% extraadrenal and 10% hereditary.

Answer 171

I. Hyperthyroidism - This woman has hyperthyroidism. Symptoms include those mentioned and heat intolerance, sweating, tremor and tachycardia. In countries where sufficient iodine intake is not an issue, Graves’ disease is the most common cause of hyperthyroidism. Treatment aims to normalise thyroid function and is achieved by radioactive iodine, antithyroid medications or with surgery. Symptomatic therapy is given with beta blockers such as propranolol.

Answer 172

C. Renal artery stenosis - This is a renal bruit which is best heard in the MCL at the costal margin. The finding of a bruit in the abdomen should make you suspicious of renal artery stenosis. RAS is due, typically, to atherosclerosis and often presents with hypertension and worsening renal function. A form of imaging is required for diagnosis.

Answer 173

F. Coarctation of the aorta - Aortic coarctation is characterised by a BP difference between the upper and lower extremities. Posterior rib notching is due to enlargement of collateral vessels due to aortic narrowing. Diagnosis is made on demonstrating narrowing of the aortic arch, typically shown by echocardiography. Treatment may involve surgical repair such as the placement of a stent. This condition is typically congenital with a male predominance. It is commonly detected in the first decade and is associated with Turner’s and DiGeorge. An ejection systolic murmur is also common present over the LSB and back.

Answer 174

H. Cushings disease - There is weight gain in Cushing’s due to hypercorticolism. Gradual bitemporal hemianopia occurs due to an enlarging pituitary adenoma. Cushing’s disease is due to an ACTH secreting pituitary adenoma and is responsible for most cases of Cushing’s syndrome. A low dose 1mg overnight dexamethasone suppresion test can be done, or a 24 hour urinary free cortisol collection to diagnose Cushing’s syndrome. Plasma ACTH should guide further investigation. If ACTH is suppressed, the problem is likely to be with the adrenals. If it not suppressed, pituitary or ectopic disease is more likely. The treatment of choice in this case is surgical resection of the adenoma.

Answer 175

L. Exercise ECG - This patient has presented with stable angina. Resting ECG is often normal however during exercise stress ECG (most often the Bruce Protocol) there will be ST segment depression during exercise indicative of ischaemia. Those unable to exercise to an adequate level may need stress myocardial perfusion imaging or stress echocardiography. 1st line treatment involves lifestyle changes and antiplatelet therapy with aspirin. Anti-anginal theray will also be given, first line being beta-blockade. Statin therapy, blood sugar control in diabetics and BP control with antihypertensives may also be necessary. Those with LMS disease, 3 vessel disease or a reduced EF may benefit from CABG. Single vessel disease may benefit from PCI.

Answer 176

D. Coronary angiogram - Variant angina (Prinzmetal) is angina caused by coronary artery vasospasm rather than atherosclerosis. It occurs at rest and in cycles. Many patients will also have some degree of atherosclerosis although not in proportion to the severity of the chest pain experienced. ECG changes are of ST elevation (rather than depression) when the patient is experiencing an attack and a stress ECG will be negative. Patients with Prinzmetal angina are often treated for ACS and indeed, cardiac biomarkers may be raised as vasospasm can cause damage to the myocardium. The gold standard investigation is with coronary angiography and the injection of agents to try to provoke a spasm.

Answer 177

C. Ventilation/perfusion scan - This patient has a PE. The study of choice is a CTPA with direct visualisation of the thrombus. If there is a contraindication to a CT scan such as contrast allergy or pregnancy, then a V/Q scan is indicated. If a V/Q scan is not possible, alternatives such as MRA can be requested. It is worth noting that in patients with cardiopulmonary disease, these tests may not be accurate. A TTE can also be used to detect RV strain seen with PE.

Answer 178

I. ECG - This patient has presented with pericarditis. Symptoms include a sharp and severe chest pain retrosternally which is worse on inspiration and when supine, relieved by sitting forwards. The classical finding on examination is a friction rub which is said to sound like ‘walking on snow’. ECG is the investigation of choice with changes seen in 90%. There may be diffuse (saddle-shaped) ST elevations on ECG. Also, an effusion may be seen on echocardiography and blood results suggesting inflammation. Complications include tamponade and constrictive pericarditis. Risk factors include viral/bacterial infection (this patient’s sore throat), male gender, post-MI (both ‘early’ and Dressler’s), post-pericardiotomy syndrome, neoplasm from local tumour invasion, uraemia and autoimmune conditions such as RA and SLE.

Answer 179

C. Chest x-ray - The signs and symptoms this patient has points to CCF (congestive cardiac failure). This patient has a history of hypertension and is elderly. Other key cardiovascular risk factors include MI, DM and dyslipiaemia. SOB with orthopnoea due to the sudden increase in pre-load, indicates LV failure. Neck vein distension is also present, which is a major Framingham criteria for diagnosis. Tachycardia and ankle oedema are both minor criteria for diagnosis. Other major criteria for diagnosis include S3 gallop, cardiomegaly and hepatojugular reflux. For all patients, initial investigations should include ECG, CXR, TTE and bloods including BNP levels. CXR may reveal pulmonary vascular redistribution to the upper zones, Kerley B lines, an increased CTR (cardiomegaly) and pleural effusion.

Answer 180

E. 24hr urine protein - The most common cause of nephrotic syndrome in adults with long standing diabetes is diabetic nephropathy. However, non-diabetic renal disease cannot be excluded. Nephrotic syndrome is defined by the presence of proteinuria (>3.5g/24h), oedema and hypoalbuminaemia. Some definitions add hyperlipidaemia. Do not confuse this with nephritic syndrome. Diagnosis is made by quantification of proteinuria with a 24 hour urine collection, although now it is common to do a spot urine protein-to-creatinine ratio for practical reasons.

Answer 181

F. Pelvic ultrasound - There is a large mass in the pelvis which is most likely malignant given the history. This needs to be investigated by pelvic ultrasound.

Answer 182

H. Plasma creatinine - Diabetic patients are at risk of diabetic nephropathy and need to have their plasma creatinine regularly checked to monitor renal function. Tiredness, loss of appetite, confusion and pruritis can all be subtle signs of worsening renal function.

Answer 183

D. Venous doppler studies - Recent surgery, especially orthopaedic surgery, is a strong risk factor for developing a DVT. Other strong risks include active malignancy, pregnancy, obesity and coagulopathies such as factor V Leiden. A Wells score is determined in all patients with a suspected DVT with the condition being likely if the score is 2 or more. If the Wells score is

Answer 184

E. Upper GI endoscopy - This patient has GORD characterised by heartburn and regurgitation of acid. It is more severe at night when the patient is lying flat and also when the patient is bending over. Risk factors include obesity and hiatus hernia. Diagnosis is generally clinical and can also be achieved by a diagnostic trial of a PPI. Normally an upper GI endoscopy is reserved for complications such as strictures, Barrett’s or cancer, or for atypical features. An OGD may show oesophagitis or Barrett’s (red velvety), however OGD may be normal. Manometry and pH monitoring may also be performed, but in this case, this patient will probably just have a therapeutic and diagnostic trial of a PPI instead of an OGD.

Answer 185

D. Exercise ECG - During exercise stress ECG (most often the Bruce Protocol) there will be ST segment depression during exercise indicative of ischaemia. Those unable to exercise to an adequate level may need stress myocardial perfusion imaging or stress echocardiography. 1st line angina treatment involves lifestyle changes and antiplatelet therapy with aspirin. Anti-anginal theray will also be given, first line being beta-blockade. Statin therapy, blood sugar control in diabetics and BP control with antihypertensives may also be necessary. Those with LMS disease, 3 vessel disease or a reduced EF may benefit from CABG. Single vessel disease may benefit from PCI.

Answer 186

I. Chest X-ray - This patient has pulmonary oedema likely due to heart failure. There is also a pleural effusion which may be due to LVF. CXR may show pulmonary vascular redistribution to the upper zones, Kerley B lines, an increased CTR (cardiomegaly) and pleural effusion consistent with pulmonary oedema. Other investigations should include ECG, TTE and bloods including BNP levels. First line treatment for chronic heart failure is with an ACE inhibitor which reduces morbidity and mortality. Salt and fluid restriction is also beneficial. All patients with chronic heart failure will also receive a beta blocker such as carvedilol. Other adjuncts include spironolactone, diuretics, hydralazine and a nitrate, and digoxin.

Answer 187

C. CTPA - This patient has a PE. Regardless of whether there is lung disease, guidelines state that the study of choice if there is an initial high probability of PE is a CTPA with direct visualisation of the thrombus (a filling defect is seen). If there is a contraindication to a CT scan such as contrast allergy or pregnancy, then a V/Q scan is indicated.

Answer 188

G. CT scan - A CT scan is indicated as soon as a diagnosis of aortic dissection is suspected and should be from the chest to the pelvis to see the full extent of the dissecting aneurysm. MRI is more sensitive and specific but is more difficult to obtain acutely. Aortic dissection typically presents with tearing/ripping chest pain and classically radiates through to the back. There may be interscapular pain with dissection of the descending aorta. Dissecting aneurysms are either type A, which involves the ascending aorta, or type B. Type A dissections require urgent surgery whereas type B can be managed medically if it is not complicated by end organ ischaemia. BP differential between the 2 arms is a hallmark feature. Pulse differences may also be present in the lower limbs. There may also be the diastolic murmur of AR in proximal dissections.

Answer 189

F. Pulmonary fibrosis - Idiopathic pulmonary fibrosis (previously known as Cryptogenic fibrosing alveolitis) progresses over several years and is characterised by pulmonary scar tissue formation and dyspnoea. Patients complain of a non-productive cough and typically reproducible and predictable SOB on exertion. Boiler makers can come into contact with small organic or inorganic dust particles which is thought to be implicated in the cascade of events leading to IPF. While this patient does not smoke, another risk factor is cigarette smoking which significantly increases the risk of IPF. The mean age of diagnosis is 60-70. End expiratory basal crackles are found on examination. These are described as ‘Velcro-like’ in quality. IPF is also associated with clubbing.

Answer 190

C. Pulmonary TB - HIV infection is a key risk factor for pulmonary TB. It is important to have a high level of suspicion when evaluating patients with risk factors who present with suggestive symptoms. Night sweats, fever, malaise, cough, haemoptysis and erythema nodosum are all suggestive. In the first half of the 20th century, tuberculosis accounted for over 90% of cases of erythema nodosum. Other key risk factors for pulmonary TB include exposure to infection and returning from or being born in a high-risk region such as Asia, Africa and Latin America. If TB is suspected, the patient should be placed in isolation and a CXR obtained with 3 sputum samples cultured for AFB being the gold standard of diagnosis. Culture takes several weeks so sputum smears will be done before culture results are known. Interferon-gamma release assays (IGRAs) are now used by some hospitals to rapidly determine a patient’s TB status. All patients who have TB should be tested for HIV within 2 months of diagnosis.

Answer 191

G. Acute lymphoblastic leukaemia - ALL presents in children with bone marrow involvement and the associated symptoms or in adults with an anterior mediastinal mass. Bone marrow infiltration leads to a pancytopenia leading to anaemia (reduced red blood cells), haemorrhage (reduced platelets) and infections (reduced mature white blood cells). In ALL, bone marrow is replaced by lymphoblasts. There may also be spread to CNS and testes. ALL is associated with Down’s syndrome. In contrast, CLL presents in older adults and is often asymptomatic, discovered by chance when a FBC is ordered. Smear/smudge cells are seen in peripheral blood smear. CLL is associated with a warm-type AIHA and there is peripheral blood lymphocytosis.

Answer 192

D. COPD - Smoking is the most important risk factor, accounting for 90% of COPD. COPD has an insidious onset and usually presents in older people with a history of cough, wheeze and SOB. This patient appears to have infective exacerbations of his COPD every winter. Patients with COPD are at a higher risk of infections and are vaccinated against influenza annually and pneumococcal pneumonia every 5 years. Spirometry is the gold standard for diagnosis, with FEV1/FVC ratio

Answer 193

E. Carcinoma of the bronchus - The history of smoking and weight loss point to a bronchial carcinoma. Initial investigation is with a CXR. Diagnosis relies on pathological confirmation from a tissue sample, often obtained from bronchoscopy. First line treatment aims at surgical resection if possible. Small cell lung cancer is treated with chemotherapy and is associated with SIADH and ectopic ACTH. Non-small cell lung cancer is more often associated with clubbing. Squamous cell carcinoma is associated with PTHrp release and is treated with radiotherapy. Adenocarcinomas are usually located peripherally in the lung and are more common in non-smokers although most cases are still associated with smoking. The paraneoplastic syndromes may include Lambert-Eaton myasthenic syndrome.

Answer 194

G. ACE inhibitor - A dry cough is a side effect of ACE inhibitors due to the build up of bradykinin which is normally degraded by ACE. ARB such as losartan will be indicated in this case. ARBs are insurmountable antagonists of AT1 receptors for angiotensin II, preventing its renal and vascular effects.

Answer 195

F. Sarcoidosis - Sarcoidosis is a chronic multisystem disease with an unknown aetiology. The painful (mauve) nodules are erythema nodosum. Lupus pernio is another typical skin manifestation of sarcoidosis presenting with indurated plaques with discoloration on the face. Parotid enlargement is a classic feature (involvement of exocrine glands). The dry cough and SOB on exertion indicate pulmonary involvement. CXR will typically show bilateral hilar lymphadenopathy and CXR findings are used in the staging of disease. Additionally, serum calcium and ACE levels may be raised. A transbronchial biopsy is essential for diagnosis in most cases and shows the presence of non-caseating granulomas. Black people have a higher lifetime risk of sarcoidosis, as do those of Scandinavian origin. The mainstay of treatment for severe disease involves systemic corticosteroids.

Answer 196

C. Asthma - SOB and the cough, which may wake the patient from sleep combined with the patient’s age and progessive course suggest asthma. Examination can show an expiratory wheeze but may be normal and treatment is step-wise based on BTS guidelines. It is worth noting that in severe exacerbations, the chest may be silent. Night symptoms occur in more severe asthma and symptoms can be exacerbated by exercise. Diagnosis is supported by PEFR variation of at least 20% over 3 days in a week over several weeks or an increase of at least 20% to treatment. Stepwise treatment is outlined below. Look up the BTS guidelines for more information. Step 1: SABA PRN, Step 2: Plus low-dose inhaled corticosteroids (ICS) , Step 3: Plus LABA, Step 4: Increase dose of ICS or add LTRA, SR theophylline or beta agonist tablet, Step 5: Daily steroid tablet and maintain ICS with specialist care.

Answer 197

D. Bronchiectasis - Bronchiectasis is permanent bronchi dilatation due to bronchial wall damage and loss of elasticity. It is often as a consequence of recurrent/severe infections and most present with chronic productive mucopurulent cough. The most common identifiable cause is CF. Chest CT is the diagnostic test. Diagnosis is aided by sputum analysis. Have a think about what you would expect to hear on ascultation of the chest.

Answer 198

K. Bronchial carcinoma - The history of smoking, weight loss, cough and haemoptysis point to a bronchial carcinoma. Initial investigation is with a CXR. Diagnosis relies on pathological confirmation from a tissue sample, often obtained from bronchoscopy. First line treatment aims at surgical resection if possible. Small cell lung cancer is treated with chemotherapy and is associated with SIADH and ectopic ACTH. Non-small cell lung cancer is more often associated with clubbing. Squamous cell carcinoma is associated with PTHrp release and is treated with radiotherapy. Adenocarcinomas are usually located peripherally in the lung and are more common in non-smokers although most cases are still associated with smoking. The paraneoplastic syndromes may include Lambert-Eaton myasthenic syndrome.

Answer 199

G. Asthma - These are infective exacerbations of asthma. The eczema is an atopic disease which is a strong risk factor for the development of asthma. A dry night time cough is commonly seen and suggestive. The hyperexpanded chest suggests persistent asthma. A Harrison’s sulcus, though rare, may also be seen. Treatment will be based on paediatric guidelines which differs from adult asthma. If you are really keen, you can look these up.

Answer 200

C. Chronic bronchitis - This patient has COPD, which is a progressive disease characterised by not fully reversible airflow limitation. COPD encompasses both emphysema and chronic bronchitis. Cigarette smoking is the most important risk factor. The hyperexpanded chest implies trapping of air due to incomplete expiration. Wheezes and coarse crackles are commonly seen in exacerbations. The cough is often the first symptom a patient complains of and is usually a morning event which is normally productive. The sputum can change quality with exacerbations/infection. Treatment aims at stopping smoking and vaccinating the patient against influence and pneumococcus with options such as bronchodilators or ICS. LTOT improves survival in those with severe COPD with a low PaO2. Lung function tests are key in diagnosis with an obstructive FEV1/FVC ratio

Answer 201

B. TB - It is important to have a high level of suspicion when evaluating patients with risk factors who present with suggestive symptoms. Night sweats, fever, malaise, weight loss, cough, haemoptysis and erythema nodosum are all suggestive. This patient’s tracheal deviation may be due to apical fibrosis or a cavitating lesion. The swollen lymph nodes in this patient’s neck may well represent a scrofula. Other key risk factors for pulmonary TB include exposure to infection and returning from or being born in a high-risk region such as Asia, Africa and Latin America. If TB is suspected, the patient should be placed in isolation and a CXR obtained with 3 sputum samples cultured for AFB being the gold standard of diagnosis. Culture takes several weeks so sputum smears will be done before culture results are known. Interferon-gamma release assays (IGRAs) are now used by some hospitals to rapidly determine a patient’s TB status. All patients who have TB should be tested for HIV within 2 months of diagnosis.

Answer 202

F. ACE inhibitor - A dry cough is a side effect of ACE inhibitors due to the build up of bradykinin which is normally degraded by ACE. ARB such as losartan will be indicated in this case. ARBs are insurmountable antagonists of AT1 receptors for angiotensin II, preventing its renal and vascular effects.

Answer 203

H. Idiopathic pulmonary fibrosis - Idiopathic pulmonary fibrosis (previously known as Cryptogenic fibrosing alveolitis) progresses over several years and is characterised by pulmonary scar tissue formation and dyspnoea. Patients complain of a non-productive cough and typically reproducible and predictable SOB on exertion. Work in power stations can involve contact with small organic or inorganic dust particles which is thought to be implicated in the cascade of events leading to IPF. Another risk factor is cigarette smoking which significantly increases the risk of IPF. The mean age of diagnosis is 60-70. End expiratory basal crackles are found on examination. These are described as ‘Velcro-like’ in quality. IPF is also associated with clubbing. A CXR in most will show reticulonodular shadowing consistent with fibrosis. This can be described as a ‘honeycomb’ pattern.

Answer 204

B. Sarcoidosis - Sarcoidosis is a chronic multisystem disease with an unknown aetiology. The lesion described is lupus pernio which is a typical skin manifestation of sarcoidosis presenting with indurated plaques with discoloration on the face. Erythema nodosum is another dermatological manifestation. Additionally, serum calcium and ACE levels may be raised. CXR will typically show bilateral hilar lymphadenopathy and CXR findings are used in the staging of disease. A transbronchial biopsy is essential for diagnosis in most cases and shows the presence of non-caseating granulomas. Black people have a higher lifetime risk of sarcoidosis, as do those of Scandinavian origin. The mainstay of treatment for severe disease involves systemic corticosteroids.

Answer 205

F. Tuberculosis - This patient should be placed in isolation. His sputum culture results suggest TB which is an AFB growing on Lowenstein-Jensen medium. A sputum culture is the most sensitive and specific test for TB and whilst growth takes a long time, the positive culture is diagnostic of TB. Sputum cultures will be repeated during treatment until 2 consecutive negative cultures. Treatment consists of anti-TB medication. Ethambutol should be stopped if the AFB is sensitive to isoniazid and rifampicin. Pyridoxine should be given with isoniazid to help prevent neuropathy. Pyrazinamide is not recommended in acute gout or pregnancy. Treatment of MDR TB involves additional therapy with agents such as amikacin and ethionamide.

Answer 206

K. Mycoplasma pneumonia - The atypical presentation (a week of headache and malaise before the cough) and the prescription of erythromycin, a macrolide antibiotic, point to infection with an atypical pneumonia. However, depending on local prescribing policies, first line therapy for a CAP like pneumococcus may also be with a macrolide. Young people who ‘live together’ are commonly affected. The cough often does not resolve and is dry in nature. Symptoms tend to be prolonged and a low-grade fever is a common finding. Mycoplasma is the only atypical pneumonia on the list although there is no reason why this cannot be Legionella or Chlamydia. All can be treated with macrolides although in EMQs Chlamydia tends to be treated with doxycyline. All 3 atypicals are to some extent sensitive to fluoroquinolones and tetracylines too although these cannot be used in pregnancy. Whether they are first or second line therapy depends on the organism.

Answer 207

L. Cystic fibrosis - CF can present in newborns with a failure to pass meconium (early stools) which can even result in bowel obstruction as in this patient. The bowel may even perforate if the patient is very unlucky resulting in a meconium peritonitis. The most conclusive diagnostic test is the sweat test which is pisitive if sweat chloride is >60mmol/L. Serum IRT from a heel prick blood spot allows screening of newborns. CF is a genetic condition with abnormal salt and water transport due to mutations in the CFTR (an apical anion channel). Heterozygotes generally do not demonstrate disease.

Answer 208

D. Wegeners Granulomatosis - Wegener’s is a systemic vasculitis affecting the small and medium vessels and presents with the classic triad of upper respiratory tract involvement, lower respiratory tract involvement and GN. It is a multisystem disease which can manifest with symptoms ranging from cutaneous, musculoskeletal, ocular and neurological features. Joint swelling and tenderness may all be present. It is associated with a positive cANCA and rhematoid factor is positive in around half.

Answer 209

F. Anaphylaxis - There is a sudden onset of both respiratory and cardiovascular complaints with the recently given IV contrast agent. This patient is having an anaphylactic reaction and the airway needs to be promptly secured with prompt treatment with adrenaline. IM adrenaline must not be delayed and the anterolateral thigh is the preferred location, with repeated doses as necessary every 10 minutes or so. A 1:1000 solution is used of 0.3-0.5mg adrenaline. Fluid replacement with IV saline is also indicated to correct the intravascular volume redistribution.

Answer 210

G. Left ventricular failure - This patient has LVF and pulmonary oedema. This accounts for the basal crepitations heard on ascultation and the SOB which is better on sitting upright. Patients need to be sat upright for this reason and IV access needs to be established. Oxygen, morphone, diuretics (frusemide or another loop diuretic) and nitrates will be given. Once stable, medical treatment of heart failure should be started which involves in the first instance, an ACE inhibitor followed by beta blockade. Ongoing diuretics are necessary if the patient has persistent symptoms of fluid overload.

Answer 211

J. Pulmonary embolus - This patient should be thrombolysed immediately if not contraindicated due to her haemodynamically unstable and critical state. Treatment should not be delayed in this obvious PE. This patient is now at a serious risk of cardiac arrest. Anticoagulation should also be started. The underlying pathophysiology is based on Virchow’s triad. SOB is a common symptom and there may also be chest pain and haemoptysis. This patient has had recent surgery, particularly orthopaedic surgery, which is a strong risk factor for PE. Other strong risk factors include DVT, obesity, prolonged bed rest, malignancy, previous VTE, pregnancy and the thrombophilias such as factor V Leiden. ECG may be normal, or may show tachycardia, new RAD, new RBBB or the classical S wave in I, Q wave with T inversion in III. Various clinical probability scores exist for PE and D-dimer can be used to exclude PE as a diagnosis. The study of choice is a CTPA with direct visualisation of the thrombus. If there is a contraindication to a CT scan such as contrast allergy or pregnancy, then a V/Q scan is indicated. If a V/Q scan is not possible, alternatives such as MRA can be requested. It is worth noting that in patients with cardiopulmonary disease, these tests may not be accurate. A TTE can also be used to detect RV strain seen with PE.

Answer 212

E. Pneumothorax - This patient has developed a right sided tension pneumothorax and will need emergency intervention in the form of the insertion of a large bore cannula into the 2nd intercostal space in the MCL of the affected side. This will need to be followed by the insertion of a chest drain. Otherwise if this was not a tension pneumothorax, for secondary spontaneous pneumothoraces, if large enough for a chest drain or the patient is clinically unstable, chest drain insertion is indicated. Simple aspiration success rate is reduced in secondary spontaneous pneumothoraces. Primary pneumothoraces occur in young people without known lung conditions. This patient has pulmonary fibrosis. Those who suffer recurrent pneumothoraces may have to undergo pleurodesis to stick the parietal and visceral pleural together by an inflammatory reaction.

Answer 213

K. Streptococcus pneumoniae - Classic lobar pneumonia with no signs and symptoms to suggest an atypical organism is most likely to due to pneumococcus. Symptoms include chills, fever, cough, SOB and pleuritic chest pain. A CXR is the most specific and sensitive test available and antibiotics are indicated.

Answer 214

H. Pneumocystus jirovecii - PCP is caused by Pneumocystis jirovecii, previously called Pneumocystis carinii. It is a fungal organism and an AIDS defining illness. Signs and symptoms occur in a patient who is immunosuppressed especially HIV with a CD4 count

Answer 215

I. Legionella pneumophila - Legionella is a gram negative rod. Legionella infecting the lungs is legionnaires’ disease or Legionella pneumonia whereas non-lung infection is known as Pontiac fever. This bacteria is found in aqueous environments such as lakes and almost all cases are from contaminated water systems, which relates to the risk factors of getting Legionella (recent water exposure like a hot tub). Smoking is also a risk factor. It can cause confusion as well as hyponatraemia, abdominal pain, diarrhoea and bradycardia. Legionella does not grow on routine culture media and diagnosis relies on urine antigen detection, serology or culture on special media.

Answer 216

F. Mycoplasma pneumoniae - The cold agglutinins is what gives this question away. Mycoplasma is associated with with cold type agglutinins and a cold AIHA. Humans are thought to be the only host for Mycoplasma. The most commonly affected are young adults living in close proximity to each other. PCR can be used in diagnosis.

Answer 217

D. Small cell carcinoma - First line treatment aims at surgical resection if possible. Small cell lung cancer is treated with chemotherapy and is also associated with SIADH and ectopic ACTH. Non-small cell lung cancer is more often associated with clubbing. Squamous cell carcinoma is associated with PTHrp release and is treated with radiotherapy. Adenocarcinomas are usually located peripherally in the lung and are more common in non-smokers although most cases are still associated with smoking. The paraneoplastic syndromes may include Lambert-Eaton myasthenic syndrome which this patient has (though weakness of the eye muscles is uncommon in Lambert-Eaton and is more prominent in myasthenia gravis). This classically presents with weakness which improves on repeated testing (in contrast to myaesthenia gravis) and is more commonly associated with small cell lung cancer than other lung cancers. It is for this reason that the most likely diagnosis is small cell and not squamous cell lung cancer.

Answer 218

B. Pulmonary embolism - This CXR finding is Hampton’s hump seen in about a third of PE. Additional CXR signs include Westermark’s sign and Fleischner’s sign. CXR is however not diagnostic and may well be normal. Patients with a high clinical suspicion of PE should be anticoagulated while waiting a definitive diagnosis unless contraindicated. The underlying pathophysiology is based on Virchow’s triad. SOB and chest pain are common symptoms and there may also be haemoptysis. Strong risk factors include DVT, obesity, surgery in the past 2 months, prolonged bed rest, malignancy (which this patient has), previous VTE, pregnancy and the thrombophilias such as factor V Leiden. ECG may be normal, or may show tachycardia, new RAD, new RBBB or the classical S wave in I, Q wave with T inversion in III. Various clinical probability scores exist for PE and D-dimer can be used to exclude PE as a diagnosis. The study of choice is a CTPA with direct visualisation of the thrombus. If there is a contraindication to a CT scan such as contrast allergy or pregnancy, then a V/Q scan is indicated. If a V/Q scan is not possible, alternatives such as MRA can be requested. It is worth noting that in patients with cardiopulmonary disease, these tests may not be accurate. A TTE can also be used to detect RV strain seen with PE.

Answer 219

H. Tuberculosis - It is important to have a high level of suspicion when evaluating patients with risk factors who present with suggestive symptoms. Night sweats, fever, malaise, cough, haemoptysis and erythema nodosum are all suggestive. Other key risk factors for pulmonary TB include exposure to infection and returning from or being born in a high-risk region such as Asia, Africa and Latin America. If TB is suspected, the patient should be placed in isolation and a CXR obtained with 3 sputum samples cultured for AFB being the gold standard of diagnosis. Culture takes several weeks so sputum smears will be done before culture results are known. Interferon-gamma release assays (IGRAs) are now used by some hospitals to rapidly determine a patient’s TB status. All patients who have TB should be tested for HIV within 2 months of diagnosis. CXR is the first line test to order. Classically, in primary disease there are middle and lower zone infiltrates. Post-primary TB usually involves apical changes with or without cavitation. However, recent students have indicated that both presentations are seen in both primary and post-primary TB. HIV positive patients tend to have a more atypical CXR including effusion, lower zone involvement and a miliary pattern.

Answer 220

I. Goodpasture's disease - Goodpasture’s is associated with anti-GBM antibodies, and of those who are positive, some will have a positive ANCA too, although this is more suggestive of diagnoses such as Churg-Strauss and microscopic polyarteritis. Definitive diagnosis is by renal biopsy showing crescentic GN and linear IgG staining on immunofluorescence. It is one of the few causes of pulmonary renal syndrome. Aggressive treatment is often needed and plasma exchange can also be performed to remove preformed antibodies.

Answer 221

I. Pneumothorax - A tension pneumothorax shifts the trachea AWAY with hyper-resonance on affected side and reduced/absent breath sounds. A tracheal shift means this is a tension pneumothorax which needs immediate relief with a wide bore cannula inserted into the second intercostal space in the MCL.

Answer 222

F. Lobar collapse - Collapse pulls the trachea TOWARDS the affected side. There is dullness and reduced/absent breath sounds due to a lack of air filled lung in this space. Do you know how to identify which lobe has collapsed on a CXR? The findings in this examination are consistent with LLL collapse. A ‘sail sign’ will classically be seen behind the cardiac shadow on CXR.

Answer 223

B Emphysema - This patient is in obvious respiratory distress and is using accessory muscles of respiration. Chest movements are reduced too. There is hyperexpansion of the lung fields due to emphysema, which has caused the respiratory distress, combined with diminished breath sounds.

Answer 224

G. Idiopathic pulmonary fibrosis - Bibasal inspiratory crackles without signs of CCF are seen in idiopathic pulmonary fibrosis. These are described as ‘Velcro-like’ in quality. IPF is also associated with clubbing. Idiopathic pulmonary fibrosis (previously known as Cryptogenic fibrosing alveolitis) progresses over several years and is characterised by pulmonary scar tissue formation and dyspnoea. Patients complain of a non-productive cough and typically reproducible and predictable SOB on exertion

Answer 225

J. Lobar pneumonia - These are the classic signs of pneumonia. Also expect to find increased vocal resonance and tactile vocal fremitus over areas of consolidation. In reality, it can be confusing if the pneumonia causes a lobar collapse as you can also find signs of collapse on examination. Always consider the history as well as examination and investigation findings. On a CXR with pneumonia, you can expect to see air space shadowing with air bronchograms. Always remember to auscultate at the right axilla when doing a respiratory examination or a RML pneumonia may be missed.

Answer 226

A. Heimlich manoeuvre - This patient has choked on some food. The patient should be encouraged to cough if they are conscious. Otherwise, external manoevres can be performed such as abdominal thrusts (Heimlich) or back blows. These actions increase intrathoracic pressure and help to dislodge the foreign body. If it still isn’t removed, a flexible bronchoscopy may be necessary. Most cases occur in very young children.

Answer 227

C. Intravenous furosemide - This patient has pulmonary oedema. CXR may show pulmonary vascular redistribution to the upper zones, Kerley B lines, an increased CTR (cardiomegaly) and pleural effusion.

Answer 228

E. Nebulised salbutamol - This patient is having an asthma attack. This patient is too breathless to speak and has a quiet chest so this is severe and ICU admission is indicated. Initial treatment is with repeated administration of an inhaled SABA with early systemic corticosteroids and supplemental oxygen, monitoring the patient’s status regularly.

Answer 229

K. Heparin - Patients with a high clinical suspicion of PE should be anticoagulated while waiting a definitive diagnosis unless contraindicated. As UFH is used in this case, a weight based dosing normogram will need to be used to establish a therapeutic APTT within the first 24 hours. Alternatives include LMWH and fondaparinux.

Answer 230

I. Chest drain - This patient has developed a pneumothorax. This is a secondary spontaneous pneumothorax, and if large enough for a chest tube or the patient is clinically unstable, chest drain insertion is indicated. Simple aspiration success rate is reduced in secondary spontaneous pneumothoraces. Primary pneumothoraces occur in young people without known lung conditions. This patient has pulmonary fibrosis. Those who suffer recurrent pneumothoraces may have to undergo pleurodesis using a chemical such as doxycycline to stick the perietal and visceral pleural together by an inflammatory reaction.

Answer 231

C. Varicella zoster - The pruritic vesicular rash (the classic description of a ‘dewdrop on a rose petal’) makes you think of VZV. The rash typically occurs on the patient’s torso and face and pneumonia is a complication occuring more commonly in those with immunosuppression. The lesions are often crusted over by 7-10 days. The diagnosis is based on clinical findings.

Answer 232

B. Streptococcus pneumoniae - This is classic CAP. Classic lobar pneumonia with no signs and symptoms to suggest an atypical organism is most likely to due to pneumococcus. Symptoms include chills, fever, cough, SOB and pleuritic chest pain. A CXR is the most specific and sensitive test available and antibiotics are indicated.

Answer 233

E. Pneumocystis jirovecii - PCP is caused by Pneumocystis jirovecii, previously called Pneumocystis carinii. It is a fungal organism and an AIDS defining illness. Signs and symptoms occur in a patient who is immunosuppressed, especially HIV with a CD4 count

Answer 234

K. Legionella pneumphila - Legionella is a gram negative rod. Legionella infecting the lungs is legionnaires’ disease or Legionella pneumonia whereas non-lung infection is known as Pontiac fever. This bacteria is found in aqueous environments such as lakes and almost all cases are from contaminated water systems, which relates to the risk factors of getting Legionella (recent water exposure like a hot tub). Smoking is also a risk factor. It can cause confusion as well as hyponatraemia, abdominal pain, diarrhoea and bradycardia. Legionella does not grow on routine culture media and diagnosis relies on urine antigen detection, serology or culture on special media. Treatment is with fluoroquinolones or macrolides. Azithromycin is considered a safer option than erythromycin. For severe cases, a combination of both is given although this is potentially toxic and can cause long QT and torsades de pointes.

Answer 235

I. Staphylococcus aureus - Think Staphylococcus aureus for post-influenza pneumonia. It causes a cavitating pneumonia which explains the CXR findings (some abscesses are also seen) and Gram stain of culture yields grape like clusters of Gram positive cocci which is consistent with staphylococcus. Treatment of staphyloccocal infection is with flucoxacillin or vancomycin if MRSA.

Answer 236

A. Atypical pneumonia - Given this is the only pneumonia option on the list, this is an easy question, although the presentation is in line with an atypical organism. The most common atypical pneumonias are Mycoplasma, Legionella and Chlamydophila (Chlamydia). All can be treated with macrolides and are to some extent sensitive to fluoroquinolones and tetracyclines too, although these are contraindicated in pregnancy. Check local prescribing policies first.

Answer 237

G. Lung abscess - A lung abscess is diagnosed on CXR with a cavitation with an air-fluid level in it. Preceding pneumonia which this patient gives a history of is a risk factor. Fever and a productive cough are common symptoms and treatment involves antibacterials and drainage/resection if unresponsive. Lung abscesses are commonly caused by aspiration of gastric contents.

Answer 238

K. Cystic fibrosis - CF is autosomal recessive and the mean age of death is around 40. There is currently no cure for this condition. The reccurent chest infections and greasy stools (fat malabsorption due to pancreatic insufficiency) should make you think of CF. A persistent cough which is productive should also raise suspicions. Examination findings here which raise your suspicion include clubbing and crackles on auscultation. Additionally, you may find nasal polyps and hepatomegaly and/or splenomegaly and a congenital absence of the vas deferens in males. There is also some failure to thrive with the patient being small for his age. The most conclusive diagnostic test is the sweat test which is positive if sweat chloride is >60mmol/L. Serum IRT from a heel prick blood spot allows screening of newborns. CF is a genetic condition with abnormal salt and water transport due to mutations in the CFTR (an apical anion channel). Heterozygotes generally do not demonstrate disease.

Answer 239

B. Bronchial carcinoma - This patient is a heavy smoker with a history of respiratory signs and weight loss. The presentation is alluding to a pancoast tumour (most are non-small cell) in the left apex causing ipsilateral Horner’s syndrome (miosis, anhidrosis, ptosis and enophthalmos). A pancoast tumour can also affect the brachial plexus leading to wasting of the intrinsic muscles of the hand as well as a hoarse bovine cough due to compression of the recurrent laryngeal nerve. Initial investigation of bronchial carcinoma is with a CXR. Diagnosis relies on pathological confirmation from a tissue sample, often obtained from bronchoscopy. First line treatment aims at surgical resection if possible. Small cell lung cancer is treated with chemotherapy and is associated with SIADH and ectopic ACTH. Non-small cell lung cancer is more often associated with clubbing. Squamous cell carcinoma is associated with PTHrp release and is treated with radiotherapy. Adenocarcinomas are usually located peripherally in the lung and are more common in non-smokers although most cases are still associated with smoking. The paraneoplastic syndromes may include Lambert-Eaton myasthenic syndrome.

Answer 240

I. Bronchial asthma - This patient has developed a primary pneumothorax. Primary pneumothoraces occur in young people without known lung conditions. The main investigation is a CXR and pneumothoraces are classified by the BTS as large (>2cm visible rim between the lung margin and the chest wall) or small (

Answer 241

F. Pneumothorax - A tension pneumothorax shifts the trachea AWAY with hyper-resonance on affected side and reduced/absent breath sounds. A tracheal shift means this is a tension pneumothorax which needs immediate relief with a wide bore cannula inserted into the second intercostal space in the MCL.

Answer 242

C. Pulmonary abscess - A lung abscess is diagnosed on CXR with a cavitation with an air-fluid level in it. Preceding pneumonia which a patient with CF is at risk of is a risk factor. Of gram positive cocci, staphylococcus occurs in grape-like clusters (this patient) whereas streptococcus occurs in chains. It is worth learning your gram stains for the main organisms. It is worth noting that Staphylococcus aureus is coagulase positive (also Yersinia pestis which causes plague) and Streptoccus pneumoniae is optochin sensitive. Fever and a productive cough are common symptoms and treatment involves antibacterials and drainage/resection if unresponsive. Lung abscesses are commonly caused by aspiration of gastric contents.

Answer 243

N. Pulmonary TB - This is pulmonary TB. The patient is from an endemic area and has presented with fever, drenching night sweats and a cough. There is also anorexia and examination findings are consistent with post-primary TB with apical consolidation. CXR is the first line test to order. Classically, in primary disease there are middle and lower zone infiltrates. Post-primary TB usually involves apical changes with or without cavitation. However, recent students have indicated that both presentations are seen in both primary and post-primary TB. HIV positive patients tend to have a more atypical CXR including effusion, lower zone involvement and a miliary pattern.

Answer 244

B. Pneumonia - The rusty coloured phlegm is hinting at a pneumococcal pneumonia.The patient has presented with common symptoms of fever, chills and a cough. There may also be SOB, rigors and pleuritic chest pain. The most specific and sensitive test is a CXR (PA and lateral) and initial treatment of a CAP is empirical with antibiotics. Often diagnosis is made solely on history and examination findings. Bronchial breathing, reduced breath sounds and the presence of a left sided parapneumonic effusion all indicate a pneumonia. Management is guided by the patient’s CURB-65 score.

Answer 245

H. Pleural mesothelioma - An ex-builder is likely to have a history of asbestos exposure which is the principal risk factor for developing a malignant pleural mesothelioma. CXR shows the presence of a unilateral effusion and irregular pleural thickening. Most patients present with SOB and chest pain. There is a 20-40 year latency period between exposure to asbestos and development of malignancy. Hence, the typical patient presents between 60-80. A more invasive investigation is needed for a definitive diagnosis.

Answer 246

D. Carcinoma of bronchus - The history of smoking and weight loss combined with examination findings point to a bronchial carcinoma. First line treatment aims at surgical resection if possible. Small cell lung cancer is treated with chemotherapy and is associated with SIADH and ectopic ACTH. Non-small cell lung cancer is more often associated with clubbing. Squamous cell carcinoma is associated with PTHrp release and is treated with radiotherapy. Adenocarcinomas are usually located peripherally in the lung and are more common in non-smokers although most cases are still associated with smoking. The paraneoplastic syndromes may include Lambert-Eaton myasthenic syndrome.

Answer 247

J. Pulmonary oedema - CXR findings here are consistent with pulmonary oedema. Pulmonary vascular redistribution to the upper zones, Kerley B lines, an increased CTR (cardiomegaly) and pleural effusion may be seen. The patient is also in CCF with evidence of LV dysfunction (SOB) and RV dysfunction (ankle swelling).

Answer 248

H. Asthma - This is likely to be asthma, exacerbated by the cold air and exercise. Treatment is in this case based on paediatric guidelines which differs from that of adult asthma. Diagnosis is supported by PEFR variation of at least 20% over 3 days in a week over several weeks or an increase of at least 20% to treatment. Stepwise treatment for adult asthma (BTS guidelines) is outlined here as this is most commonly examined. Step 1: SABA PRN, Step 2: Plus low-dose inhaled corticosteroids (ICS) , Step 3: Plus LABA, Step 4: Increase dose of ICS or add LTRA, SR theophylline or beta agonist tablet, Step 5: Daily steroid tablet and maintain ICS with specialist care.

Answer 249

E. Pneumonia - This patient has pneumonia. Symptoms include chills, fever, cough, SOB and pleuritic chest pain. Examination findings are consistent with his diagnosis. A CXR is the most specific and sensitive test available and antibiotics are indicated.

Answer 250

G. Pulmonary embolism - Patients with a high clinical suspicion of PE should be anticoagulated while waiting a definitive diagnosis unless contraindicated. The underlying pathophysiology is based on Virchow’s triad. SOB and chest pain are common symptoms and there may also be haemoptysis. Strong risk factors include DVT, obesity, surgery in the past 2 months, prolonged bed rest, malignancy, previous VTE, pregnancy and the thrombophilias such as factor V Leiden. ECG may be normal, or may show tachycardia, new RAD, new RBBB or the classical S wave in I, Q wave with T inversion in III. Various clinical probability scores exist for PE and D-dimer can be used to exclude PE as a diagnosis. The study of choice is a CTPA with direct visualisation of the thrombus. If there is a contraindication to a CT scan such as contrast allergy or pregnancy, then a V/Q scan is indicated. If a V/Q scan is not possible, alternatives such as MRA can be requested. It is worth noting that in patients with cardiopulmonary disease, these tests may not be accurate. A TTE can also be used to detect RV strain seen with PE.

Answer 251

F. Lung tumour - This is a smoker with respiratory complaints and unexplained weight loss pointing to malignancy. The hepatomegaly is likely to be nodular due to hepatic secondaries from a primary lung cancer and this hepatic dysfunction has caused this patient’s jaundice. First line treatment aims at surgical resection if possible. Small cell lung cancer is treated with chemotherapy and is associated with SIADH and ectopic ACTH. Non-small cell lung cancer is more often associated with clubbing. Squamous cell carcinoma is associated with PTHrp release and is treated with radiotherapy. Adenocarcinomas are usually located peripherally in the lung and are more common in non-smokers although most cases are still associated with smoking.

Answer 252

K. Sarcoidosis - Sarcoidosis is a chronic multisystem disease with an unknown aetiology. Lung involvement is very common. The strange red left shin is erythema nodosum and are tender erythematous nodules. Lupus pernio is another typical skin manifestation of sarcoidosis presenting with indurated plaques with discoloration on the face. CXR will typically show bilateral hilar lymphadenopathy and CXR findings are used in the staging of disease. Additionally, serum calcium and ACE levels may be raised. A transbronchial biopsy is essential for diagnosis in most cases and shows the presence of non-caseating granulomas. Black people have a higher lifetime risk of sarcoidosis, as do those of Scandinavian origin. The mainstay of treatment for severe disease involves systemic corticosteroids.

Answer 253

E. Asthma - This patient has signs and symptoms of asthma. Diagnosis is supported by PEFR variation of at least 20% over 3 days in a week over several weeks or an increase of at least 20% to treatment. Stepwise treatment is based on BTS guidelines: Step 1: SABA PRN, Step 2: Plus low-dose inhaled corticosteroids (ICS) , Step 3: Plus LABA, Step 4: Increase dose of ICS or add LTRA, SR theophylline or beta agonist tablet, Step 5: Daily steroid tablet and maintain ICS with specialist care.

Answer 254

F. Pneumonia - This is basal pneumonia which can present with upper abdominal pain. The symptoms this patient gives are consistent with pneumonia. Treatment is guided by the CURB-65 score. A CXR is the most specific and sensitive test available and antibiotics are indicated. CXR may show airspace shadowing with air bronchograms. Make sure you can spot consolidation on a CXR.

Answer 255

I. Lung cancer - Weight loss combined with worsening respirtory symptoms and the past history of smoking point to malignancy. First line treatment aims at surgical resection if possible. Small cell lung cancer is treated with chemotherapy and is associated with SIADH and ectopic ACTH. Non-small cell lung cancer is more often associated with clubbing. Squamous cell carcinoma is associated with PTHrp release and is treated with radiotherapy. Adenocarcinomas are usually located peripherally in the lung and are more common in non-smokers although most cases are still associated with smoking.

Answer 256

A. Cystic Fibrosis - This infant has presented with cystic fibrosis which is an autosomal recessive condition characterised by a mutation in the CFTR gene on chromosome 7 (delta-F508). In this case there is failure to thrive, recurrent chest infections and evidence of pancreatic insufficiency (steatorrhoea). The most conclusive diagnostic test is the sweat test which is pisitive if sweat chloride is >60mmol/L. Serum IRT from a heel prick blood spot allows screening of newborns. CF is a genetic condition with abnormal salt and water transport due to mutations in the CFTR (an apical anion channel). Heterozygotes generally do not demonstrate disease.

Answer 257

C. Tuberculosis - It is important to have a high level of suspicion when evaluating patients with risk factors who present with suggestive symptoms. Night sweats, fever, malaise, cough, haemoptysis and erythema nodosum are all suggestive. Other key risk factors for pulmonary TB include exposure to infection and returning from or being born in a high-risk region such as Asia, Africa and Latin America. If TB is suspected, the patient should be placed in isolation and a CXR obtained with 3 sputum samples cultured for AFB being the gold standard of diagnosis. Culture takes several weeks so sputum smears will be done before culture results are known. Interferon-gamma release assays (IGRAs) are now used by some hospitals to rapidly determine a patient’s TB status. All patients who have TB should be tested for HIV within 2 months of diagnosis.

Answer 258

K. V/Q scan - This patient has a PE. The study of choice is a CTPA with direct visualisation of the thrombus. If there is a contraindication to a CT scan such as contrast allergy (in this case) or pregnancy, then a V/Q scan is indicated. If a V/Q scan is not possible, alternatives such as MRA can be requested. It is worth noting that in patients with cardiopulmonary disease, these tests may not be accurate. A TTE can also be used to detect RV strain seen with PE. Patients with a high clinical suspicion of PE should be anticoagulated while waiting a definitive diagnosis unless contraindicated. The underlying pathophysiology is based on Virchow’s triad. SOB and chest pain are common symptoms and there may also be haemoptysis. Strong risk factors include DVT, obesity, surgery in the past 2 months, prolonged bed rest, malignancy (which this patient has), previous VTE, pregnancy and the thrombophilias such as factor V Leiden.

Answer 259

A. Sputum cultures - This sounds like TB. The patient should be placed in isolation and a CXR obtained with 3 sputum samples cultured for AFB being the gold standard of diagnosis. Treatment is with anti-TB medication: generally 2 months of rifampicin, isoniazid (given with pyridoxine to prevent associated neuropathy), pyrazinamide and ethambutol followed by 4 months of rifampicin and isoniazid alone. Treatment of MDR TB is more complicated and involves longer treatment with more drugs. Note that CNS TB requires 12 months of treatment.

Answer 260

C. History only - This is a result of intubation which has caused some iatrogenic trauma to this patient’s upper airway.

Answer 261

B. Bronchoscopy - This sounds like bronchial carcinoma from the history of smoking, respiratory complaints and weight loss. Initial investigation of bronchial carcinoma is with a CXR but definitive diagnosis relies on pathological confirmation from a tissue sample, often obtained from bronchoscopy. During bronchoscopy, endobronchial masses can be biopsied and washings/alveolar lavage can also be performed for cytological analysis. Trans-thoracic needle aspiration may be needed for peripheral lesions that cannot be reached by bronchoscopy. First line treatment aims at surgical resection if possible.

Answer 262

F. Chest x-ray - This is chest trauma which has obviously caused some damage. This will need to be visualised by performing a chest XR. A CT scan may also be necessary. There is a chance of atelectasis given this patient’s history and findings on examination.

Answer 263

L. Intramuscular adrenaline - There is a sudden onset of respiratory (and cardiovascular) complaints with the recently given aspirin tablet. This patient is having an anaphylactic reaction and the airway needs to be promptly secured and prompt treatment started with adrenaline. IM adrenaline must not be delayed and the anterolateral thigh is the preferred location, with repeated doses as necessary every 10 minutes or so. A 1:1000 solution is used of 0.3-0.5mg adrenaline. Fluid replacement with IV saline is also indicated to correct the intravascular volume redistribution.

Answer 264

F. Influenza immunisation - The GOLD guidelines published in April 2010 provide a framework for a stepwise approach to treating COPD. Stages of COPD are based on predicted FEV1. For all stages of COPD, influenza vaccination is given yearly and pneumococcal vaccine is given every 5 years. Long term oxygen is only added if there is chronic respiratory failure in stage IV disease. Stage II involves the addition of long acting bronchodilators and rehabilitation. Stage III involves adding inhaled GCs if there are repeated exacerbations.

Answer 265

B. 24% continuous oxygen - This patient with COPD is having an acute exacerbation. He is being treated accordingly but will need supplemental oxygen as a result of his ABG results. >8kPa is an acceptable level of arterial oxygenation or SaO2 >90%. Ceftriaxone is an acceptable antibiotic. High risk individuals should get tazocin or meropenem (especially if pseudomonas is suspected). Check local antibiotic prescribing policies. Chronic oxygen therapy is indicated for patients with PaO2

Answer 266

C. Long acting beta agonist - This question requires knowledge of stepwise BTS treatment guidelines for adults: Step 1: SABA PRN, Step 2: Plus low-dose inhaled corticosteroids (ICS) , Step 3: Plus LABA, Step 4: Increase dose of ICS or add LTRA, SR theophylline or beta agonist tablet, Step 5: Daily steroid tablet and maintain ICS with specialist care. This patient is still symptomatic on Step 2, so has to be moved up to Step 3. The rest of the question such as his HDM allergy is not particularly relevant in answering this question.

Answer 267

A. Oral prednisolone - Acute asthma exacerbation this time. Bear in mind that treatment guidelines differ for adults and children. The PEFR is 50% predicted making this a moderate exacerbation of asthma (40-69%). A mild exacerbation is defined by a PEFR of 70% of more of predicted whereas severe is defined as 26 to 39% with life-threatening falling under 25%. Note that these values are for children. For moderate exacerbations like this, an oral corticosteroid needs to be prescribed alongside inhaled SABA. If this were life-threatening or severe, then IV may be indicated. It is worth knowing the symptoms and signs of mild, moderate and severe/life threatening asthma. The treatment guidelines that you need to familiarise yourself with tend to be for adults.

Answer 268

C. COPD - Smoking is the most important risk factor, accounting for 90% of COPD. COPD has an insidious onset and usually presents in older people with a history of cough, wheeze and SOB. Patients with COPD are at a higher risk of infections and are vaccinated against influenza annually and pneumococcal pneumonia every 5 years. Spirometry is the gold standard for diagnosis, with FEV1/FVC ratio

Answer 269

E. Anaemia - Anaemia is defined by haemoglobin concentration (

Answer 270

B. Pneumonia - This patient has pneumonia. Symptoms include chills, fever, cough, SOB and pleuritic chest pain. Examination findings are consistent with his diagnosis. A CXR is the most specific and sensitive test available and antibiotics are indicated.

Answer 271

K. Aspirin poisoning - This patient has angina so probably has a stash of aspirin. Tinnitis is common in the early stages of acute salicylate poisoning and reflects CNS toxicity. There may also be deafness and both are reversible. GIT decontamination should be considered as an adjunct on arrival to A&E and activated charcoal can be given. The mainstay of treatment is alkaline diuresis induced by an infusion of sodium bicarbonate. In cases of severe poisoning, it is still started as a bridge to haemodialysis.

Answer 272

A. Mitral stenosis - Practically every single case of mitral stenosis is caused by rheumatic heart disease. The major criteria for rheumatic fever can be remember by CASES: carditis, arthritis, Sydenham’s chorea, erythema marginatum and subcutaneous nodules. The process tends to also cause regurgitation. Mitral stenosis is characteristically a grade 1-2 low pitch murmur heard in mid-diastole which has a rumbling nature and there is no radiation. There can be an associated malar flush, tapping apex beat and a diastolic thrill palpable at the apex, in the 5th intercostal space in the MCL. The first heart sound is also characteristically loud and often this is the most striking feature on ascultation. It is a difficult murmur to pick up so if you are ever asked at this stage to spot this murmur, it will most likely be based on the loud S1. Mitral stenosis is associated with AF caused by LA enlargement (seen by the irregularly irregular pulse).

Answer 273

H. Gastroscopy - Weight loss is one of the most common presenting symptoms in patients with gastric cancer. Epigastric pain is present in about 80% and may resemble that of a gastric ulcer. Although commonly mentioned in EMQs, lymphadenopathy is an uncommon presentation. On examination there may be the presence of a left supraclavicular node (Virchow’s node), a periumbilical nodule (Sister Mary Joseph’s nodule) or a left axillary nodule (Irish node). These are rare findings. A mass may be palpable in advanced disease. More proximal tumours can present with dysphagia. Acanthosis nigricans, a black velvety rash, may also be present. Strong risk factors include pernicious anaemia, Helicobacter pylori and the consumption of N-nitroso compounds found in cured meats. The peak incidence occurs between 50-70 and men are twice as likely to have gastric cancer. Most are adenocarcinomas. The first test to order for suspected gastric malignancy is an urgent upper GI endoscopy with biopsy of the lesion. The mainstay of treatment is surgical resection unless there is evidence of metastatic disease.

Answer 274

J. Serum Amylase - This patient has acute pancreatitis. He has vomited and is describing mid-epigastric pain radiating around to the back. This pain is classically relieved if the patient curls up and is worse with movement. This patient also vomited too. Complicated haemorrhagic pancreatitis may exhibit Cullen’s sign, Grey-Turner’s sign and Fox’s sign. Make sure you know what these are and you are familiar with the causes of acute pancreatitis. Those caused by hypocalcaemia may display Chvostek’s sign and Trousseau’s sign. Key to diagnosis is serum amylase or lipase levels which are massively elevated. Prognostic criteria are outlined in Ranson’s criteria applied on admission and after 48 hours, or the modified Glasgow score which you can find in your Oxford Handbook. An abdominal CT is however the most sensitive and specific study and findings may include enlargement of the pancreas with irregular contours, necrosis, pseudocysts and peripancreatic fat obliteration. For interest, urinary trypsinogen-2 is now considered a better screening test than amylase but is not currently clinically used.

Answer 275

A. History only - The intermittent diarrhoea, dyspepsia and bloating without symptoms suggestive of IBD make IBS a more likely diagnosis. IBS is a chronic condition with abdominal pain associated with bowel dysfunction and is a diagnosis of exclusion. The pain or discomfort may be relieved by defecation, as in this case. Examination is usually unremarkable and the diagnosis is based on the patient’s history in line with the Rome Criteria. If the patient presents with any worrying symptoms, then these will warrant a more thorough investigation. Treatment depends on the patient’s predominant symptoms. Antispasmodics relieve abdominal pain or discomfort but do not affect bowel habit. Examples include peppermint oil and dicycloverine. Laxatives can also be used such as lactulose. Lifestyle and dietary modifications combined with reassurance remain the 1st line intervention for functional bowel disease. IBS is linked with stressful jobs such as working as a secretary and there is a female/male ratio of 2:1.

Answer 276

C.Ultrasound abdomen - Cholecystitis is acute GB inflammation caused by an obstruction at the cystic duct. It occurs as a major complication of gallstones and classically presents with RUQ pain and fever. Gallstones in EMQs classically involves the Fs (Fat, Forty, Female, Fertile, Fair). USS is the definitive initial investigation. HIDA scanning and MRI may help if the diagnosis remains unclear. Treatment is with cholecystectomy. Make sure you know the difference between ascending cholangitis, cholecystitis and biliary colic.

Answer 277

E. Stool examination for pathogens - This patient has most likely has infectious diarrhoea. There is a short history in a previous well person. This man has probably eaten something dodgy like a kebab. Do you know the organisms that can cause bloody diarrhoea? Think of the organisms which cause bloody diarrhoea such as EHEC. The mainstay of treatment is rehydration and supportive therapy. Antibiotics may be indicated, particularly in severe cases. The diagnosis would be confirmed by stool examination.

Answer 278

F. Prolapse - Rectal prolapse tends to present as a mass protruding from the rectum, especially with straining on the toilet. This may be associated with mucous or blood-stained discharge, pain and even faecal incontinence. In contrast, haemorrhoids tend to present with painless PR bleeding or perianal pain with a tender mass in the area. This can be confirmed on examination. Tenesmus is the constant feeling of needing to pass stools, even if there is nothing to pass. It may also be a symptom of rectal carcinoma, and is caused by a SOL.

Answer 279

G. Fissure - This patient has an anal fissure. This causes severe pain on defecation and may continue for 1 to 2 hours. A small amount of fresh blood is often passed on the stool. Hard stools is a strong risk factor and this patient’s constipation will likely be the cause. Opiates are associated with constipation and subsequently anal fissures too and fissures may also occur in the third trimester of pregnancy or after delivery. Initial treatment is with topical GTN or diltiazem along with supportive measures such a high fibre diet. Resistant or chronic fissures may benefit from surgical measures or botulinum toxin.

Answer 280

E. Abscess - Typically, this presents with perianal pain. The location is important and affects management and diagnosis. If it is inter-sphincteric, then anaesthesia tends to be required to examine the rectal passage adequately for diagnosis – the pain tends to be so bad that a DRE is impossible otherwise. Abscesses found above levator ani may minic an abdominal condition. CT/MRI may be needed to establish the diagnosis. Risk factors include conditions like Crohn’s and anal fistulae (a complication of an abscess can also be a fistula). This accounts for the patient’s symptoms such as pruritis ani. A pilodinal abscess would be difficult to distinguish from an anorectal abscess on history alone but are usually located in the inter-gluteal region and often have a sinus tract in the midline. If the two are difficult to distinguish (such as if the patient cannot be examined), then MRI or CT of the pelvis may be used. Treatment involves drainage of the abscess surgically and the fistula, if present, can also be managed with a fistulotomy or seton insertion. Some patients will also get adjunctive antibiotics, such as the elderly and diabetics.

Answer 281

C. Haemorrhoids - Haemorrhoids are vascular rich cushions in the anal canal and presents, typically, as painless bright PR bleeding or with sudden onset pain in the area associated with a palpable mass. Pruritus ani is common and there is often perianal pain or discomfort. Diagnosis is made visually. Grade 1 is limited to within the anal canal. Grade 2 protrudes but spontaneously reduces when the patient stops straining. Grade 3 protrudes and reduces fully on manual pressure. Grade 4 is irreducible. Treatment includes fibre, ligation, photocoagulation, sclerotherapy or surgical haemorrhoidectomy. Haemorrhoidectomy is the treatment of choice of choice for patients with grade 4 haemorrhoids or for any patient who has failed with more conservative treatment such as sclerotherapy.

Answer 282

L. Carcinoma head of pancreas - Pancreatic cancer (of the head) typically presents with painless obstructive jaundice and weight loss and generally presents late. Whipple’s procedure or Traverso-Longmire procedure (pancreaticoduodenectomy) offers the only hope of a cure but only a small minority are elegible for these procedures. The first tests to order are an abdominal USS and LFTs. Note Courvoisier’s law: Jaundice and a palpable painless gallbladder is unlikely to be caused by gallstones. The tumour marker for pancreatic cancer is CA19-9 which is useful in preoperative staging.

Answer 283

K. Cirrhosis - This sales rep who has just lost his job has been hitting the bottle. The gynaecomastia is a sign of chronic liver disease and ascites indicates a degree of decompensation. Alcoholic liver disease is the most common cause of cirrhosis in the Western world.

Answer 284

I. Ascending cholangitis - Charcot’s triad of ascending cholangitis: fever with or without rigors, RUQ pain and jaundice. Cholangitis is infection of the biliary tree and can quickly become septic. Drainage of the biliary tree is crucial and is undertaken via ERCP. Make sure you know the difference between ascending cholangitis, cholecystitis and biliary colic.

Answer 285

B. Hepatits B - This could well be HCV although this is not an option. There are HBV risk factors in this patient including IVDU. Serum LFTs will shown a transaminitis. HBsAg, HBcAg and HBeAg can be measured. In HCV, HCV RNA can be measured. HAV is not associated with IVDU. Serum IgM anti-HAV can be measured.

Answer 286

I. Hirschsprung - In this condition there is colonic obstruction associated with absent intramural ganglion cells. The lumen is hence tonically contracted. Hirschsprung’s disease is commonly diagnosed in the first year of life and presents with vomiting, distension and/or colitis. There tends to be explosure liquid foul stools, delayed meconium passage and fever (enterocolitis). However, sometimes, it can present with intermittent bouts of symptoms later on in life and have minimal or absent symptoms in the first few days/weeks (adult presentations are rare). It can be associated with Down’s and MEN2A. The diagnosis is definitively made on a rectal biopsy with stain for ganglion cells in the submucosal plexus. This will be absent in Hirschsprung’s with the presence of other features such as thickened non-myelinated nerves and increased acetylcholinesterase. A contrast enema will also be done and will show a contracted distal bowel with the proximal bowel dilated, making the location of the transition zone visible on XR. An AXR is always the first investigation performed but a normal film does not exclude the possibility of this diagnosis (it is a non-specific investigation, though having said that, if there are no distended bowel loops then Hirschsprung’s is unlikely). Manometry is not usually performed, but the reflex where when the rectum is distended, pressure in the anal canal falls (as the internal sphincter relaxes) is absent. Initial treatment is with irrigation followed by surgery.

Answer 287

E. Hypothyroidism - This patient has hypothyroidism. Worldwide, the most common cause is iodine deficiency. Other causes include Hashimoto’s or secondary and tertiary hypothyroidism. It can also result from viral de Quervain’s thyroiditis or postpartum thyroiditis. Symptoms include those mentioned (depression, fatigue, weight gain, bradycardia and sluggish reflexes) as well as others such as constipation, cold intolerance, menstrual problems in females, dry skin and muscle cramps. Diagnosis is based on measurement of TSH and thyroid hormones. Treatment is by replacement of T4 with or without T3 in combination. If the patient has normal T3 and T4 but mildly elevated TSH, this is described as subclinical hypothyroidism.

Answer 288

G. Irritable bowel syndrome - IBS is a chronic condition with abdominal pain associated with bowel dysfunction and is a diagnosis of exclusion. The pain or discomfort may be relieved by defecation. Examination is usually unremarkable and the diagnosis is based on the patient’s history in line with the Rome Criteria. If the patient presents with any worrying symptoms, then these will warrant a more thorough investigation. Treatment depends on the patient’s predominant symptoms. Antispasmodics relieve abdominal pain or discomfort but do not affect bowel habit. Examples include peppermint oil and dicycloverine. Laxatives can also be used such as lactulose. Lifestyle and dietary modifications combined with reassurance remain the 1st line intervention for functional bowel disease. IBS is linked with stressful jobs and there is a female/male ratio of 2:1.

Answer 289

J. Adverse effect of drugs - This patient is on opiates. Let us review opiates such as morphine and codeine. They are commonly prescribed for analgesia and can also be used as an anti-tussive (suppresses ACh and NK release needed for cough activation and inteferes with serotonin receptors in the DRN). They are also abused for their euphoric effects mediated by their action on dopamine release at the nucleus accumbens. They have a host of side effects, including, in this case, GI effects (receptors present on the myenteric and submucosal plexus) of decreased gastric motility, emptying and increased gut water reabsorption... in short, it causes constipation. Other side effects include respiratory depression (desensitises central chemoreceptors to lessen the response to PaCO2), N&V by stimulating the CTZ, pupillary constriction via the Edinger-Westphal nucleus and causes symptoms like pruritis, urticaria, hypotension through histamine release via a direct effect on mast cells. Arrestins also promote internalisation of opioid receptors which leads to tolerance with prolonged treatment. Prolonged use will also lead to dependance and withdrawl effects if stopped, such as cravings, and the physical effects may be very profound, resembling flu. Naloxone is indicated in an overdose. It is worth looking at the pathways to help you remember the effects.

Answer 290

C. Colorectal carcinoma - This a rectal carcinoma. Tenesmus, blood and mucus PR alongside weight loss and anorexia are all highly suggestive. Treatment of rectal carcinoma involves surgical excision where possible. This can either be an anterior resection (tumours in the upper 1/3 of the rectum) or an abdominoperineal resection (if the tumour lies lower down). APER involves the formation of a permanent colostomy and has a high incidence of sexual and urinary dysfunction. Anterior resection involves a colo-anal anastamosis.

Answer 291

B. Hypercalcaemia - Symptoms of high calcium include confusion, constipation, polyuria, polydipsia, depression, kidney stones and lethargy. This can be remembered by ‘stones, bones, abdominal groans and psychiatric moans’. 90% of hypercalcaemia is caused by primary hyperparathyroidism or cancer. Malignancy can cause hypercalcaemia either by direct bony involvement leading to osteolytic lesions or paraneoplastic syndromes involving PTHrp release. The tumour is typically very advanced if hypercalcaemia is a feature. Less common causes include vitamin D overdose, hyperthyroidism, immobilisation, Paget’s and milk-alkali syndrome. The classic bone disease in hyperparathyroidism is osteitis fibrosa cystica which causes pain. The serum PTH level is elevated in primary hyperparathyroidism whereas it may be very low in malignancy due to negative feedback.

Answer 292

F. Cryptospradium - I’m sure at some point most of you will see ‘diarrhoea’ and ‘HIV’ together and jump straight to Cryptosporidium. This is a disease caused by a protozoa and diagnosis is made in the lab by detection of oocysts or antigens in stool. The presentation is of watery diarrhoea, often accompanied with severe pain in the tummy, often lasting more than 7 days. It is self-limiting if the patient is immunocompetent but those who are immunocompromised can suffer a chronic sveere course. Those most at risk are those with T cell deficiencies, such as HIV, and those with haematological malignancies, especially children. In immunocompetent people, nitazoxanide can be used in treatment (as can paramomycin). In immunocompromised patients, treatment mainly aims at treating the primary disorder, such as using HAART to improve CD4 cell count and to restore immunity. Protease inhibitors such as ritanovir also act directly to reduce host cell invasion by the sporozoites and reduce parasite development.

Answer 293

B. Staphylococcus aureus - This patient has infectious vomiting predominant food poisoning. When vomiting is the main presenting symptom, you should be thinking of Staphylococcus aureus, Bacillus cereus or norovirus. There is a short history in a previous well person. This man has probably eaten something dodgy like a kebab with undercooked chicken, or something like that. The mainstay of treatment is rehydration and supportive therapy. Antibiotics may be indicated, particularly in severe cases.

Answer 294

H. Ulcerative colitis - While this could be Crohn’s disease, bloody diarrhoea is more commonly a presentation of UC than Crohn’s. UC is characterised by diffuse mucosal inflammation running a relapsing and remitting course. Bloody diarrhoea is commonly experienced by patients who may also complain of other symptoms such as (lower) abdominal pain, faecal urgency and the host of extra-intestinal manifestations associated with UC. Diagnosis of UC requires endoscopy with biopsy and a negative stool culture to rule out infectious gastroenteritis. Flare ups are usually linked to pathogens so a stool culture will always be needed in these cases. Toxic megacolon is a complication which is associated with a risk of perforation. UC is also linked with bowel adenocarinoma and PSC. Treatment involves mesalazine (5-ASA) used to induce and maintain remission.

Answer 295

C. Chronic pancreatitis - This is chronic pancreatitis which is most commonly associated with chronic alcohol abuse. Features include the epigastric pain here, which classically radiates to the back, and steatorrhoea from malabsorption, described here with the pale, foul-smelling and difficult to flush stools. There may additionally be DM due to pancreatic failure and the patient may be malnourished. The diagnosis is based on findings and imaging – your options are USS which is less sensitive, or CT, which is more sensitive but involves radiation exposure. AXR is not a sensitive enough test. There is no real definitive treatment, which is mainly symptomatic and the underlying and precipitating factors are treated – in this case, this man’s alcohol excess. Complications of chronic pancreatic imflammation include the development of pseudocysts, calficiation, DM and malabsorption.

Answer 296

E. Villous adenoma of the rectum - Do not be fooled by the bendrofluazide, which is taken by many patients, but does not by itself lead to such profound hypokalaemia (but I’m sure contributes), and in any case would not account for the GI symptoms experienced. This patient has a villous adenoma, which is a type of polyp in the GIT with a malignant potential. Most colorectal cancers arise from an adenoma and polypectomy reduces the incidence of colorectal cancer. The non-neoplastic polyps include hyperplastic ones, hamartomas, inflammatory and lymphoid polyps. Villous adenomas secrete large amounts of mucus and result in hypokalaemia. Adenomatous polyps are increasingly common with age. FAP and Gardner’s syndrome, for example, predispose to adenomatous polyps (Peutz-Jeghers leads to hamartomatous polyps). This patient is going to need a colonoscopy with biopsy histology to see if the polyp is benign or malignant.

Answer 297

K. Campylobacter - So why is this Campylobacter? Well, it doesn’t have to be. Salmonella, E. Coli, Shigella, Listeria, Vibrio species etc all present with symptoms which are not drastically different and the only way to be sure is to do a stool culture. The only real option here are between Campylobacter, Staphylococcus aureus and Clostridium difficile. However, this patient does not have a history of recent antibiotic use. Staphylococcus tends to present with vomiting as the main feature and the watery diarrhoea here is typical of Campylobacter. UC and CD are chronic conditions (it is worth noting that Yersinia enterocolitis can mimic Crohn’s RLQ pain). This person has most likely eaten something dodgy on holiday. Erythromycin can be used effectively if started early but resistance is a problem and only a small number will benefit. Campylobacter jejuni is the main cause of food poisoning (also coli and fetus species cause disease). Diarrhoea normally resolves in 5-7 days and the patient will need fluid/electrolyte replacement. Campylbacter is one of the infections which is commonly linked to Guillain-Barre (although still a rare phenomenon).

Answer 298

B. Mallory-Weiss Syndrome - This occurs after a rise in abdominal pressure which induces a tear in the oesophageal mucosa, causing subsequent GI bleeding. It commonly presents with haematemesis after an episode of retching/vomiting/coughing/straining. Hence, risk factors include anything which can cause vomiting like heavy alcohol use, which is commonly the case in EMQs. Also, other conditions would include food poisoning, bowel obstruction, hyperemesis gravidarum, bulimia, the chronic cough of COPD, meningitis etc... you name it really. Classically, MWT presents with a small self limiting episode of haematemesis. Definitive diagnosis is made by OGD. Treatment is supportive because most cases, as mentioned, are self limiting and emergency treatment is not offered unless the patient is showing signs of clinical instability. If the patient is actively bleeding, treatment will be with therapeutic endoscopy in most cases, and very very few cases will require more intervention such as angiography with embolisation.

Answer 299

B. Mallory-Weiss Syndrome - This occurs after a rise in abdominal pressure which induces a tear in the oesophageal mucosa, causing subsequent GI bleeding. It commonly presents with haematemesis after an episode of retching/vomiting/coughing/straining. Hence, risk factors include anything which can cause vomiting like heavy alcohol use, which is commonly the case in EMQs. Also, other conditions would include food poisoning, bowel obstruction, hyperemesis gravidarum, bulimia, the chronic cough of COPD, meningitis etc... you name it really. Classically, MWT presents with a small self limiting episode of haematemesis. Definitive diagnosis is made by OGD. Treatment is supportive because most cases, as mentioned, are self limiting and emergency treatment is not offered unless the patient is showing signs of clinical instability. If the patient is actively bleeding, treatment will be with therapeutic endoscopy in most cases, and very very few cases will require more intervention such as angiography with embolisation.

Answer 300

A. Peptic ulcer - This patient has a bleeding peptic ulcer (the black tarry stools and the coffee-ground vomit from the UGI bleed). Epigastric pain and tenderness related to eating a meal is typical of a peptic ulcer. 80% are duodenal and 20% are gastric. Ulcers may cause iron deficiency anaemia and associated symptoms may feature. Key risk factors are NSAID use, like in this patient, H. pylori infection, smoking and a family history of PUD. Zollinger-Ellison syndrome should be considered if there are multiple ulcers or ulcers refractory to treatment. Gastric ulcers classically cause pain which is exacerbated by eating and immediately relieved on vomiting. There is usually also weight loss due to a fear of food and its association with pain. Duodenal ulcers are classically made worse by hunger and are relieved by eating and the patient may wake at night with the pain. As a result, weight gain is typically a feature. In reality, it is difficult to differentiate the site of the ulcer based on these features. The most specific and sensitive test is an upper GI endoscopy which is initially ordered if the patient has ‘red flag’ symptoms, is >55 years of age or fails to respond to treatment. Duodenal ulcers rarely undergo malignant transformation so do not require a compulsory biopsy but gastric ulcers require biopsies to rule this out. In patients who are 55 or younger without ‘red flags’, testing for Helicobacter pylori (breath testing with radiolabelled urea or stool antigen testing) is necessary. Management is aimed at correcting the underlying cause such as discontinuing NSAIDs. H. pylori eradication should be started if the organism is present with triple therapy. Otherwise, a PPI is indicated.

Answer 301

E. Oesophageal varices - Oesophageal varices occurs as a result of portal hypertension which is a complication of cirrhosis, caused in this patient by his long history of alcohol excess. Other signs may be present such as spider naevi, ascites, caput medusa (vascular collaterals in the abdominal wall), jaundice etc. Splenomegaly is also commonly found and hence patents often have thrombocytopenia and anaemia as a result. The bleeding carries a significant morbidity and mortality, and beta-blockers and/or endoscopic ligation can prevent variceal bleeding prophylactically (though beta blockers are not be used in the acute setting of a variceal bleed – do not get confused here!). Oesophageal varices are basically dilated veins and these can be seen on OGD. Worldwide, HBV and HCV are also major causes of cirrhosis, leading to varices and HIV co-infection can rapidly speed up the progression to cirrhosis in chronic liver failure. The size of the varices is the key predictor of haemorrhage. Acute bleed can be managed with resuscitation, terlipression (DDAVP)/somatostatin analogues/endoscopic ligation. Additionally, a shunt can be deployed and antbiotic prophylaxis started.

Answer 302

I. Osler-Weber-Rendu synrome - This woman has the facial telangiectasia of OWR, also called hereditary haemorrhagic telangiectasia. This causes abnormal blod vessels pretty much everywhere which are prone to bleed. It is an autosomal dominant condition so a positive FH can often be found.

Answer 303

D. Liver cirrhosis - Cirrhosis is the end-stage of chronic liver disease, in this case due to alcoholic liver disease. Cirrhosis results in hepatic insufficiency and portal hypertension. This has resulted in the patient’s ascites which is a symptom of decompensated cirrhosis. Other complications include variceal bleeds, jaundice, hepatic encephalopathy, hepatorenal syndrome and the development of HCC. Palmar erythema affects the thenar and hypothenar eminences. Apart from spider naevi and palmar erythema, other signs you might find include telangiectasia, bruising, gynaecomastia, Dupuytren’s contracture, parotid swelling and a red tongue. The patient will have to undergo a diagnostic paracentesis for the ascitic presentation. The treatment of ascites involves restricting salt intake and the use of diuretics (frusemide and spironolactone).

Answer 304

K. Pancreatic pseudocyst - This patient has developed a pancreatic psuedocyst as a complication of pancreatitis. Pseudocysts are collections of fluids with a high concentration of enzymes. The walls are fibrotic membranes of the peritoneum, mesentery and serosa which stops the fluid from leaking out. The wall is not epithelium and indeed there is no epithelial lining – it is not a real cyst. In patients who fail to respond to treatment, this should be considered as a possible diagnosis. The most common finding is pain, followed by a palpable mass. CT scan is diagnostic. Treatment options include excision, drainage (surgical or percutaneous, or internal e.g. cystojejunostomy Roux-en-Y etc, which I’m sure is going into too much surgery than is necessary now but surgical wannabes can look up the procedures... if they really want to – also cystogastrostomy and cystoduodenostomy). The pseudocyst can be complicated with infection, rupture and haemorrhage. Pancreatic abscess would give a fever and CT will show a ring-enhancing fluid collection with gas. Treatment would be drainage and antibiotics. Pancreatic ascites is pancreatic fluid accumulating in the peritoneal cavity due to chronic pseudocyst leakage (in most cases, anyway) – there will be weight loss and the ascites will not respond to diuretics. A pancreatic effusion is secondary to a fistula draining into the chest, from the pancreas. These are actually all more common complications to arise from pancreatitis than a pseudocyst.

Answer 305

L. Divaricaction of the recti - Divaricate means to spread apart. The rectus goes from the pubic crest, tubercle and symphysis to the costal cartilages 5,6 and 7, costal margin of 7, sternum and diaphragm. It is innervated by T7-12. Normally, the rectus muscles meet in the midline (linea alba). However, some people have a defect above the umbilicus which causes the gap between the recti to be wider than normal. Hence, when the patient sites up, the rectus muscles will spread apart. Surgica correction is possible but most are asymptomatic. It is not a true hernia, and this is the only option from the list that fits the presentation.

Answer 306

D. Splenic rupture - Infectious mononucleosis caused by EBV infection causes splenomegaly. This makes the patient susceptible to splenic rupture due to trauma, such as during this rugby practice session. Rupture is a cause of splenomegaly, and splenomegaly is a risk factor for rupture. If the patient is previously known to have mono, with an enlarged spleen, then they should really avoid contact sports. It would be an irresponsible doctor to not advise against this. This is a medical emergency as the spleen is a very vascular organ and bleeding can rapidly lead to shock and death.

Answer 307

C. Sigmoid volvulus - While this woman has indeed presented with very non-specific symptoms, the only feasible answer from the list is a sigmoid volvulus. A volvulus is bowel obstruction occuring due to a loop of bowel twisting on its own mesenteric axis. Broadly speaking, there are three types: small bowel, sigmoid and gastric. This is something you need to be able to recognise on AXR and it appears as a dilated loop of large bowel present in the lower abdomen, resembling a coffee bean shape (or like an upside down U shape). The rest of the bowel is usually dilated. For a caecal volvulus, the caecum leaves the RLQ to appear like a second satomach bubble in the centre of the film. There is often associated small bowel dilation. A gastric volvulus is very rare.

Answer 308

J. Oesophageal varices - Oesophageal varices occurs as a result of portal hypertension which is a complication of cirrhosis, caused in this patient by his long history of alcohol excess. Other signs may be present such as spider naevi, ascites, caput medusa (vascular collaterals in the abdominal wall), jaundice etc. Splenomegaly is also commonly found and hence patents often have thrombocytopenia and anaemia as a result. The bleeding (explaining the BP and HR of this patient) carries a significant morbidity and mortality, and beta-blockers and/or endoscopic ligation can prevent variceal bleeding prophylactically (though beta blockers are not be used in the acute setting of a variceal bleed – do not get confused here!). Oesophageal varices are basically dilated veins and these can be seen on OGD. Worldwide, HBV and HCV are also major causes of cirrhosis, leading to varices and HIV co-infection can rapidly speed up the progression to cirrhosis in chronic liver failure. The size of the varices is the key predictor of haemorrhage. Acute bleed can be managed with resuscitation, terlipression (DDAVP)/somatostatin analogues/endoscopic ligation. Additionally, a shunt can be deployed and antbiotic prophylaxis started.

Answer 309

I. Bowel obstruction - Bowel obstruction has several causes such as adhesions or cancer. The lack of bowel motions, vomiting, distended abdomen and tinkling bowel signs are all indicative. The proximal segment of bowel dilates and distal bowel collapses. Completely obstructed patients generally require surgery. If, on AXR, air is seen to be seeping past the obstruction then the obstruction is partial. As a standard, all patients should be made NBM and given supplemental oxygen, IV fluids and NG decompression (to reduce flow/gastric contents/air towards the obstruction), unless they are rushed off for an emergency laparotomy because, for example, they have complete SBO and are peritonitic.

Answer 310

H. Uraemia - This patient has developed acute renal failure, probably associated with the longstanding CCF. Advanced heart failure will lead to depressed renal perfusion and ARF. The decreased urine output is a symptom and the vomiting here is caused by uraemia or a general build up of waste products. An acute increase in creatinine will be seen, commonly with hyperkalaemia, hyperphosphataemia and a metabolic acidosis. There may also be respiratory compensation for this. Treatment is largely supportive, managing, in this case, the heart failure, and correcting abnormalities like volume status and the metabolic acidosis. Dialysis may be required.

Answer 311

K. Pancreatitis - This patient has acute pancreatitis. He has vomited and is describing mid-epigastric pain radiating around to the back. This patient can also have nausea and vomiting too, with agitation and confusion. A pleural effusion is seen in half of patients with acute pancreatitis. Complicated haemorrhagic pancreatitis may exhibit Cullen’s sign, Grey-Turner’s sign and Fox’s sign. Make sure you know what these are and you are familiar with the causes of acute pancreatitis (GET SMASHED). Those caused by hypocalcaemia may display Chvostek’s sign and Trousseau’s sign. Key to diagnosis is serum amylase or lipase levels which are massively elevated. Prognostic criteria are outlined in Ranson’s criteria applied on admission and after 48 hours, or the modified Glasgow score which you can find in your Oxford Handbook. An abdominal CT is however the most sensitive and specific study and findings may include enlargement of the pancreas with irregular contours, necrosis, pseudocysts and peripancreatic fat obliteration. For interest, urinary trypsinogen-2 is now considered a better screening test than amylase but is not currently clinically used.

Answer 312

A. Salmonella - The only option on the list which fits is Salmonella. This could obviously be E coli or Campylobacter too, for instance. This is infectious gastroenteritis and Salmonella is a common cause, pretty much able to contaminate any food. It is commonly linked to poultry, dairy items and undercooked eggs. It is self-limiting and diagnosis is on isolating the organism from a stool culture. Treatment is supportive with fluid and electrolyte replacement and antibiotics are generally used only for patients with risk factors for severe disease or those with extra-GI complications.

Answer 313

B. Viral gastroenteritis - Viral gastroenteritis often presents with mainly UGI symptoms like N&V more so than diarrhoea. It is on this basis that this is likely to be viral gastroenteritis caused by organisms such as rotavirus, norovirus and adenovirus. Staphylococcus aureus and Bacillus cereus also tend to cause mostly UGI symptoms and acts by preformed toxins, though these are not on the list of available options. The differentiation is made on studying the stool and identifying the organism.

Answer 314

E. Malaria - In the Western world, almost all cases of malaria occurs in travellers so an adequate travel history is crucial or the diagnosis may be missed. Patients typically present with non-specific symptoms such as a fever, sweats, chills and myalgia. This woman has just returned from an endemic area. Sometimes EMQs will describe patterns of fevers occuring at regular intervals of 48-72 hours associated with P. vivax, P. ovale and P. malariae infections but in most patients there is no specific pattern. Hepatosplenomegaly is a common presenting sign although not common at presentation in a first world setting. Thrombocytopenia is common with falciparum infection and a mild degree of anaemia are commonly seen. WCC can be high, low or normal. Pregnant women affected by P. falciparum are susceptible to the complications of pregnancy due to placental parasite sequestration. Treatment of malaria in pregnancy must be managed with an ID specialist and should be treated with IV antimalarial therapy. The test of choice is Giesma-stained thick and thin blood smears. Thick films sensitively detect parasites whereas thin films allow species identification and calculation of parasitaemia to guide treatment. Studies have shown that for P falciparum, the most effective treatment is artesunate which is more effective than quinine without the risk of cinchonism. Numerous studies such as the AQUAMAT study in The Lancet showing that quinine should no longer be the established treatment of choice.

Answer 315

K. Polycythaemia rubra vera - PRV is a disease of middle and older age and is strongly associated with the JAK2V617 mutation. Pruritis is a common feature and is often severe and evoked by contact with water. Facial redness and fullness is commonly observed and splenomegaly is a common finding. It is a myeloproliferative disorder with raised Hct, Hb and RBC count. Blood hence becomes very viscous. There is a clear link between Budd-Chiari syndrome and subsequent PRV. Treatment is with venesection. Around 30% will go on to develop myelofibrosis.

Answer 316

F. CLL - This elderly woman has CLL. CLL presents in older adults (generally >60) and is often asymptomatic. Smear cells can be seen in peripheral blood smear and it is associated with a warm type AIHA accounting for her pallor and fatigue (hence the Coombs’ test is positive). Painless lymphadenopathy may be present and splenomegaly is a common finding. A WCC with differential is required to make a diagnosis. An absolute lymphocytosis will be seen. CML is not associated with an AIHA and tends to present at a younger age.

Answer 317

L. COngestive heart failure - The significant ankle oedema and raised JVP point towards right sided heart failure.The presence of 1-2 spider naevi is completely normal. Initial investigations should include ECG, CXR, TTE and bloods including BNP levels. First line treatment is with an ACE inhibitor which reduces morbidity and mortality. Salt and fluid restriction is also beneficial. All patients with chronic heart failure will also receive a beta blocker such as carvedilol. Other adjuncts include spironolactone, diuretics, hydralazine and a nitrate, and digoxin.

Answer 318

J. Cirrhosis with hepatoma - HCV has caused this patient’s hepatic cirrhosis which has resulted in a hepatoma (HCC). It is likely that his HCV infection has resulted from contaminated blood products due to his haemophilia. This was a key problem before blood donor screening took place. Unlike HBV, HCV infection almost always results in cirrhosis before a hepatoma develops. The length of time the patient has HCV is a good correlate to the development of HCC. HCV is also strongly associated with IVDU which accounts for most infections. Treatment of the hepatoma is guided by staging and prognosis. Treatment includes resection, transplant, percutaneous ablation and chemo-embolisation.

Answer 319

N. Severe emphysema - The liver is palpable in this man because severe emphysema has resulted in hyperexpanded lung fields.

Answer 320

E. Irritable bowel syndrome - IBS is a chronic condition with abdominal pain associated with bowel dysfunction and is a diagnosis of exclusion. The pain or discomfort may be relieved by defecation. Examination is usually unremarkable and the diagnosis is based on the patient’s history in line with the Rome Criteria. If the patient presents with any worrying symptoms, then these will warrant a more thorough investigation. Treatment depends on the patient’s predominant symptoms. Antispasmodics relieve abdominal pain or discomfort but do not affect bowel habit. Examples include peppermint oil and dicycloverine. Laxatives can also be used such as lactulose. Lifestyle and dietary modifications combined with reassurance remain the 1st line intervention for functional bowel disease. IBS is linked with stressful jobs such as working as a secretary and there is a female/male ratio of 2:1. ‘Pellet-like’ stools is an indicator in EMQs.

Answer 321

C. Colorectal cancer - This a rectal carcinoma. Tenesmus, blood and mucus PR alongside weight loss and anorexia are all highly suggestive. Treatment of rectal carcinoma involves surgical excision where possible. This can either be an anterior resection (tumours in the upper 1/3 of the rectum) or an abdominoperineal resection (if the tumour lies lower down). APER involves the formation of a permanent colostomy and has a high incidence of sexual and urinary dysfunction. Anterior resection involves a colo-anal anastamosis.

Answer 322

H. Inflammatory bowel disease - This patient gives a history of IBD. The history would be more suggestive of UC where the mainstay of treatment is with 5-ASA. A colonoscopy is required to assess the extent of disease and for a definitive diagnosis. Biopsy in CD will show transmural granulomatous inflammation. CD can affect the whole GIT but favours the TI and proximal colon and is macroscopically characterised by skip lesions. UC on the other hand is characterised by the presence of crypt abscesses, which is pathognomic. CD risk is increased 3-4 fold by smoking whereas smoking seems protective in UC. The mainstay of treatment in CD is with steroids and azathioprine to revent relapses and for those suffering side effects of steroid treatment. TNF-alpha inhibitors also have a role. Surgery in CD is only indicated in a small number of patients who bleed, for bowel perforation and cases of complete obstruction. The aim is to rest distal disease by temporarily diverting faecal flow.

Answer 323

B. Infectious diarrhoea - This patient has low grade pyrexia and recent foreign travel making infectious diarrhoea very likely. This man has probably eaten something dodgy while on holiday in India. Think of the organisms which cause bloody diarrhoea such as EHEC. E coli is the most common cause of traveller’s diarrhoea. There may be a contact history due to faecal-oral spread. The mainstay of treatment is rehydration and supportive therapy. Antibiotics may be indicated, particularly in severe ETEC.

Answer 324

I. Ischaemic collitis - Ischaemic colitis causes focal or diffuse abdominal pain and often has a more insidious onset than mesenteric ischaemia (over several hours or days). The recent operation in the approximate area has resulted in an incomplete blood supply to that part of the bowel. Mesenteric ischaemia and ischaemic colitis all form part of ‘ischaemic bowel disease’.

Answer 325

B. Heart failure - This patient has heart failure, which has possibly occured as a consequence of his MI. SOB indicates pulmonary oedema due to LV failure. The raised JVP, peripheral oedema and tender hepatomegaly indicates RV failure. Initial investigations should include ECG, CXR, TTE and bloods including BNP levels. First line treatment is with an ACE inhibitor which reduces morbidity and mortality. Salt and fluid restriction is also beneficial. All patients with chronic heart failure will also receive a beta blocker such as carvedilol. Other adjuncts include spironolactone, diuretics, hydralazine and a nitrate, and digoxin.

Answer 326

K. Carcinoma of the caecum with peritoneal secondaries - The weight loss of 10kg, fatigue and non-tender irregular RIF mass point to caecal carcinoma. Right sided colorectal cancer tends to present with anaemic symptoms. Almost 90% are anaemic at diagnosis. The progressive abdominal distension indicates the presence of peritoneal secondaries, which causes vague symptoms. Treatment in this case for a cancer that has become widely metastatic will be palliative.

Answer 327

J. Nephrotic syndrome - Nephrotic syndrome is the triad of proteinuria (>3.5g/24h), hypoalbuminaemia (

Answer 328

E. Hiatus hernia - A hiatus hernia is where intraabdominal contents protrude through the oesophageal hiatus of the diaphragm. Risk factors inclyde obesity and high intra-abdominal pressure. The condition may be asymptomatic, or it may present with symptoms (which are non-specific) such as heartburn, dysphagia, pain on swallowing, wheezing, hoarseness and chest pain. A CXR is the first test done and may show an air bubble in the wrong place but barium studies are diagnostic and treatment depends on the symptoms and anatomy of the hernia. Hernias can be sliding or rolling (or mixed, or giant), uncomplicated or complicated by, for instance, obstruction and bleeding. Do you know the difference between a sliding and a rolling hiatal hernia?

Answer 329

B. Carcinoma of oesophagus - Dysphagia (in this progressive pattern) coupled with weight loss points to malignancy. Dysphagia occurs when there is obstruction of more than 2/3 of the lumen and presence indicates locally advanced disease. There may additionally be odynophagia. Men are twice as likely to develop oesophageal cancer. GORD, Barrett’s oesophagus, FH, tobacco and alcohol are all risk factors. The two main types are squamous cell carcinoma and adenocarcinoma. Tumours in the upper 2/3 of the oesophagus are SCC whereas those that lie in the lower 1/3 are adenocarcinomas. The main test to order is an OGD with biopsy. Treatment is either surgical resection or with chemo or radiotherapy alongside endoscopic ablation with or without stenting and brachytherapy.

Answer 330

C. Plummer-Vinson syndrome - Plummer-Vinson syndrome is the association of chronic Iron Deficiency Anaemia (IDA) (shown here by the koilonychia and paleness on examination) with dysphagia due to a post cricoid web. Roughly 7% of those with IDA may complain of gradual onset dysphagia with the discomfort found in the area of the cricoid cartilage. Invasive procedures may be needed for management such as endoscopic dilation of the web but treatment is largely aimed at correcting the IDA.

Answer 331

G. Myesthenia gravis - Myasthenia gravis is an autoimmune condition with antibodies affecting the NMJ, mostly the nAChR at the post-synaptic muscle membrane. Although some have antibodies against MuSK, and there are other proteins involved. MG is characterised by muscle weakness which increases with exercise (fatigue, demonstrated here as the dysphagia gets worse as he eats, unlike Lambert-Eaton myasthenic syndrome). Commonly, presentations include diplopia and drooping eyelids like this patient, and there may also be SOB, proximal limb weakness, facial paresis and oropharyngeal weakness. MG is associated with thymic hyperplasia in 70% or thymoma in 10%, and these associations can also crop up in EMQs. There will be elevated serum AChR receptor antibody titres or MuSK antibodies. Electrophysiology will demonstrate a decremental response on repetitive nerve stimulation. Treatment includes anticholinesterases (pyridostigmine, and immunotherapy. Patients may also require a thymectomy. Some 15-20% may experience a myasthenic crisis (which needs mechanical ventilation). Do you know what the Tensilon test is and why edrophonium is given in this test?

Answer 332

K. Achalasia - This is achalasia which is a motility disorder with loss of peristalsis in the distal oesophagus and failure of the LOS to relax in response to swallowing. This presents commonly with dysphagia to both liquids and solids, regurgitation and retrosternal chest pain, which can be slowly progressive over time. In structural obstruction such as cancer, dysphagia to liquids is uncommon unless the disease is very advanced. Retrosternal pressure experienced can be precipitated by drinking liquids but is eased by continuing to drink, and the pain may be relieved by cold water. This may wake the individual from sleep. A UGI endoscopy is needed to exclude malignancy as a cause of dysphagia. The diagnosis is established on manometry or barium studies. Treatment is symptomatic.

Answer 333

M. Primary biliary cirrhosis - Primary biliary cirrhosis (PBC) is a chronic condition where the intrahepatic small bile ducts are progressively damaged (and eventually lost) occuring on a background of portal tract inflammation. Fibrosis develops, ultimately leading to cirrhosis (which is defined as fibrosis with nodular regeneration). It is widely believed to be autoimmune in aetiology as almost all patients have AMA. The pointers in this question which would raise your suspicion, is xanthelasma around the eyes, pruritis in the absence of an obvious dermatological cause, fatigue and the features of liver disease typical of cirrhosis (jaundice) and splenomegaly as a feature of portal hypertension. The patient also has dry eyes and dry mouth from associated Sjogren’s syndrome. The joint pains could indicate RA.

Answer 334

F. Liver cirrhosis - Cirrhosis is the end-stage of chronic liver disease, resulting in hepatic insufficiency and portal hypertension. This has resulted in this man’s jaundice. Palmar erythema affects the thenar and hypothenar eminences. Other signs include spider naevi, telangiectasia, Dupuytren’s contracture, parotid swelling, leuconychia from hypoalbuminaemia, gynaecomastia and bruising. Management is aimed at treating the underlying liver disease. The only curative option, once decompensated, is liver transplantation.

Answer 335

K. Inflammatory bowel disease - This patient gives a history of IBD. The history would be more suggestive of UC (but could be CD) where the mainstay of treatment is with 5-ASA. A colonoscopy is required to assess the extent of disease and for a definitive diagnosis. Biopsy in CD will show transmural granulomatous inflammation. CD can affect the whole GIT but favours the TI and proximal colon and is macroscopically characterised by skip lesions. UC on the other hand is characterised by the presence of crypt abscesses, which is pathognomic. CD risk is increased 3-4 fold by smoking whereas smoking seems protective in UC. The mainstay of treatment in CD is with steroids and azathioprine to revent relapses and for those suffering side effects of steroid treatment. TNF-alpha inhibitors also have a role. Surgery in CD is only indicated in a small number of patients who bleed, for bowel perforation and cases of complete obstruction. The aim is to rest distal disease by temporarily diverting faecal flow.

Answer 336

G. Irritable bowel syndrome - IBS is a chronic condition with abdominal pain associated with bowel dysfunction and is a diagnosis of exclusion. The pain or discomfort may be relieved by defecation. Examination is usually unremarkable and the diagnosis is based on the patient’s history in line with the Rome Criteria. If the patient presents with any worrying symptoms, then these will warrant a more thorough investigation. Treatment depends on the patient’s predominant symptoms. Antispasmodics relieve abdominal pain or discomfort but do not affect bowel habit. Examples include peppermint oil and dicycloverine. Laxatives can also be used such as lactulose. Lifestyle and dietary modifications combined with reassurance remain the 1st line intervention for functional bowel disease. IBS is linked with stressful jobs such as working as a secretary and there is a female/male ratio of 2:1. ‘Pellet-like’ stools is an indicator in EMQs.

Answer 337

D. Cancer of the pancreas - Pancreatic cancer typically presents with painless obstructive jaundice and weight loss and generally presents late. It can however, like this case, present with abdominal pain which is typically non-specific in the upper abdomen. If the patient presents with persistent back pain, then this symptom is consistent with retroperitoneal metastases. It is estimated that 1 in 4 cases can be linked to smoking. Whipple’s procedure or Traverso-Longmire procedure (pancreaticoduodenectomy) offers the only hope of a cure but only a small minority are elegible for these procedures. The first tests to order are an abdominal USS and LFTs. Note Courvoisier’s law with regard to this question: Jaundice and a palpable painless gallbladder is unlikely to be caused by gallstones. The tumour marker for pancreatic cancer is CA19-9 which is useful in preoperative staging.

Answer 338

A. Alcoholic cirrhosis - Cirrhosis is the end-stage of chronic liver disease, in this case due to alcoholic liver disease. Cirrhosis results in hepatic insufficiency and portal hypertension. The high GGT here is as a result of high alcohol consumption. Liver function tests are mistakenly named really because they don’t test liver function. They are better called ‘liver tests’ or ‘tests of liver chemistry’. The tests of liver synthetic function come from measuring albumin levels (produced by the liver) and the prothrombin time, as the liver has a key role in the manufacture of clotting factors. Some general points on liver tests to consider: These tests tend to include bilirubin (breakdown product of RBC after hepatic conjugation, and is secreted in the biliary system), AST, ALT, GGT, ALP (alk phos) and LDH. It’s really all about pattern recognition. Isolated elevation of liver tests tends to make you think of non-hepatic causes and you should take into account the patient’s history during interpretation. A normal liver panel does not mean that the person does not have liver disease, and liver tests are elevated in a small percentage of normals. You can split the possible causes into three broad categories. It is worth noting that people with any pattern can have cirrhosis. AST is also present in heart, skeletal muscle, kidney, brain and in RBCs too. 80% of AST is found in the mitochondria and 20% in the cytoplasmic matrix. ALP, which is a cytoplasmic enzyme, can come from bone, kidney, intestines or the placenta. GGT may come from the heart or RBCs. The first is predominantly elevated AST/ALT, which is described as a hepatocellular pattern and occurs in conditions such as viral hepatitis, alcoholic disease, Wilson’s etc. Here the hepatocytes get damaged and enzymes leak out. The second is predominantly elevated ALP (GGT too, but an isolated rise in GGT is common so many hospitals remove GGT from the liver panel. GGT is only useful if there is an isolated rise in ALP). This is seen in a cholestatic (here, the hepatobiliary system is affected) or infiltrative pattern in bile duct obstruction, malignancy e.g. HCC or pancreatic, PBC/PSC etc or infiltrative conditions such as TB and lymphomas. The last is an isolated hyperbilirubinaemia seen in haemolysis, intra-abdominal bleeding or with conditions such as Gilbert’s. BR elevation can be further divided into conjugated or unconjugated. Unconjugated is normally due to breakdown of RBC beyond the liver’s capacity to conjugate. Conjugated occurs in liver disease and problems with obstructed bile flow.

Answer 339

D. Hepatocellular carcinoma - The pattern here is cholestatic. Furthermore, elevated AFP is given, which is a tumour marker for HCC, differentiating this from other potential causes like pancreatic cancer. AST and ALT may be normal or elevated. Liver function tests are mistakenly named really because they don’t test liver function. They are better called ‘liver tests’ or ‘tests of liver chemistry’. The tests of liver synthetic function come from measuring albumin levels (produced by the liver) and the prothrombin time, as the liver has a key role in the manufacture of clotting factors. Some general points on liver tests to consider: These tests tend to include bilirubin (breakdown product of RBC after hepatic conjugation, and is secreted in the biliary system), AST, ALT, GGT, ALP (alk phos) and LDH. It’s really all about pattern recognition. Isolated elevation of liver tests tends to make you think of non-hepatic causes and you should take into account the patient’s history during interpretation. A normal liver panel does not mean that the person does not have liver disease, and liver tests are elevated in a small percentage of normals. You can split the possible causes into three broad categories. It is worth noting that people with any pattern can have cirrhosis. AST is also present in heart, skeletal muscle, kidney, brain and in RBCs too. 80% of AST is found in the mitochondria and 20% in the cytoplasmic matrix. ALP, which is a cytoplasmic enzyme, can come from bone, kidney, intestines or the placenta. GGT may come from the heart or RBCs. The first is predominantly elevated AST/ALT, which is described as a hepatocellular pattern and occurs in conditions such as viral hepatitis, alcoholic disease, Wilson’s etc. Here the hepatocytes get damaged and enzymes leak out. The second is predominantly elevated ALP (GGT too, but an isolated rise in GGT is common so many hospitals remove GGT from the liver panel. GGT is only useful if there is an isolated rise in ALP). This is seen in a cholestatic (here, the hepatobiliary system is affected) or infiltrative pattern in bile duct obstruction, malignancy e.g. HCC or pancreatic, PBC/PSC etc or infiltrative conditions such as TB and lymphomas. The last is an isolated hyperbilirubinaemia seen in haemolysis, intra-abdominal bleeding or with conditions such as Gilbert’s. BR elevation can be further divided into conjugated or unconjugated. Unconjugated is normally due to breakdown of RBC beyond the liver’s capacity to conjugate. Conjugated occurs in liver disease and problems with obstructed bile flow.

Answer 340

L. Gallstones - This is again a cholestatic pattern and the ultrasound scan means this is either choledocholithiasis, pancreatic cancer or cholangiocarcinoma. The fact there is RUQ pain points more towards gallstones. The other two tend to present painlessly. Liver function tests are mistakenly named really because they don’t test liver function. They are better called ‘liver tests’ or ‘tests of liver chemistry’. The tests of liver synthetic function come from measuring albumin levels (produced by the liver) and the prothrombin time, as the liver has a key role in the manufacture of clotting factors. Some general points on liver tests to consider: These tests tend to include bilirubin (breakdown product of RBC after hepatic conjugation, and is secreted in the biliary system), AST, ALT, GGT, ALP (alk phos) and LDH. It’s really all about pattern recognition. Isolated elevation of liver tests tends to make you think of non-hepatic causes and you should take into account the patient’s history during interpretation. A normal liver panel does not mean that the person does not have liver disease, and liver tests are elevated in a small percentage of normals. You can split the possible causes into three broad categories. It is worth noting that people with any pattern can have cirrhosis. AST is also present in heart, skeletal muscle, kidney, brain and in RBCs too. 80% of AST is found in the mitochondria and 20% in the cytoplasmic matrix. ALP, which is a cytoplasmic enzyme, can come from bone, kidney, intestines or the placenta. GGT may come from the heart or RBCs. The first is predominantly elevated AST/ALT, which is described as a hepatocellular pattern and occurs in conditions such as viral hepatitis, alcoholic disease, Wilson’s etc. Here the hepatocytes get damaged and enzymes leak out. The second is predominantly elevated ALP (GGT too, but an isolated rise in GGT is common so many hospitals remove GGT from the liver panel. GGT is only useful if there is an isolated rise in ALP). This is seen in a cholestatic (here, the hepatobiliary system is affected) or infiltrative pattern in bile duct obstruction, malignancy e.g. HCC or pancreatic, PBC/PSC etc or infiltrative conditions such as TB and lymphomas. The last is an isolated hyperbilirubinaemia seen in haemolysis, intra-abdominal bleeding or with conditions such as Gilbert’s. BR elevation can be further divided into conjugated or unconjugated. Unconjugated is normally due to breakdown of RBC beyond the liver’s capacity to conjugate. Conjugated occurs in liver disease and problems with obstructed bile flow.

Answer 341

B. Gilberts syndrome - Gilbert’s occurs in an asymptomatic patient, often as an incidental finding or mild jaundice occuring in adolescence/young adult age. There is elevated unconjugated BR with other liver tests being normal. The blood smear is also normal with normal reticulocyte count, and normal Hb indicating that this is not due to haemolysis. It is a common syndrome and is not really a disease, more a physiological variant. No treatment is needed and this condition is due to decreased UDPGT activity leading to decreased conjugation of unconjugated bilirubin, leading to elevated levels. Liver function tests are mistakenly named really because they don’t test liver function. They are better called ‘liver tests’ or ‘tests of liver chemistry’. The tests of liver synthetic function come from measuring albumin levels (produced by the liver) and the prothrombin time, as the liver has a key role in the manufacture of clotting factors. Some general points on liver tests to consider: These tests tend to include bilirubin (breakdown product of RBC after hepatic conjugation, and is secreted in the biliary system), AST, ALT, GGT, ALP (alk phos) and LDH. It’s really all about pattern recognition. Isolated elevation of liver tests tends to make you think of non-hepatic causes and you should take into account the patient’s history during interpretation. A normal liver panel does not mean that the person does not have liver disease, and liver tests are elevated in a small percentage of normals. You can split the possible causes into three broad categories. It is worth noting that people with any pattern can have cirrhosis. AST is also present in heart, skeletal muscle, kidney, brain and in RBCs too. 80% of AST is found in the mitochondria and 20% in the cytoplasmic matrix. ALP, which is a cytoplasmic enzyme, can come from bone, kidney, intestines or the placenta. GGT may come from the heart or RBCs. The first is predominantly elevated AST/ALT, which is described as a hepatocellular pattern and occurs in conditions such as viral hepatitis, alcoholic disease, Wilson’s etc. Here the hepatocytes get damaged and enzymes leak out. The second is predominantly elevated ALP (GGT too, but an isolated rise in GGT is common so many hospitals remove GGT from the liver panel. GGT is only useful if there is an isolated rise in ALP). This is seen in a cholestatic (here, the hepatobiliary system is affected) or infiltrative pattern in bile duct obstruction, malignancy e.g. HCC or pancreatic, PBC/PSC etc or infiltrative conditions such as TB and lymphomas. The last is an isolated hyperbilirubinaemia seen in haemolysis, intra-abdominal bleeding or with conditions such as Gilbert’s. BR elevation can be further divided into conjugated or unconjugated. Unconjugated is normally due to breakdown of RBC beyond the liver’s capacity to conjugate. Conjugated occurs in liver disease and problems with obstructed bile flow.

Answer 342

L. CML - This is CML which tends to present in the 30-60 age group. At presentation 1/3 may be asymptomatic though if symptomatic, it presents with symptoms including fever, weight loss and night sweats. There is myeloid stem cell proliferation and presents with raised neutrophils, metamyelocytes and basophils. The patient’s granulocytosis is suggestive of CML. CML is associated with the philadelphia chromosome characterised by t(9;22) of bcr-abl. There tends to be massive splenomegaly which is the most common physical finding on examination. This conditon may transform to AML or ALL in what is known as a ‘blast crisis’. CML responds to imatinib, which is an anti-bcr-abl antibody and gives long term remission in most patients.

Answer 343

H. Hodgkin's disease - This is a case of lymphoma. Reed-Sternberg cells are binucleate cells characteristically seen in Hodgkin’s lymphoma. Hodgkin’s is localised to a single group of nodes (normally the cervical and/or supraclavicular) and extranodal involvement is rare. Mediastinal involvement is common. Spread is contiguous and B symptoms may be present such as a low grade fever, weight loss and night sweats. Pruritis may be found in approximately 10% of cases but has no prognostic significance. 50% of cases is associated with EBV infection and distribution is bimodal with peaks in young and old. There is classically pain in lymph nodes on alcohol consumption.

Answer 344

E. Infectious mononucleosis - This is caused by EBV and characterised by fever, pharyngitis and lymphadenopathy with atypical lymphocytosis. Positive heterophile antibody test and serological testing for EBV antibodies are diagnostic. Splenomegaly is common and enlargement occurs in the first week, lasting 3-4 weeks. It is worth remembering that splenomegaly is always an abnormal examination finding. IM is commonly named the ‘kissing’ disease as EBV is most commonly transmitted by saliva. Penetrative sex and general promiscuity in young women also increases the risk.

Answer 345

G. Bacterial endocarditis - Any patient presenting with fever and a new murmur should always make you think of bacterial endocarditis. The classic new or worsening murmur is actually rare. As are splinter haemorrhages, which this patient has. Other uncommon signs you may find include Janeway lesions (painless macular haemorrhagic plaques on the palms and soles) and Osler nodes (painful nodules on the pads of the fingers and toes). Roth spots may also be seen on fundoscopy. Three sets of bood cultures are required and this patient will have to go for an echocardiogram.The Duke criteria is used for diagnosis.

Answer 346

J. Ideopathic myelofibrosis - This is a case of myelofibrosis. Leucoerythroblastosis and splenomegaly are common findings. Strong risk factors include exposure to radiation and industrial solvents. BM biopsy is essential for diagnosis. Extramedullary haematopoiesis leads to dacrocytes in the peripheral blood smear. Those without symptoms can be managed with folate and pyridoxine supplements. Otherwise options such as a BM transplant and hydroxycarbamide can be considered.

Answer 347

F. Haemorrhoidectomy - Haemorrhoidectomy is the treatment of choice of choice for patients with grade 4 haemorrhoids or for any patient who has failed with more conservative treatment such as sclerotherapy. Grade 1 is limited to within the anal canal. Grade 2 protrudes but spontaneously reduces when the patient stops straining. Grade 3 protrudes and reduces fully on manual pressure. Grade 4 is irreducible. Lord’s anal stretch is no longer recommended due to the risk of faecal incontinence.

Answer 348

E. Topical GTN - This patient has an anal fissure. This causes severe pain on defecation and may continue for 1 to 2 hours. A small amount of fresh blood is often passed on the stool. Initial treatment is with topical GTN or diltiazem along with supportive measures such a high fibre diet. Resistant or chronic fissures may benefit from surgical measures or botulinum toxin.

Answer 349

K. IV corticosteroid - IV corticosteroids are used in fulminant disease. These patients need to be admitted. If there is no response to IV corticosteroids within 24 to 48 hours then surgery is indicated.

Answer 350

C. IV immunoglobulin - ITP is thought to be due to an autoimmune phenomenon. Treatment is based on the patient’s platelet count and bleeding symptoms. This patient has severe active bleeding and must be started on IVIG plus corticosteroids, which she is already on. Platelet transfusions should be considered with tranexamic acid as an adjunct.

Answer 351

D. Anterior resection - Treatment of rectal carcinoma involves surgical excision where possible. This can either be an anterior resection (tumours in the upper 1/3 of the rectum) or an abdominoperineal resection (if the tumour lies lower down). APER involves the formation of a permanent colostomy and has a high incidence of sexual and urinary dysfunction. Anterior resection involves a colo-anal anastamosis.

Answer 352

D. Colonic carcinoma - The symptoms of colorectal cancer are non-specific but the GI symptoms here combined with weight loss should make you suspicious. Symptoms include change in bowel habit, rectal bleeding and anaemia, commonly associated with right sided cancer. Distension, weight loss and vomiting are usually associated with advanced disease. Colonoscopy will be needed in this patient for diagnosis and a biopsy of any suspicious lesion found. Main curative treatment is surgical resection. CEA is the classic tumour marker, which is raised in about 80% but is not really sensitive or specific enough to be useful in diagnosis or screening, but is used to monitor treatment and recurrence in those who have already been diagnosed.

Answer 353

G. Ulcerative collitis - While this could be Crohn’s disease, bloody diarrhoea is more commonly a presentation of UC than Crohn’s. UC is characterised by diffuse mucosal inflammation running a relapsing and remitting course. Bloody diarrhoea is commonly experienced by patients who may also complain of other symptoms such as (lower) abdominal pain, faecal urgency and the host of extra-intestinal manifestations associated with UC. Diagnosis of UC requires endoscopy with biopsy and a negative stool culture to rule out infectious gastroenteritis. Flare ups are usually linked to pathogens so a stool culture will always be needed in these cases. Toxic megacolon is a complication which is associated with a risk of perforation. UC is also linked with bowel adenocarinoma and PSC. Treatment involves mesalazine (5-ASA) used to induce and maintain remission.

Answer 354

H. Colonic polyp - Most polyps are asymptomatic and rectal bleeding is indeed an unusual symptom to present with (though FOBT may be positive), but the lack of previous symptoms and normal examination findings makes this likely to be a bleeding polyp. The next step here would be endoscopy with polypectomy for histological examination. Indeed they are frequently found incidentally on screening with FOBT or colonoscopy for another reason. If there are a few polyps, all of them will be removed. If there are many, a sample will be removed for biopsy. Polyps can also be inherited in FAP, also seen in Gardner syndrome and Peutz-Jeghers. They can be neoplastic such as adenomas or non-neoplastic such as hyperplastic polyps.

Answer 355

B Haemorrhoids - Haemorrhoids are vascular rich cushions in the anal canal and presents, typically, as painless bright PR bleeding or with sudden onset pain in the area associated with a palpable mass. Pruritus ani is common and there is often perianal pain or discomfort. Diagnosis is made visually. Grade 1 is limited to within the anal canal. Grade 2 protrudes but spontaneously reduces when the patient stops straining. Grade 3 protrudes and reduces fully on manual pressure. Grade 4 is irreducible. Treatment includes fibre, ligation, photocoagulation, sclerotherapy or surgical haemorrhoidectomy. Haemorrhoidectomy is the treatment of choice of choice for patients with grade 4 haemorrhoids or for any patient who has failed with more conservative treatment such as sclerotherapy.

Answer 356

A Infective collitis - This patient has recent foreign travel and a short history, previously normal, making infectious diarrhoea very likely. Probably eaten something dodgy while on elective. Think of the organisms which cause bloody diarrhoea such as EHEC. E coli is the most common cause of traveller’s diarrhoea. There may be a contact history due to faecal-oral spread. The mainstay of treatment is rehydration and supportive therapy. Antibiotics may be indicated, particularly in severe ETEC.

Answer 357

C. Fibroadenosis - Fibrocystic breasts are characterised by ‘lumpy’ breasts associated with pain which fluctuates with the menstrual cycle (it is worse during the luteal phase of menses). Risk factors include obesity, nulliparity, HRT and late onset menopause and first childbirth. It is a diagnosis of exclusion, and is considered to be an exaggerated physiological phenomenon rather than a disease (54% of clinically normal breasts are found on autopsy to have fibrocystic changes). Symptoms typically arise between the 3rd and 4th decases of life. There may also be a nipple discharge, which can be suspicious if bloody or profuse etc and may indicate the presence of an intraductal papilloma, cancer, or duct ectasia. Cysts can be aspirated if symptomatic (asymptomatic or small ones do not require intervention). If the aspirate is straw coloured and completely aspirated, there is no need for cytology, but if the aspirate is bloody, cytology or biopsy is needed to exclude cancer. There is improvement of mastalgia and cysts at menopause and until then it runs a chronic relapsing course.

Answer 358

B. Fibroadenoma - This is a fibroadenoma which tends to be asymptomatic and found incidentally, typically in a patient

Answer 359

E. Breast abscess - Breast abscess presents with mastalgia and fever. Breast infection typically affects women who are lactating and the most commonly implicated pathogen is staphylococcus aureus. The painful, hard and red lump indicates the development of an abscess. Antibiotic therapy is indicated with surgical intervention such as aspiration and drainage with possible duct excision. Prompt management of mastitis when it presents will usually lead to a good timely resolution and prevent the development of complications such as an abscess. An USS can help to identify the underlying abscess which usually forms a hypoechoic lesion. Needle aspiration can be used both therapeutically and diagnostically and can be guided by ultrasound.

Answer 360

D. Gynaecomasatia - This is a boy who has enlarged breasts. Normal to see gynaecomastia in puberty. Other causes include liver disease and as a side effect of drugs such as digoxin, spironolactone and cimetidine.

Answer 361

I. Duct ectasia - Duct ectasia happens because the lactiferous duct gets blocked. Ectasia means widening. It can mimic breast cancer as the discharge can be bloody sometimes and signs can include nipple inversion. The green nipple discharge is typical in EMQs. It is a self limiting condition.

Answer 362

F. Carcinoma of the breast - Patient’s with breast cancer, on examination (familiarise yourself with how to conduct a breast exam), tend to demonstrate a firm hard lump which may be associated with axillary lymphadenopathy, skin changes such as the orange peel (peau d’orange) and nipple changes/discharge. The skin changes here are most likely associated with locally advanced cancer. Many breast cancers are also diagnosed on routine mammography which can show microcalcifications, in the absence of a palpable mass. MRI is more sensitive but less specific so is recommended only in patients who are at high risk, such as BRCA1/2 mutation, history of chest radiation or certain syndromes like Cowden’s or Li-Fraumeni. FNA is also useful in rapid diagnosis, although is operator dependent when it comes to how sensitive and specific it is, and a core biopsy is preferred in most cases for diagnosis as it can differentiate pre-invasive and invasive disease. Treatment is MDT involving surgeons, oncologists, radiation oncologists etc. Do you know about the current NHS breast screening programme?

Answer 363

A. Fibrocystic changes - Fibrocystic breasts are characterised by ‘lumpy’ breasts associated with pain which fluctuates with the menstrual cycle (it is worse during the luteal phase of menses). Risk factors include obesity, nulliparity, HRT and late onset menopause and first childbirth. It is a diagnosis of exclusion, and is considered to be an exaggerated physiological phenomenon rather than a disease (54% of clinically normal breasts are found on autopsy to have fibrocystic changes). Symptoms typically arise between the 3rd and 4th decases of life. There may also be a nipple discharge, which can be suspicious if bloody or profuse etc and may indicate the presence of an intraductal papilloma, cancer, or duct ectasia. Cysts can be aspirated if symptomatic (asymptomatic or small ones do not require intervention). If the aspirate is straw coloured and completely aspirated, there is no need for cytology, but if the aspirate is bloody, cytology or biopsy is needed to exclude cancer. There is improvement of mastalgia and cysts at menopause and until then it runs a chronic relapsing course.

Answer 364

K. Breast abscess - Breast abscess presents with mastalgia and fever. Breast infection typically affects women who are lactating and the most commonly implicated pathogen is staphylococcus aureus. The painful, hot, engorged and red breast suggests the possible development of an abscess. Antibiotic therapy is indicated with surgical intervention such as aspiration and drainage with possible duct excision. Prompt management of mastitis when it presents will usually lead to a good timely resolution and prevent the development of complications such as an abscess. An USS can help to identify the underlying abscess which usually forms a hypoechoic lesion. Needle aspiration can be used both therapeutically and diagnostically and can be guided by ultrasound.

Answer 365

J. Fibroadenoma - This is a fibroadenoma which tends to be asymptomatic and found incidentally, typically in a patient

Answer 366

B. Breast cancer - There is a painless lump here which fluctuates in size. Breast cancer progressively increases in size regardless of the menstrual cycle, unlike fibrocystic breasts which may vary in size with the menstrual cycle. When examining the breasts, they should first be examined sat upright to inspect for any changes in skin colour, any dimpling and asymmetry. The axilla, supra and infra-clavicular nodes should also be checked for any nodal metastases. Then, with the patient supine (and arm behind the head), the breast tissue should be felt and the tissue at the beneath the nipple. Many breast cancers are also diagnosed on routine mammography which can show microcalcifications, in the absence of a palpable mass. MRI is more sensitive but less specific so is recommended only in patients who are at high risk, such as BRCA1/2 mutation, history of chest radiation or certain syndromes like Cowden’s or Li-Fraumeni. The estimated cumulative lifetime incidence of breast cancer in those with BRCA mutations is 87%. FNA is also useful in rapid diagnosis, although is operator dependent when it comes to how sensitive and specific it is, and a core biopsy is preferred in most cases for diagnosis as it can differentiate pre-invasive and invasive disease. Treatment is MDT involving surgeons, oncologists, radiation oncologists etc. Treatment with less than a total mastectomy will require radiotherapy to the remaining breast tissue afterwards.

Answer 367

K. Gynaecomastia - This is a man who has presented with breast enlargement i.e. gynaecomastia. The main cause is either chronic liver disease or drug induced. Drugs include the H2 receptor antagonist cimetidine, as well as digoxin and spironolactone. Gynaecomastia is benign in 99% of cases and results from relative excess of oestrogen or lack of testosterone. Oestrogenisation can follow from anabolic steroid use/abuse. This can hence occur physiologically during the newborn period, puberty and with age and obesity (not true gynaecomastia). Treatment can be offered if there is pain or embarassment including anti-oestrogens such as tamoxifen, androgens or surgical options like liposuction. The drugs that induce gynaecomastia can be subdivided into those that reduce testosterone synthesis (GnRH agonists, cancer drugs, ketoconazole, metronidazole, spironolactone), those that impair the action of testosterone (spironolactone again, finasteride, H2 blockers, PPIs) and those that act via oestrogen (digoxin, PHT, anabolic steroids).

Answer 368

D. Fat necrosis - Prior breast trauma or breast surgery such as augmentation can lead to fat necrosis. Examination will reveal a firm mass with irregular borders which can resemble malignancy. Biopsy will reveal fat necrosis. USS and mammography shows indistinct margins, sometimes with calcifications which may again resemble findings seen in carcinoma.

Answer 369

H. Fibroadenoma - Fibroadenomas are typically asymptomatic and are found incidentally in patients

Answer 370

C. Breast abscess - Breast abscess presents with mastalgia and fever. Breast infection typically affects women who are lactating and the most commonly implicated pathogen is staphylococcus aureus. The painful, hard and red lump indicates the development of an abscess. Antibiotic therapy is indicated with surgical intervention such as aspiration and drainage with possible duct excision. Prompt management of mastitis when it presents will usually lead to a good timely resolution and prevent the development of complications such as an abscess. An USS can help to identify the underlying abscess which usually forms a hypoechoic lesion. Needle aspiration can be used both therapeutically and diagnostically and can be guided by ultrasound.

Answer 371

J. Breast carcinoma - This lady’s advanced age and the fact the lump is hard, irregular and non-tender all point towards malignancy. The mass is typically fixed but can be mobile in early stages. There may be associated changes such as nipple inversion, peau d’orange, lymphadenopathy and skin retraction. Skin changes are always associated with locally advanced cancer. Triple assessment is indicated with a mammogram and biopsy. Risk factors include FH, prolonged oestrogen exposure and high levels of alcohol consumption.

Answer 372

C. Acute pyogenic mastitis - Breast infection typically affects women who are lactating and the most commonly implicated pathogen is staphylococcus aureus. The painful lump indicates the development of an abscess. Antibiotic therapy is indicated with surgical intervention such as aspiration and drainage with possible duct excision. Prompt management of mastitis when it presents will usually lead to a good timely resolution and prevent the development of complications such as an abscess. An USS can help to identify the underlying abscess which usually forms a hypoechoic lesion. Needle aspiration can be used both therapeutically and diagnostically and can be guided by ultrasound.

Answer 373

E. Fibrocysitic - Fibrocystic breasts are characterised by ‘lumpy’ breasts associated with pain which fluctuates with the menstrual cycle (it is worse during the luteal phase of menses). Risk factors include obesity, nulliparity, HRT and late onset menopause and first childbirth. It is a diagnosis of exclusion, and is considered to be an exaggerated physiological phenomenon rather than a disease (54% of clinically normal breasts are found on autopsy to have fibrocystic changes). Symptoms typically arise between the 3rd and 4th decases of life. There may also be a nipple discharge, which can be suspicious if bloody or profuse etc and may indicate the presence of an intraductal papilloma, cancer, or duct ectasia. Cysts can be aspirated if symptomatic (asymptomatic or small ones do not require intervention). If the aspirate is straw coloured and completely aspirated, there is no need for cytology, but if the aspirate is bloody, cytology or biopsy is needed to exclude cancer. There is improvement of mastalgia and cysts at menopause and until then it runs a chronic relapsing course.

Answer 374

J. Breast cancer - This lady’s advanced age and the fact the lump is hard and fixed all point towards malignancy. There may be associated changes like in this patient, ranging from nipple inversion, peau d’orange, dimpling and lymphadenopathy to skin retraction. Skin changes are always associated with locally advanced cancer. Triple assessment is indicated with a mammogram and biopsy. Risk factors include FH, prolonged oestrogen exposure and high levels of alcohol consumption.

Answer 375

D. Duct ectasia - Duct ectasia happens because the lactiferous duct gets blocked. Ectasia means widening. It can mimic breast cancer as the discharge can be bloody sometimes and signs can include nipple inversion. It is a self limiting condition. The cytology of inflammatory cells confirms this diagnosis as duct ectasia is where the duct is dilated and is associated with inflammation.

Answer 376

D. Phylloides tumour - A phylloides tumour is a fast growing fibroepithelial mass, as demonstrated here by the history and biopsy findings. It can be benign or malignant. The only cure is surgical, commonly a WLE.

Answer 377

F. Breast abscess - Breast abscess presents with mastalgia and fever. Breast infection typically affects women who are lactating and the most commonly implicated pathogen is staphylococcus aureus. The painful, hard and red lump indicates the development of an abscess. Antibiotic therapy is indicated with surgical intervention such as aspiration and drainage with possible duct excision. Prompt management of mastitis when it presents will usually lead to a good timely resolution and prevent the development of complications such as an abscess. An USS can help to identify the underlying abscess which usually forms a hypoechoic lesion. Needle aspiration can be used both therapeutically and diagnostically and can be guided by ultrasound.

Answer 378

G. Fibroadenoma - Fibroadenomas are typically asymptomatic and are found incidentally in patients

Answer 379

J. Invasive breast cancer - Patient’s wth breast cancer, on examination (familiarise yourself with how to conduct a breast exam), tend to demonstrate a firm hard lump which may be associated with axillary lymphadenopathy, skin changes such as the orange peel (peau d’orange) and nipple changes/discharge. The skin changes here are most likely associated with locally advanced cancer. Many breast cancers are also diagnosed on routine mammography which can show microcalcifications, in the absence of a palpable mass. MRI is more sensitive but less specific so is recommended only in patients who are at high risk, such as BRCA1/2 mutation, history of chest radiation or certain syndromes like Cowden’s or Li-Fraumeni. FNA is also useful in rapid diagnosis, although is operator dependent when it comes to how sensitive and specific it is, and a core biopsy is preferred in most cases for diagnosis as it can differentiate pre-invasive and invasive disease. Treatment is MDT involving surgeons, oncologists, radiation oncologists etc. Do you know about the current NHS breast screening programme?

Answer 380

C. Gynaecomastia - This is a man who has presented with breast enlargement i.e. gynaecomastia. The main cause is either chronic liver disease or drug induced. Drugs include the H2 receptor antagonist cimetidine, as well as digoxin and spironolactone. Gynaecomastia is benign in 99% of cases and results from relative excess of oestrogen or lack of testosterone. Oestrogenisation can follow from anabolic steroid use/abuse. This can hence occur physiologically during the newborn period, puberty and with age and obesity (not true gynaecomastia). Treatment can be offered if there is pain or embarassment including anti-oestrogens such as tamoxifen, androgens or surgical options like liposuction. The drugs that induce gynaecomastia can be subdivided into those that reduce testosterone synthesis (GnRH agonists, cancer drugs, ketoconazole, metronidazole, spironolactone), those that impair the action of testosterone (spironolactone again, finasteride, H2 blockers, PPIs) and those that act via oestrogen (digoxin, PHT, anabolic steroids).

Answer 381

A. Thyroglossal cyst - This midline neck swelling moves up on both swallowing and tongue protrusion making this a thyroglossal cyst. Thyroid nodules do not move on protrusion of the tongue. It is a cyst that forms from a remnant thyroglossal duct and can hence develop anywhere along the length of this embryological duct, which is a midline structure between the foramen caecum at the back of the tongue and the thyroid gland.

Answer 382

J. Branchial cyst - A branchial cyst is a congenital abnormality arising due to failure to obliterate the 2nd branchial cleft in development. They are congenital, hence the presence since childhood. They most commonly are found along the anterior border of sternocleidomastoid like in this case. They are generally asymptomatic unless they become infected. Treatment can be conservative or involve surgical excision.

Answer 383

K. Sebaceous cyst - This is a sebaceous cyst evidence by the swelling which becomes inflamed at times and expresses thick, foul smelling white keratin matter, which is when it becomes infected. They are common in hairier areas and are generally smooth and round. They can be caused by blockage of sebaceous glands.

Answer 384

B. Sialolithiasis - This patient has sialolithiasis. Salivary gland stones cause pain whenever the patient salivates (eating or thinking of food). Stones are commonly found in the submandibular gland. Diagnosis can be confirmed by facial radiographs but a small number of sialoliths may not be seen on the plain film due to low calcium phosphate content. In this case, sialography used in combination with CT will demonstrate the stone. A complication is the development of sialadenitis.

Answer 385

I. Carotid body tumour - A carotid body tumour is also called a chemodectoma. It is a paraganglioma – a tumour derived from neural crest cells. PGs can occur anywhere neural crest cells migrate so in the head and neck, other PGs include, for example, tympanic paragangliomas arising from the tympanic plexus. They are more common at high altitudes and in females and most are spontaneous occuring in the 3rd and 4th decade. Most present with a painless lateral neck mass which grows slowly. It will move from side to side but not really up and down (think of the anatomy here, this is known as Fontaine’s sign). Larger tumours may cause cranial nerve palsies. There may be an associated carotid bruit or pulsatility. There may also be dysphagia or pain on swallowing owing to compression. Biopsy of this mass is contraindicated for very obvious reasons. The first investigation would be with a colour doppler ultrasound and gold standard for diagnosis is with digital subtraction angiography. MRI can also be utilised. Treatment is surgical.

Answer 386

F. Parotid carcinoma - The main pointers to suggest malignancy here are the involvement of the facial nerve causing a facial palsy, the rapid rate of growth and pain. Another suggestion would be ulceration, if present. There is swelling at the angle of the jaw, which could feasibly be a submandibular tumour, although most salivary gland tumours are parotid. A benign tumour would likely be indolent and slow growing, and asymptomatic (though a lymphoma can too). FNA cytology will be needed and MRI offers excellent views of the soft tissue structures surrounding the tumour and the relation of the tumour to its surroundings.

Answer 387

F. Thyroglossal cyst - This midline neck swelling moves up on both swallowing and tongue protrusion making this a thyroglossal cyst. Thyroid nodules do not move on protrusion of the tongue. It is a cyst that forms from a remnant thyroglossal duct and can hence develop anywhere along the length of this embryological duct, which is a midline structure between the foramen caecum at the back of the tongue and the thyroid gland.

Answer 388

C. Carcinomatous lymph node - Weight loss is one of the most common presenting symptoms in patients with gastric cancer. Epigastric pain is present in about 80%. Although commonly mentioned in EMQs, lymphadenopathy is an uncommon presentation. The left supraclavicular node here is Virchow’s node. It becomes enlarged due to the pattern of lymphatic drainage into the thoracic duct. There may also be a periumbilical nodule (Sister Mary Joseph’s nodule) or a left axillary nodule (Irish node). These are rare findings.

Answer 389

E. Hodgkin's lymphoma - This is a case of lymphoma. Reed-Sternberg cells are binucleate cells characteristically seen in Hodgkin’s lymphoma. Hodgkin’s is localised to a single group of nodes (normally the cervical and/or supraclavicular) and extranodal involvement is rare. Mediastinal involvement is common. Spread is contiguous and B symptoms may be present such as a low grade fever, weight loss and night sweats. Pruritis may be found in approximately 10% of cases but has no prognostic significance. 50% of cases is associated with EBV infection and distribution is bimodal with peaks in young and old. There is classically pain in lymph nodes on alcohol consumption.

Answer 390

H. Carotid body tumour - A carotid body tumour is also called a chemodectoma. It is a paraganglioma – a tumour derived from neural crest cells. PGs can occur anywhere neural crest cells migrate so in the head and neck, other PGs include, for example, tympanic paragangliomas arising from the tympanic plexus. They are more common at high altitudes and in females and most are spontaneous occuring in the 3rd and 4th decade. Syncope is really quite rare and most present with a painless lateral neck mass which grows slowly. It will move from side to side but not really up and down (think of the anatomy here, this is known as Fontaine’s sign). Larger tumours may cause cranial nerve palsies. There may be an associated carotid bruit, or in this case, pulsatility. There may also be dysphagia or pain on swallowing owing to compression. Biopsy of this mass is contraindicated for very obvious reasons. The first investigation would be with a colour doppler ultrasound and gold standard for diagnosis is with digital subtraction angiography. MRI can also be utilised. Treatment is surgical.

Answer 391

D. TB abscess - This is a cold abscess of TB, which is a cold mass with overlying skin changes where the skin turns a purple-ish colour. The accompanying symptoms are those of TB infection (fever, feeling unwell, tachycardia). Effectively, it is a scrofula, which is TB infection of the lymph nodes in the neck (lymphadenitis). If you are lucky enough to see one on the wards, you won’t forget it.

Answer 392

A. Sialogram - This patient has sialolithiasis. Salivary gland stones cause pain whenever the patient salivates (eating or thinking of food, like Pavlov's dogs!). Stones are commonly found in the submandibular gland. At night time, there is less salivation accounting for the lack of pain. Diagnosis can be confirmed by facial radiographs but a small number of sialoliths may not be seen on the plain film due to low calcium phosphate content. In this case, sialography used in combination with CT will demonstrate the stone. A complication is the development of sialadenitis.

Answer 393

H. Carbimazole - This woman has hyperthyroidism. The smooth midline swelling and the audible bruit point towards a diagnosis of Graves’ disease. In countries where sufficient iodine intake is not an issue, Graves’ disease is the most common cause of hyperthyroidism. Peripheral manifestations such as ophthalmopathy, pretibial myxoedema and hyperthyroid acropachy do not occur with other causes of hyperthyroidism. Acropachy manifests as clubbing with soft tissue swelling. Pretibial myxoedema is almost always associated with ophthalmopathy. A thyroid isotope scan such as Tc-99 pertechnetate is used to differentiate the diffuse enlargement of Graves’ with the patchy uptake of a multinodular goitre. This is not necessary based on the examination findings. Treatment aims to normalise thyroid function and is achieved by radioactive iodine, antithyroid medications or with surgery. They are all effective and relatively safe options. Symptomatic therapy is given with beta blockers such as propranolol.

Answer 394

C. Full blood count and Paul Bunnell test - Infectious mononucleosis is caused by EBV and is characterised by fever, pharyngitis and lymphadenopathy. A FBC will show an atypical lymphocytosis. Confirmation of IM involves detection of the existence of heterophile antibodies using the Paul Bunnell monospot. A more accurate test is a serological test detecting EBV specific antibodies. Treatment is usually symptomatic but IM carries rare but potentially life threatening complications.

Answer 395

G. Technetium thyroid scan - This is a case of toxic multinodular goitre. It is most common in older patients and is associated with head and neck irradiation and iodine deficiency. TSH is the initial screening test and if supressed, T4/T3 levels are measured. As the peripheral stigmata of Graves’ disease is absent, thyroid scan and uptake is indicated. I-123 is the preferred isotope but as this option is not given, Tc-99 can be used although there is a risk of false positive images. The scan will show multiple hot and cold areas consistent with areas of autonomy and areas of suppression. Definitive treatment is commonly given in the form of radioactive iodine.

Answer 396

E. Upper GI endoscopy - The bilateral mass in the last part of this question is a multinodular goitre which does not need a biopsy. The patient is euthyroid. If the nodule was unilateral, then a biopsy is essential to establish or exclude malignancy. However, her dysphagia needs to be investigated with an upper GI endoscopy.

Answer 397

I. Grave's disease - This woman has symptoms of hyperthyroidism (weight loss and palpitations) and a goitre. In countries where sufficient iodine intake is not an issue, Graves’ disease is the most common cause of hyperthyroidism. Graves’ gives a diffuse goitre. Peripheral manifestations such as ophthalmopathy, pretibial myxoedema and hyperthyroid acropachy do not occur with other causes of hyperthyroidism. Ophthalmopathy includes lid retraction, exophthalmos and eye movement restriction leading to diplopia. Acropachy is an uncommon manifestation presenting as clubbing with soft tissue swelling. Pretibial myxoedema is almost always associated with ophthalmopathy. Treatment aims to normalise thyroid function and is achieved by radioactive iodine, antithyroid medications or with surgery. They are all effective and relatively safe options. Symptomatic therapy is given with beta blockers such as propranolol.

Answer 398

B. Thyroglossal cyst - This midline neck swelling moves up on both swallowing and tongue protrusion making this a thyroglossal cyst. It is a cyst that forms from a remnant thyroglossal duct and can hence develop anywhere along the length of this embryological duct, which is a midline structure between the foramen caecum at the back of the tongue and the thyroid gland.

Answer 399

E. De Quervain's thyroiditis - This girl has de Quervain’s thyroiditis (which can also be called subacute granulomatous thyroiditis) which is inflammation of the thyroid characterised by a triphasic course where there is transient thyrotoxicosis followed by a hypothyroid phase before a return to euthyroidism. The thyrotoxic phase (symptoms of hyperthyroidism may be present) is characterised by pain and tenderness of the thyroid, which tends to be larger, firm and tender to touch. This girl also has a fever and neck pain which is making it painful for her to swallow. This is a self-limiting condition and no specific treatment is needed though NSAIDs and beta blockers can be used for symptomatic relief. Roughly 30-40% describe a prior viral infection.

Answer 400

K. Hodgkin's disease - This is a case of lymphoma. Reed-Sternberg cells are binucleate cells characteristically seen in Hodgkin’s lymphoma. Hodgkin’s is localised to a single group of nodes (normally the cervical and/or supraclavicular) and extranodal involvement is rare. Mediastinal involvement is common. Spread is contiguous and B symptoms may be present such as a low grade fever, weight loss and night sweats. Pruritis may be found in approximately 10% of cases but has no prognostic significance. 50% of cases is associated with EBV infection and distribution is bimodal with peaks in young and old. There is classically pain in lymph nodes on alcohol consumption.

Answer 401

A. Superior vena cava syndrome - SVC syndrome occurs due to SVCO. The most common cause of this is malignancy (this smoker probably has lung cancer which is the most likely cause in those >50). There are also benign causes such as iatrogenically due to pacemaker leads and central venous catheters causing SVC thrombosis. Common symptoms are facial swelling and arm swelling as seen, as well as dyspnoea, cough and facial plethora. There may also be a headache, chest pain, blurry vision and stridor. These symptoms tend to be worse on bending forwards or lying down. The most useful imaging test is a chest CT with IV contrast which establishes the diagnosis and also shows the exact location of the pathology. A CXR in SVCO can show a widened mediastinum or the lung cancer which is the cause.

Answer 402

D. Myxoedema - This medical student has hypothyroidism. Worldwide, the most common cause is iodine deficiency. Other causes include Hashimoto’s or secondary and tertiary hypothyroidism. It can also result from viral de Quervain’s thyroiditis or postpartum thyroiditis. Symptoms include those mentioned (weight gain, lethargy, sluggish reflexes, bradycardia and constipation) as well as depression, fatigue, constipation, cold intolerance, menstrual problems in females, dry skin and muscle cramps. Diagnosis is based on measurement of TSH and thyroid hormones. Treatment is by replacement of T4 with or without T3 in combination. If the patient has normal T3 and T4 but mildly elevated TSH, this is described as subclinical hypothyroidism.

Answer 403

F. Stomach carcinoma - The presence of Virchow’s node (Troisier’s sign), a hard enlarged node in the left supraclavicular fossa, points towards a malignancy in the abdominal cavity. This is most often stomach cancer. The lymph drainage of the abdominal cavity drains into Virchow’s node as the lymph drains most of the body from the thoracic duct and enters the venous circulation at the left subclavian vein.

Answer 404

C. IV Saline - This patient is very dehydrated Fluid replacement therapy aims to maintain physiological parameters. Systolic BP >90 is required for adequate organ perfusion. IV 0.9% saline is the first line treatment for volume depletion in almost all situations. It is worth noting that normal saline in large amounts carries a risk of inducing a metabolic acidosis due to the high chloride content. 5% dextrose is equivalent to water when given, and is not approriate for volume resuscitation since it will distribute throughout the total body water. Only 1/12 will remain in the intravascular space. 5% dextrose may be used, however, in resuscitation or replacement in diabetics on an insulin drip to prevent hypoglycaemia. The insulin and dextrose infusion should go in the same cannula so there is no risk of giving unopposed insulin. For example, if the arm you are giving the dextrose infusion were to clog up, or more likely, if the patient bends their arm if the cannula is sited in the antecubital fossa. Sodium bicarbonate solution can be used acutely to treat severe metabolic acidosis. Lactated Ringer’s solution may also be helpful, but may contribute to hyperkalaemia in the setting of renal failure. IV colloids are less preferred. These include albumin, starches, dextrans and gelatins. Colloids are used when there is a risk of tissue oedema as there is a reduced proportion of administered fluid lost into the interstitial space. However, they are expensive and have not shown to benefit mortality in many studies, and indeed a systematic review in the BMJ of 37 RCTs has shown a 4% increase in absolute mortality. Crystalloids remain first choice for fluid resuscitation in the first instance. Blood is the best intravascular volume expander (replacing like for like), especially if the patient is anaemic or is actively bleeding. It is usually given as packed red cells with saline. Mild volume depletion can be managed by ORT. Glucose is typically added to promote the sodium/glucose co-transporter. Depending on the site of loss, antiemetics and antidiarrhoeals (in non-infectious diarrhoea) may be indicated. Vasopressors are often needed in sepsis. So, having said all of this...

Answer 405

D. Administer diuretics - This patient has developed pulmonary oedema. This patient should be positioned upright and given diuretics (frusemide). Fluid replacement therapy aims to maintain physiological parameters. Systolic BP >90 is required for adequate organ perfusion. IV 0.9% saline is the first line treatment for volume depletion in almost all situations. It is worth noting that normal saline in large amounts carries a risk of inducing a metabolic acidosis due to the high chloride content. 5% dextrose is equivalent to water when given, and is not approriate for volume resuscitation since it will distribute throughout the total body water. Only 1/12 will remain in the intravascular space. 5% dextrose may be used, however, in resuscitation or replacement in diabetics on an insulin drip to prevent hypoglycaemia. The insulin and dextrose infusion should go in the same cannula so there is no risk of giving unopposed insulin. For example, if the arm you are giving the dextrose infusion were to clog up, or more likely, if the patient bends their arm if the cannula is sited in the antecubital fossa. Sodium bicarbonate solution can be used acutely to treat severe metabolic acidosis. Lactated Ringer’s solution may also be helpful, but may contribute to hyperkalaemia in the setting of renal failure. IV colloids are less preferred. These include albumin, starches, dextrans and gelatins. Colloids are used when there is a risk of tissue oedema as there is a reduced proportion of administered fluid lost into the interstitial space. However, they are expensive and have not shown to benefit mortality in many studies, and indeed a systematic review in the BMJ of 37 RCTs has shown a 4% increase in absolute mortality. Crystalloids remain first choice for fluid resuscitation in the first instance. Blood is the best intravascular volume expander (replacing like for like), especially if the patient is anaemic or is actively bleeding. It is usually given as packed red cells with saline. Mild volume depletion can be managed by ORT. Glucose is typically added to promote the sodium/glucose co-transporter. Depending on the site of loss, antiemetics and antidiarrhoeals (in non-infectious diarrhoea) may be indicated. Vasopressors are often needed in sepsis. So, having said all of this...

Answer 406

C. IV saline - As mentioned, first line fluid resuscitation is with 0.9% saline. Fluid replacement therapy aims to maintain physiological parameters. Systolic BP >90 is required for adequate organ perfusion. IV 0.9% saline is the first line treatment for volume depletion in almost all situations. It is worth noting that normal saline in large amounts carries a risk of inducing a metabolic acidosis due to the high chloride content. 5% dextrose is equivalent to water when given, and is not approriate for volume resuscitation since it will distribute throughout the total body water. Only 1/12 will remain in the intravascular space. 5% dextrose may be used, however, in resuscitation or replacement in diabetics on an insulin drip to prevent hypoglycaemia. The insulin and dextrose infusion should go in the same cannula so there is no risk of giving unopposed insulin. For example, if the arm you are giving the dextrose infusion were to clog up, or more likely, if the patient bends their arm if the cannula is sited in the antecubital fossa. Sodium bicarbonate solution can be used acutely to treat severe metabolic acidosis. Lactated Ringer’s solution may also be helpful, but may contribute to hyperkalaemia in the setting of renal failure. IV colloids are less preferred. These include albumin, starches, dextrans and gelatins. Colloids are used when there is a risk of tissue oedema as there is a reduced proportion of administered fluid lost into the interstitial space. However, they are expensive and have not shown to benefit mortality in many studies, and indeed a systematic review in the BMJ of 37 RCTs has shown a 4% increase in absolute mortality. Crystalloids remain first choice for fluid resuscitation in the first instance. Blood is the best intravascular volume expander (replacing like for like), especially if the patient is anaemic or is actively bleeding. It is usually given as packed red cells with saline. Mild volume depletion can be managed by ORT. Glucose is typically added to promote the sodium/glucose co-transporter. Depending on the site of loss, antiemetics and antidiarrhoeals (in non-infectious diarrhoea) may be indicated. Vasopressors are often needed in sepsis. So, having said all of this...

Answer 407

C. IV saline - Initial treatment of DKA aims at correcting severe volume depletion, again with IV saline infusion at a rate of 1-1.5L for the first hour. When glucose reaches 11.1mmol, fluid should be changed to 5% dextrose to prevent hypoglycaemia. Bicarbonate therapy may be necessary in adults with pH 90 is required for adequate organ perfusion. IV 0.9% saline is the first line treatment for volume depletion in almost all situations. It is worth noting that normal saline in large amounts carries a risk of inducing a metabolic acidosis due to the high chloride content. 5% dextrose is equivalent to water when given, and is not approriate for volume resuscitation since it will distribute throughout the total body water. Only 1/12 will remain in the intravascular space. 5% dextrose may be used, however, in resuscitation or replacement in diabetics on an insulin drip to prevent hypoglycaemia. The insulin and dextrose infusion should go in the same cannula so there is no risk of giving unopposed insulin. For example, if the arm you are giving the dextrose infusion were to clog up, or more likely, if the patient bends their arm if the cannula is sited in the antecubital fossa. Sodium bicarbonate solution can be used acutely to treat severe metabolic acidosis. Lactated Ringer’s solution may also be helpful, but may contribute to hyperkalaemia in the setting of renal failure. IV colloids are less preferred. These include albumin, starches, dextrans and gelatins. Colloids are used when there is a risk of tissue oedema as there is a reduced proportion of administered fluid lost into the interstitial space. However, they are expensive and have not shown to benefit mortality in many studies, and indeed a systematic review in the BMJ of 37 RCTs has shown a 4% increase in absolute mortality. Crystalloids remain first choice for fluid resuscitation in the first instance. Blood is the best intravascular volume expander (replacing like for like), especially if the patient is anaemic or is actively bleeding. It is usually given as packed red cells with saline. Mild volume depletion can be managed by ORT. Glucose is typically added to promote the sodium/glucose co-transporter. Depending on the site of loss, antiemetics and antidiarrhoeals (in non-infectious diarrhoea) may be indicated. Vasopressors are often needed in sepsis. So, having said all of this...

Answer 408

B. Blood transfusions - This patient has been given IV saline already as the initial choice for volume expansion. His anaemia and clinical state warrants a blood transfusion. 1 unit of blood raises the Hb concentration by 1g/dL. Commonly, transfusion begins with 2 units of packed RBCs and the patient reponse is monitored. Fluid replacement therapy aims to maintain physiological parameters. Systolic BP >90 is required for adequate organ perfusion. IV 0.9% saline is the first line treatment for volume depletion in almost all situations. It is worth noting that normal saline in large amounts carries a risk of inducing a metabolic acidosis due to the high chloride content. 5% dextrose is equivalent to water when given, and is not approriate for volume resuscitation since it will distribute throughout the total body water. Only 1/12 will remain in the intravascular space. 5% dextrose may be used, however, in resuscitation or replacement in diabetics on an insulin drip to prevent hypoglycaemia. The insulin and dextrose infusion should go in the same cannula so there is no risk of giving unopposed insulin. For example, if the arm you are giving the dextrose infusion were to clog up, or more likely, if the patient bends their arm if the cannula is sited in the antecubital fossa. Sodium bicarbonate solution can be used acutely to treat severe metabolic acidosis. Lactated Ringer’s solution may also be helpful, but may contribute to hyperkalaemia in the setting of renal failure. IV colloids are less preferred. These include albumin, starches, dextrans and gelatins. Colloids are used when there is a risk of tissue oedema as there is a reduced proportion of administered fluid lost into the interstitial space. However, they are expensive and have not shown to benefit mortality in many studies, and indeed a systematic review in the BMJ of 37 RCTs has shown a 4% increase in absolute mortality. Crystalloids remain first choice for fluid resuscitation in the first instance. Blood is the best intravascular volume expander (replacing like for like), especially if the patient is anaemic or is actively bleeding. It is usually given as packed red cells with saline. Mild volume depletion can be managed by ORT. Glucose is typically added to promote the sodium/glucose co-transporter. Depending on the site of loss, antiemetics and antidiarrhoeals (in non-infectious diarrhoea) may be indicated. Vasopressors are often needed in sepsis. So, having said all of this...

Answer 409

A. Diclofenac - NSAIDS may impair renal function and provoke renal failure, especially in patients with pre-existing impairment. NSAIDs should be avoided if possible in these patients or used with caution at the lowest effective dose for the shortest possible time. The mechanism of damage involves reducing creatinine clearance. NSAIDs are also contraindicated in asthmatics as it causes bronchospasm due to the accumulation of leukotrienes.

Answer 410

G. Epidural bupivacaine fentanyl - Epidurals are relatively contraindicated in anticoagulated patients. Insertion of the epidural needle may lead traumatic bleeding into the epidural space and with clotting abnormalities, the development of a haematoma which can lead to spinal cord compression. Coagulopathy, raised ICP and infection at the injection site are absolute contraindications. Relative contraindications include anticoagulated patients and those with anatomical abnormalities of the vertebral column. NSAIDs do not increase the risk of epidural haematoma.

Answer 411

B. Codydramol - Co-dydramol is a combination of dihydrocodeine and paracetamol and the patient is known to be allergic to dihydrocodeine.

Answer 412

A. Diclofenac - NSAIDs inhibit COX which has the effect of reducing PGE2 levels. PGE2 plays a role in gastric cytoprotection by downregulating HCl production and increasing mucus and the production of bicarbonate. This leads to gastric irritation and ulceration. A PPI can be prescribed alongside NSAIDs or misoprostol can be used, which is a stable PGE1 analogue which mimics local PG to maintain the gastroduodenal mucosal barrier.

Answer 413

C. Oropharyngeal airway with oxygen - An oropharyngeal (or Guedel) airway is sized from the angle of the mandible to the level of the incisors. It is a non-definitive airway adjunct. The patient is breathing in an obstructed manner indicating some degree of partial upper airway obstruction. The Guedel will keep the airway patent and prevent the tongue obstructing the airway by depressing it. The Guedel can only be used if the patient has a reduced GCS as it can initiate a gag reflex. If the patient was not unconscious, then a nasopharyngeal airway can be used (usually inserted in the right nostril). Additionally, a Guedel is contraindicated if the patient has injuries to the face or a condition that prevents the mouth from opening. Airway manoeuvres can also be used such as a jaw thrust or head tilt chin lift in addition to maintain a patent airway. A jaw thrust can onlybe done if the patient is unconscious. Think about where your fingers are digging in.

Answer 414

D. Single lumen cuffed ET tube - This patient is unconscious and is about to undergo a CT scan. He needs a definitive airway. The cuffed end creates a seal the prevent the aspiration of stomach contents. Reduced consciousness is a major risk factor for aspiration due to an inadequate cough reflex and impaired closure of the glottis.

Answer 415

I. LAryngeal mask airway (LMA) - An LMA is not a definitive airway. It forms a cuff over the opening of the larynx and works better than a bag and mask. It is less likely to result in gastric distension and reflux due to the lower pressures it generates. However, as it lives above the glottis, it does not prevent reflux. It is popular in short day case surgery and this patient is young and healthy without a known risk of aspiration.

Answer 416

J. Facemask with oxygen - This woman is fine apart from a low oxygen saturation. She needs some oxygen via a facemask.

Answer 417

D. SIngle lumen cuffed ET tube - This patient is elderly, having surgery in a recumbent position and has known aspiration risk factors of obesity and GORD. She will need a definitive airway with a cuffed ET tube to create a seal to prevent aspiration of stomach contents. The ease of intubation can be determined by an airway assessment including a Mallampatti score (I to IV). A double lumen ET tube is designed for some intrathoracic operations. An uncuffed ET tube is preferred in children as the trachea is not as strong. If it is an emergency setting and ET intubation fails, cricothyroidotomy can be performed which allows approximately 30 minutes of ventilation. Cricothyroidotomy will only work with at least a partially patent larynx.

Answer 418

G. Intravenous N-acetylcysteine - Paracetamol OD can occur after a single large OD or repeated ODs. Often, the patient is asymptomatic at initial presentation but if untreated may cause liver injury over the 2-4 days after ingestion, including fulminant liver failure. Paracetamol is the most frequent intentional OD drug in this country. The risk of liver damage is increased after taking drugs which induce CYP 450. Inducers include St John’s wort, barbiturates, phenytoin, tetracycline, chronic alcohol use and carbamazepine. A serum paracetamol level is important to order as early as possible, but at the earliest 4 hours post-ingestion.Treatment if indicated is with N-acetylcysteine with the level based on a paracetamol treatment graph. NAC will reduce hepatic damage caused by NAPQI. There is insufficient endogenous glutathione to neutralise this in an overdose and NAC binds and neutralises NAPQI. In patients allergic to NAC, oral methionine can be used.

Answer 419

C. Intravenous naloxone - Signs of opiate OD include CNS depression, miosis (pinpoint pupils) and apnoea. Naloxone is indicated both therapeutically and diagnostically. If there is a response, then it is diagnostic. Another diagnosis should be sought if the patient is unresponsive. IV is the preferred route of administration although naloxone can be given IM or SC if IV access cannot be established. Ventilatory support is key with 100% oxygen.

Answer 420

I. Haemodialysis - Haemodialysis is the treatment of choice for severe lithium poisoning. Note that activated charcoal does not adsorb lithium.

Answer 421

B. Alkaline diuresis - This patient has ingested at least 9000mg of aspirin. GIT decontamination should be considered as an adjunct on arrival to A&E and activated charcoal can be given. The mainstay of treatment is alkaline diuresis induced by an infusion of sodium bicarbonate. In cases of severe poisoning, it is still started as a bridge to haemodialysis.

Answer 422

F. Intravenous atropine - Organophosphate poisoning can occur due to occupational or accidental exposure, or if you were being attacked by Saddam Hussein’s regime. Treatment is with resuscitation and supportive care, removal of contaminated clothing, washing the skin and starting full atropinisation IV. Over treament is very much preferred to under treatment and the skin should feel dry when the patient has received adequate atropine. Pupils will also be dilated. Pralidoxime is often given as an adjunct in very severe cases but evidence does not support its routine use, unless you’ve just been attacked by a nerve agent by terrorists, which is unlikely in this agricultural worker.

Answer 423

C. 13 - M6 V4 E3 = 13 A GCS less than or equal to 8 is deemed a coma. Head injury can also be classified into mild (13-14), moderate (8-12) and severe (

Answer 424

B. 10 - M3 V4 E3 = 10 A GCS less than or equal to 8 is deemed a coma. Head injury can also be classified into mild (13-14), moderate (8-12) and severe (

Answer 425

F. 3 - M1 V1 E1 = 3, the worst possible GCS score A GCS less than or equal to 8 is deemed a coma. Head injury can also be classified into mild (13-14), moderate (8-12) and severe (

Answer 426

B. 10 - M5 V2 E3 = 10 A GCS less than or equal to 8 is deemed a coma. Head injury can also be classified into mild (13-14), moderate (8-12) and severe (

Answer 427

E. Urinary tract infection - E coli causes most uncomplicated cases and Staph saprophyticus is implicated in 5-20%. Sexual intercourse is the single biggest risk factor in women, although other risks include spermicide use, post-menopause, FH and presence of a foreign body like an indwelling catheter. Symptoms include frequency, dysuria, back pain, haematuria with upper tract involvement suggested by urgency, suprapubic pain, fever and/or tenderness over the costovertebral angle. The first test is a dipstick analysis, although if negative with a suggestive history, it is still likely to be a UTI. Dipstick will show positive nitrite and leucocyte esterase. MSU should be sent for MC+S in some cases such as atypical symptoms or unexpected urinalysis findings.

Answer 428

F. Bladder cancer - Gross haematuria is the primary symptom of bladder cancer. Risk factors include smoking, exposure to carcinogens such as the aromatic amines used in rubber and dye industries, age >55, pelvic radiation and Schistosomiasis resulting in SCC (related to chronic inflammation – so other risks also include UTI, stones etc). Bladder cancer is the most common cancer in Egypt, for the latter reason. Cystoscopy and urinary cytology are key in diagnosis. Low grade tumours are papillary and easy to see on cystoscopy whereas high grade tumours and carcinoma in situ are often difficult to visualise. Resection provides diagnosis and primary treatment in one step.

Answer 429

E. Ureteric colic - This patient has renal colic which classically presents with severe flank pain radiating to the groin. Microscopic haematuria is present in up to 90% of cases. Up to 85% of stones are visible on a plain KUB although urate stones are radiolucent. If the stone is radio-opaque, calcification will be seen within the urinary tract. In pregnancy, a renal USS is first line. The IVP has now been replaced by the CT scan which is the new diagnostic standard. A non-contrast helical (or spiral) CT is preferred due to high sensitivity and specificity and acurately determines presence, site and size of stones. Stones are analysed after they are extracted or when they are expelled to check their composition. It is worth noting that in all females of child bearing age, a urine pregnancy test is necessary to exclude an ectopic pregnancy.

Answer 430

C. Prostatic varices - The examination findings and haematuria at the beginning of the stream, combined with this patient’s history of hypertension suggests prostatic varices as a cause of the bleeding. A varix is an abnormally dilated vein which is prone to rupture and haemorrhage.

Answer 431

G. Trauma - This patient has had a catheter removed two days ago, which accounts for his gross haematuria (instrumentation of the urinary tract causing damage). The diagnosis is clinical based on the presence of the catheter.

Answer 432

D. Bladder cancer - Gross (classically painless) haematuria is the primary symptom of bladder cancer. Risk factors include smoking, exposure to carcinogens such as the aromatic amines used in rubber and dye industries, age >55, pelvic radiation and Schistosomiasis resulting in SCC (related to chronic inflammation – so other risks also include UTI, stones etc). Bladder cancer is the most common cancer in Egypt, for the latter reason. Cystoscopy and urinary cytology are key in diagnosis. Low grade tumours are papillary and easy to see on cystoscopy whereas high grade tumours and carcinoma in situ are often difficult to visualise. Resection provides diagnosis and primary treatment in one step.

Answer 433

A. UTI - This young woman has presented with a UTI (dysuria, frequency, and foul smelling urine). Sexual activity the strongest risk factor for UTIs in women. It is diagnosed with a urine dipstick and microscopic analysis for bacteria, WBC and RBC and urine culture with antibiotic sensitivities. Antibiotic selection should be based on local guidelines or known sensitivities. Uncomplicated UTIs with no known antibiotic resistance can be treated with co-trimoxazole or nitrofurantoin. If there is resistance, a quinolone can be considered such as ciprofloxacin.

Answer 434

C. Henoch_Schonlein purpura - Henoch-Schonlein purpura is the most common vasculitis in childhood and in all cases there is a rash of palpable purpura which are typically non-blanching. If there is no rash, then it is not HSP. They are normally 2-10mm in diameter and are due to the extravasation of blood into the skin. They can occur anywhere on the body but are usually concentrated on the lower extremities. Half of all patients have abdominal pain and arthralgias are commonly present (found in about 80%) and often associated with oedema. The joints most often affected are the knees and ankles. About half will show signs of renal disease such as proteinuria or haematuria. Risk factors for this condition include being male, age 3-15 and history of prior UTI. Complications can occur and the most common cause of death is renal failure. Serum IgA levels may also be elevated.

Answer 435

F. Ureteric colic - This patient has renal colic which classically presents with severe flank pain radiating to the groin. Microscopic haematuria is present in up to 90% of cases and macroscopic haematuria may also be present although this is rare. Dehydration is a strong risk factor for renal stone formation and this man’s job may make him susceptible to inadequate fluid intake. A low urine output can lead to higher levels of urinary solutes, therefore leading to stone formation. Up to 85% of stones are visible on a plain KUB although urate stones are radiolucent. If the stone is radio-opaque, calcification will be seen within the urinary tract. In pregnancy, a renal USS is first line. The IVP has now been replaced by the CT scan which is the new diagnostic standard otherwise. A non-contrast helical (or spiral) CT is preferred due to high sensitivity and specificity and acurately determines presence, site and size of stones. Stones are analysed after they are extracted or when they are expelled to check their composition. It is worth noting that in all females of child bearing age, a urine pregnancy test is necessary to exclude an ectopic pregnancy.

Answer 436

E. Post infectious glomerulonephritis - This is post-infectious glomerulonephritis caused by group A beta-haemolytic streptococcus with renal endothelial cell damage. Serological markers would expect to show antibodies to streptococcus and low complement and treatment here is with antibiotics. The high ASOT (antistreptolysin O antibody titres) indicates post-streptococcal GN. There may also be positive anti-Dnase and antihyaluronidase in post-streptococcal GN.

Answer 437

M. Renal cell carcinoma - Renal cancer arising from the parenchyma/cortex is known as renal cell carcinoma. Clear cell renal cell carcinoma accounts for most primary renal cancers. They are often asymptomatic and diagnosed incidentally like on imaging when localised malignant looking renal masses are seen. Surgery for early local disease (which is diagnosed in more than half) can be curative in up to 90%. Renal masses are usually only symptomatic in late disease. The classic triad is of haematuria, flank pain and an abdominal mass – this is only seen in 10%. Uncommonly, a patient may present with symptoms of metastatic disease such as bone pain or respiratory symptoms. Symptoms, if present, also include abdominal pain, oedema/ascites from IVC disruption and scrotal varicocele in males. Risk factors include: smoking, male gender, living in developed countries, obesity, hypertension, FH, high parity and ionising radiation.

Answer 438

K. Polycystic kidney disease - This sounds like autosomal dominant polycystic kidney disease. There may like in this case be a FH, of PKD or ESRF or cerebrovascular events (intracranial berry aneurysms in the circle of Willis and subsequent SAH – the bleed in the brain). Patients may have haematuria (which can be gross), palpable kidneys and symptoms of a UTI which is common in those with the condition. Hypertension and flank pain are also commonly seen. Hepatosplenomegaly may also be found. A renal ultrasound is the first test to order when the diagnosis is suspected. If the ultrasound is equivocal, a CT scan can be done of the abdomen and pelvis.

Answer 439

F. Pyelonephritis - UTIs involving the renal parenchyma typically presents with fever. Acute onset fever with urinary symptoms and flank pain point to acute pyelonephritis. Urinalysis is highly sensitive but not very specific. Pregnancy is a risk factor for complicated disease as the enlarging uterus compresses the ureters and hormonal changes increase the likelihood of obstructive uropathy. In uncomplicated pyelonephritis, the most common cause is E. coli and gram stain will typically reveal gram negative rods, either E. coli, Proteus or Klebsiella. Gram positive cocci that could be implicated include enterococci and staphylococci. Older patients can often also present non-specifically. Treatment should start before culture results are received to prevent the patient from deteriorating, with empirical antibiotics.

Answer 440

L. Renal tuberculosis - GU TB common presents with symptoms of dysuria, haematuria and urinary frequency. Some 20-30% of patients may also be asymptomatic, and sometimes extensive renal destruction may have occured by the time it is diagnosed. Genital TB in men may present as a scrotal mass and in women there may be pelvic pain. Diagnosis relies on culturing TB from morning urine samples (3 samples are recommended) or biopsy of the lesion. CXR in these patients is abnormal in most cases. Up to 90% are TST positive on Mantoux testing. Extrapulmonary TB can also include pericardial, abdominal, disseminated, skeletal, CNS and pleural TB. Homelessness here is an epidemiological risk factor. Other risks include birth in high TB countries (Asia, Latin America and Africa in particular) and exposure to TB.

Answer 441

J. Renal artery stenosis - Renal artery stenosis is basically narrowing of the renal artery. It occurs typically due to atherosclerosis or fibromuscular dysplasia. The presentation tends to be with accelerated or difficult to control hypertension. Acute kidney injury can be seen after starting an ACE inhibitor or an angiotensin II receptor antagonist which this patient has been prescribed. The afferent arteriole is stenosed in RAS and angiotensin II is needed to maintain GFR by constricting the efferent arteriole. ACE inhibitors prevent conversion of angiotensin I to angiotensin II, which is needed to maintain renal perfusion pressure in those with RAS. The result is this patient has acute renal failure and has developed uraemia. The metabolic acidosis is also as a result of renal failure. There may not be any clinical consequences of RAS – just because someone’s renal arteries are narrowed does not mean they are suffering worsening kidney function, although this may be the case, especially after blockade of the renin-angiotensin system. A definitive diagnosis is made on imaging, where there is some controversy on what is most appropriate to use. USS is safe and non-invasive but the sensitivity and specificity is low. CT/MR angiography has the risk of contrast nephropathy and nephrogenic systemic fibrosis. Conventional angiography (the best test available) has the risk of bleeding and emboli as well as contrast related risks already mentioned.

Answer 442

C. IV dextrose - This patient is profoundly hypoglycaemic (symptoms of hypoglycaemia are present when glucose drops

Answer 443

H. Commence CPR - This patient has cardiac arrest and the first thing you need to do is commence CPR. It is thought to work by raising intrathoracic pressure and providing direct cardiac compression. 30 compressions (at 100/min) and 2 breaths for a total of 5 cycles (2 minutes), makes up one cycle of CPR. Compressions are the first priority, breaths may follow but compressions are paramount. Further management depends on whether the patient has a shockable rhythm (pulseless CT or VF) or non-shockable rhythm (PEA or asystole). Do you know when a precordial thump can be used?

Answer 444

A. Intravenous naloxone - Opiate OD signs include CNS depression, miosis and apnoea. Finding small constricted pupils in someone who is unconscious is highly indicative. Naloxone is indicated both therapeutically and diagnostically. If there is a response, then it is diagnostic. Another diagnosis should be sought if the patient is unresponsive. IV is the preferred route of administration although naloxone can be given IM or SC if IV access cannot be established. Ventilatory support is key with 100% oxygen. You can check out Toxbase for a full database on poisons and treatments.

Answer 445

K. CT scan brain - A CT head is indicated here in this possible SAH. This may show hyperdense areas in the basal cisterns, major fissures and sulci.

Answer 446

E. Endotracheal intubation - This is life threatening asthma that has not responded to therapy. There is cyanosis and respiratory acidosis despite the tachypnoea of 50/min. This patient is clearly too dyspnoeic to speak and oxygen saturation may be quite low. Pulsus paradoxus may also be observed. This patient should be intubated (mechanical ventilation is required) and transfered to ICU and given supplemental oxygen. IV corticosteroids should also be used and heliox therapy considered (helium-oxygen).

Answer 447

E. Extensive necrosis - Paracetamol OD can occur after a single large OD or repeated ODs. Often, the patient is asymptomatic at initial presentation but if untreated may cause liver injury over the 2-4 days after ingestion, including fulminant liver failure. Paracetamol is the most frequent intentional OD drug in this country. The risk of liver damage is increased after taking drugs which induce CYP 450. Inducers include St John’s wort, barbiturates, phenytoin, tetracycline, chronic alcohol use and carbamazepine. A serum paracetamol level is important to order as early as possible, but at the earliest 4 hours post-ingestion.Treatment if indicated is with N-acetylcysteine with the level based on a paracetamol treatment graph.

Answer 448

F. Hepatocellular carcinoma - Patients with cirrhosis, especially those with alcoholic liver disease, are at a high risk of developing HCC. Cirrhosis is irreversible so despite stopping drinking, he is still at risk of HCC (hepatoma). Patients with cirrhosis should be screened for HCC with serum AFP and USS at 6 month intervals.

Answer 449

G. Cholecystitis - Cholecystitis is acute GB inflammation caused by an obstruction at the cystic duct. It occurs as a major complication of gallstones and classically presents with RUQ pain and fever. Gallstones in EMQs classically involves the Fs (Fat, Forty, Female, Fertile, Fair). USS is the definitive initial investigation. HIDA scanning and MRI may help if the diagnosis remains unclear. Treatment is with cholecystectomy.

Answer 450

I. Carcinoma of bronchus - The history of smoking and weight loss point to a bronchial carcinoma. Initial investigation is with a CXR. Diagnosis relies on pathological confirmation from a tissue sample, often obtained from bronchoscopy. First line treatment aims at surgical resection if possible. Small cell lung cancer is treated with chemotherapy and is associated with SIADH and ectopic ACTH. Non-small cell lung cancer is more often associated with clubbing. Squamous cell carcinoma is associated with PTHrp release and is treated with radiotherapy. Adenocarcinomas are usually located peripherally in the lung and are more common in non-smokers although most cases are still associated with smoking. The paraneoplastic syndromes may include Lambert-Eaton myasthenic syndrome.

Answer 451

C. Angiotensin converting enzyme inhibitor - A dry cough is a side effect of ACE inhibitors due to the build up of bradykinin which is normally degraded by ACE. ARB such as losartan will be indicated in this case. ARBs are insurmountable antagonists of AT1 receptors for angiotensin II, preventing its renal and vascular effects.

Answer 452

F. Sarcoidosis - Sarcoidosis is a chronic multisystem disease with an unknown aetiology. The painful (mauve) nodules are erythema nodosum. Lupus pernio is another typical skin manifestation of sarcoidosis presenting with indurated plaques with discoloration on the face. Parotid enlargement is a classic feature (involvement of exocrine glands). The dry cough and SOB on exertion indicate pulmonary involvement. CXR will typically show bilateral hilar lymphadenopathy and CXR findings are used in the staging of disease. Additionally, serum calcium and ACE levels may be raised. A transbronchial biopsy is essential for diagnosis in most cases and shows the presence of non-caseating granulomas. Black people have a higher lifetime risk of sarcoidosis, as do those of Scandinavian origin. The mainstay of treatment for severe disease involves systemic corticosteroids.

Answer 453

E. Asthma - SOB and the cough, which may wake the patient from sleep combined with the patient’s age and progessive course suggest asthma. Examination can show an expiratory wheeze but may be normal and treatment is step-wise based on BTS guidelines. It is worth noting that in severe exacerbations, the chest may be silent. Night symptoms occur in more severe asthma and symptoms can be exacerbated by exercise. Diagnosis is supported by PEFR variation of at least 20% over 3 days in a week over several weeks or an increase of at least 20% to treatment. Stepwise treatment is outlined below. Look up the BTS guidelines for more information. Step 1: SABA PRN, Step 2: Plus low-dose inhaled corticosteroids (ICS) , Step 3: Plus LABA, Step 4: Increase dose of ICS or add LTRA, SR theophylline or beta agonist tablet, Step 5: Daily steroid tablet and maintain ICS with specialist care.

Answer 454

J. Bronchiectasis -Bronchiectasis is permanent bronchi dilatation due to bronchial wall damage and loss of elasticity. It is often as a consequence of recurrent/severe infections and most present with chronic productive mucopurulent cough. The most common identifiable cause is CF. Chest CT is the diagnostic test. Diagnosis is aided by sputum analysis. Have a think about what you would expect to hear on ascultation of the chest.

Answer 455

B. Anorexia nervosa - Anorexia nervosa often occurs in women which is a key risk factor. Puberty/adolescence and obsessive personality traits are additional risks. There is a higher incidence reported in western cultures (presumably skinny models on TV, adverts etc...) and studies on immigrants moving to a western culture exhibited a higher incidence. There is also postulated to be a genetic contribution from twin studies.Those who in higher socioeconomic classes are more affected. BMI is usually

Answer 456

F. Infestation with helminths - The only option on the list that would give eosinophilia is infection with helminths. There is a risk factor here, having returned from Africa where the sanitation, hygiene and agricultural practices may leave much to be desired. Testing for stool ova and parasites will be needed to see exactly which helminth is causing this infection, although this is not very sensitive for strongyloides larvae. IgG serology can be used with >95% sensitivity if stool samples are negative in the case of strongyloides. This could be strongyloides or ascariasis or a rarer organism. Treatment will be with antihelminths depending on the organism. Ivermectin for strongyloides is the drug of choice. Other antihelmintic agents include albendazole, mebendazole and pyrantel pamoate.

Answer 457

A. Malignancy - This person likely has lung cancer which has resulted in the significant weight loss of 10kg. This is post-obstructive pneymonia which is common in lung cancer patients and is caused, most of the time, by a large and centrally obstructing tumour. It is essential to relieve this obstruction in this case and many techniques can be tried. There is also bone pain here in the lower spinal column which is due to metastases. The prognosis here is not good.

Answer 458

E. Malabsorption - There is frequent loose stools here and abdominal pain. Combined with the IDA, this points to malabsorption. This could well be a presentation of coeliac disease – IDA is one of the most common clinical presentations and abdominal pain and diarrhoea are common. Coeliac disease is a systemic autoimmune condition triggered by dietary gluten peptides found in grains. It is a relatively common condition. The only treatment is a strict gluten-free diet for life.

Answer 459

M. HIV - HIV is a retrovirus and there are two types, HIV 1 which is the main virus responsible and HIV 2 which is restricted to parts of West Africa. Weight loss is common in HIV and if more than 10% body weight is lost of BMI reduces to 18.5, this is an indication of more severe immunocompromise. Weight loss in HIV may result from malnutrition, co-existent TB infection or HIV wasting syndrome, the latter being an AIDS defining illness. Generalised lymphadenopathy is also common and is characterised by the painless enlargement of 2 more more non-contiguous sites of >1cm for >3 months. Neutropenia is also seen due to CD4 deficiency and thrombocytopenia may also be seen along with an anaemic picture. There are WHO (stage 1-4) and CDC criteria used in clinical staging. This patient needs to have a CD4 count, HBV and HCV screen, VDRL (syphilis), tuberculin skin test (TB) and CXR. HIV viral load will also be assessed. Prophylaxis and immunisations should be considered against infections such as hepatitis, influenza, PCP and TB. When to initiate HAART depends on the clinical stage, CD4 and co-morbidities. This patient will need to be started on HAART. Classes of antiretrovirals include NRTIs, NNRTIs, protease inhibitors, fusion inhibitors and integrase inhibitors.

Answer 460

C. Subcutaneous low molecular weight heparin - Women developing a DVT during pregnancy can be treated with heparin or LMWH. However, LMWH is preferred due to more dependable pharmacokinetics. The agents you will commonly hear include enoxaparin, dalteparin and tinzaparin.

Answer 461

G. Reassure and discharge - This is likely to be a musculoskeletal injury in an otherwise fit, young man. Eccentric contraction causes more frequent injury. Inadequate warm-up before exercise can predispose to muscle injuries.

Answer 462

G. Reassure and discharge - A popliteal cyst is an accumulation of synovial fluid which in this case has resulted from this woman’s arthritis. This is an accumulation of synovial fluid behind the knee, usually in response to injury or inflammation. It will self-resolve but the underlying cause should be addressed i.e. arthritis. First line treatment for grade 1 or 2 injuries is with RICE: rest, ice, compression and elevation followed by gentle mobilisation. Adjunctive analgesia can be offered with paracetamol. Treatment is conservative, particularly if asymptomatic. Surgery is only indicated in those with extensive symptoms where conservative and percutaneous treatments have failed. Corticosteroid injections (intra-articular) can also be considered.

Answer 463

B. Fondapariunux - A factor Xa antagonist is preferred if the patient has or has had heparin-induced thrombocytopenia. If the patient has a low BP then systemic thrombolysis would be indicated to prevent possible cardiac arrest.

Answer 464

C. Subcutaneous low molecular weight heparin - Patients with recurrent thromboembolism despite on warfarin should be given heparin or LMWH. LMWH is again the primary option for reasons discussed. They should be given for at least 5 days until INR is between 2 and 3 (the target INR). Fondaparinux would be used instead if there was a high chance of delayed HIT. Warfarin is continued and efforts must be made to work out how this patient has developed a DVT despite on warfarin therapy. There may be subtherapeutic dosing, the presence of a malignancy or antiphospholipid syndrome. If there is documented thrombosis progression despite adequate anticoagulation, without HIT and other causes are excluded, an IVC filtre may be indicated but evidence of its efficacy have been debated by recent studies.

Answer 465

D. Fibroadenosis - Fibrocystic breasts are characterised by ‘lumpy’ breasts associated with pain which fluctuates with the menstrual cycle (it is worse during the luteal phase of menses). Risk factors include obesity, nulliparity, HRT and late onset menopause and first childbirth. It is a diagnosis of exclusion, and is considered to be an exaggerated physiological phenomenon rather than a disease (54% of clinically normal breasts are found on autopsy to have fibrocystic changes). Symptoms typically arise between the 3rd and 4th decases of life. There may also be a nipple discharge, which can be suspicious if bloody or profuse etc and may indicate the presence of an intraductal papilloma, cancer, or duct ectasia. Cysts can be aspirated if symptomatic (asymptomatic or small ones do not require intervention). If the aspirate is straw coloured and completely aspirated, there is no need for cytology, but if the aspirate is bloody, cytology or biopsy is needed to exclude cancer. There is improvement of mastalgia and cysts at menopause and until then it runs a chronic relapsing course.

Answer 466

G. Fibroadenoma - This is a fibroadenoma which tends to be asymptomatic and found incidentally, typically in a patient

Answer 467

H. Breast abscess - Breast abscess presents with mastalgia and fever. Breast infection typically affects women who are lactating and the most commonly implicated pathogen is staphylococcus aureus. The painful, hard and red lump indicates the development of an abscess. Antibiotic therapy is indicated with surgical intervention such as aspiration and drainage with possible duct excision. Prompt management of mastitis when it presents will usually lead to a good timely resolution and prevent the development of complications such as an abscess. An USS can help to identify the underlying abscess which usually forms a hypoechoic lesion. Needle aspiration can be used both therapeutically and diagnostically and can be guided by ultrasound.

Answer 468

F. Gynaecomastia - This is a boy who has enlarged breasts. Normal to see gynaecomastia in puberty. Other causes include liver disease and as a side effect of drugs such as digoxin, spironolactone and cimetidine.

Answer 469

C. Duct ectasia - Duct ectasia happens because the lactiferous duct gets blocked. Ectasia means widening. It can mimic breast cancer as the discharge can be bloody sometimes and signs can include nipple inversion. The green nipple discharge is typical in EMQs. It is a self limiting condition. 1) Fibrocystic breasts are characterised by ‘lumpy’ breasts associated with pain which fluctuates with the menstrual cycle (it is worse during the luteal phase of menses). Risk factors include obesity, nulliparity, HRT and late onset menopause and first childbirth. It is a diagnosis of exclusion, and is considered to be an exaggerated physiological phenomenon rather than a disease (54% of clinically normal breasts are found on autopsy to have fibrocystic changes). Symptoms typically arise between the 3rd and 4th decases of life. There may also be a nipple discharge, which can be suspicious if bloody or profuse etc and may indicate the presence of an intraductal papilloma, cancer, or duct ectasia. Cysts can be aspirated if symptomatic (asymptomatic or small ones do not require intervention). If the aspirate is straw coloured and completely aspirated, there is no need for cytology, but if the aspirate is bloody, cytology or biopsy is needed to exclude cancer. There is improvement of mastalgia and cysts at menopause and until then it runs a chronic relapsing course.

Answer 470

B. Abdominal ultrasound - This is clubbing due to cirrhosis as a result of chronic alcohol excess. Ultrasound here is the only feasible option on the list and can detect advanced cirrhosis and possible signs of portal hypertension, such as ascites, splenomegaly and increased portal vein diameter. The ultrasound may show a nodular liver surface, the liver may itself be shrunk due to cirrhosis and the left hand side of the liver may be hypertrophied. USS findings with a good clinical suspicion is enough for diagnosis.

Answer 471

D. Chest x-ray - Idiopathic pulmonary fibrosis (previously known as Cryptogenic fibrosing alveolitis) progresses over several years and is characterised by pulmonary scar tissue formation and dyspnoea. Patients complain of a non-productive cough and typically reproducible and predictable SOB on exertion. This man’s work means he comes into contact with small organic or inorganic dust particles which is thought to be implicated in the cascade of events leading to IPF. Another risk factor is cigarette smoking which significantly increases the risk of IPF. The mean age of diagnosis is 60-70. CXR here will show reticular opacities. A high resolution CT scan can also be done if it was an option on this list.

Answer 472

A. Echocardiogram - The only cardiac investigation here is an echocardiogram. Cardiovascular causes of clubbing include cyanotic congenital heart disease, infective endocarditis and atrial myxoma. This could well be Eisenmenger’s syndrome caused by shunt reversal.

Answer 473

G. Colonoscopy - This woman has inflammatory bowel disease (which is a cause of clubbing), which by the history is more likely to be UC than CD, whereby the mainstay of treatment is with 5-ASA. A colonoscopy is required to assess the extent of disease and for a definitive diagnosis. Biopsy in CD will show transmural granulomatous inflammation. CD can affect the whole GIT but favours the TI and proximal colon and is macroscopically characterised by skip lesions. UC on the other hand is characterised by the presence of crypt abscesses, which is pathognomic. CD risk is increased 3-4 fold by smoking whereas smoking seems protective in UC. The mainstay of treatment in CD is with steroids and azathioprine to revent relapses and for those suffering side effects of steroid treatment. TNF-alpha inhibitors also have a role. Surgery in CD is only indicated in a small number of patients who bleed, for bowel perforation and cases of complete obstruction. The aim is to rest distal disease by temporarily diverting faecal flow.While a stool culture may be useful in ruling out infectious gastroenteritis it will not provide the diagnosis here.

Answer 474

C. Bronchoscopy - The history of smoking and weight loss point to a bronchial carcinoma. Whilst the initial investigation is with a CXR, diagnosis relies on pathological confirmation from a tissue sample, often obtained from bronchoscopy. Non-small cell lung cancer is more often associated with clubbing.

Answer 475

C. Tuberculosis - This patient has pulmonary TB. Risk factors include HIV infection, exposure to infection and returning from or being born in a high risk region such as Somalia, or other areas of Africa, Asia and Latin America. The night sweats here, weight loss and haemoptysis are all suggestive. The CXR finding of a cavitating lesion and upper zone changes are also consistent with TB. If TB is suspected, the patient should be placed in isolation and 3 sputum samples cultured for AFB being the gold standard of diagnosis. Culture takes several weeks so sputum smears will be done before culture results are known. Interferon-gamma release assays (IGRAs) are now used by some hospitals to rapidly determine a patient’s TB status. All patients who have TB should be tested for HIV within 2 months of diagnosis.

Answer 476

J. Carcinoma of bronchus - The weight loss, respiratory symptoms of haemoptysis and the history of smoking makes this likely to be bronchial carcinoma. Lobar collapse here may be a consequence of obstruction. First line treatment aims at surgical resection if possible. Small cell lung cancer is treated with chemotherapy and is associated with SIADH and ectopic ACTH. Non-small cell lung cancer is more often associated with clubbing. Squamous cell carcinoma is associated with PTHrp release and is treated with radiotherapy. Adenocarcinomas are usually located peripherally in the lung and are more common in non-smokers although most cases are still associated with smoking. The paraneoplastic syndromes may include Lambert-Eaton myasthenic syndrome.

Answer 477

F. Bronchiectasis - Bronchiectasis is permanent bronchi dilatation due to bronchial wall damage and loss of elasticity. It is often as a consequence of recurrent/severe infections and most present with chronic productive mucopurulent cough. The most common identifiable cause is CF but this history here of pertussis is responsible in this instance. Chest CT is the diagnostic test. Diagnosis is aided by sputum analysis. Not only should the GP have this diagnosis in mind but should know whether the patient is actually clubbed, instead of ‘thinking’ she is clubbed. Bronchiectasis is a cause of clubbing.

Answer 478

I. Pulmonary embolism - The underlying pathophysiology of PE is based on Virchow’s triad. SOB is a common symptom and there may also be pleuritic chest pain and haemoptysis. Strong risk factors include recent surgery, DVT, obesity, prolonged bed rest, malignancy, previous VTE, pregnancy and the thrombophilias such as factor V Leiden. The oral contraceptive pill is associated with an increased risk of VTE but is a weak risk factor. However, the rest of this history still makes the diagnosis in this case. CXR may be normal like in this case or may have findings suggestive of PE such as band atelectasis, hemidiaphragm elevation, Fleischner’s sign, Westermark’s sign and Hampton hump. ECG may be normal, or may show tachycardia, new RAD, new RBBB or the classical S wave in I, Q wave with T inversion in III. Various clinical probability scores exist for PE and D-dimer can be used to exclude PE as a diagnosis.

Answer 479

K. Thrombocytopenia - HIV is implicated in idiopathic thrombocytopenic purpura, which is a condition of abnormally low platelet count of unknown cause. This question makes this dead easy by giving you the platelet count as 28 x109/L and all you need to do is to appreciate that a count under 150 x109/L is defined as thrombocytopenia. As well as the count, the patient has obvious symptoms of thrombocytopenia here with bruising, haemoptysis and epistaxis.

Answer 480

H. Gilbert's syndrome - Gilbert’s occurs in an asymptomatic patient, often as an incidental finding or mild jaundice occuring in adolescence/young adult age. There is elevated unconjugated BR with other liver tests being normal. The blood smear is also normal with normal reticulocyte count, and normal Hb indicating that this is not due to haemolysis. It is a common syndrome and is not really a disease, more a physiological variant. No treatment is needed and this condition is due to decreased UDPGT activity leading to decreased conjugation of unconjugated bilirubin, leading to elevated levels.

Answer 481

A. Autoimmune haemolytic anaemia - This woman is complaining of symptoms of anaemia with SOB and pallor. Haemolytic anaemia can be either hereditary or acquired. Hereditary can be divided into 3 broad groups – either inherited defects in the membrane such as hereditary spherocytosis, enzyme deficiencies such as G6PDH deficiency or abnormal Hb production such as that seen in sickle cell anaemia and thalassaemia. Acquired can be either immune or non-immune in cause. Autoimmune HA here is due to autoantibodies, which occurs most often as part of another autoimmune process like SLE or RA or related to a lymphoproliferative disorder such as CLL. FBC here shows a low Hb consistent with the diagnosis, though it is important here to also request MCHC and reticulocyte count. Both of these would be increased in haemolytic anaemia. Increased breakdown of haem leads to an unconjugated hyperbilirubinaemia, though not >70-85, which would indicate some degree of liver impairment as well. LDH would also be raised, which can be helpful if there is no concurrent tissue damage. Haptoglobin (which binds free Hb) will also be low, and these two markers combined is 90% specific for HA. The giveaway for AIHA here is the positive DAT or Coombs’ test. This test detects IgG or complement bound to RBCs (hence a positive result suggests an immune cause of HA). The patient’s RBCs are washed and mixed with antiserum for IgG and C3d. The presence of IgG often indicates the presence of a warm antibody, whereas C3d suggests a cold antibody.

Answer 482

C. Alcoholic cirrhosis - This is a case of alcoholic cirrhosis. AST and ALT are elevated in all forms of alcoholic liver disease, both to 2, which is seen in about 70% of cases, and AST is usually above ALT level. The elevated ALP here may represent cholestasis. GGT is a more sensitive marker than AST or ALT for heavy alcohol use and elevation here suggests the cause is alcohol. The anaemia here in alcoholic liver disease can be due to many causes like iron deficiency, folate deficiency and hypersplenism. Thrombocytopenia may be secondary to alcohol induced BM suppression, folate deficiency or hypersplenism. Those with advanced cirrhosis frequently are also hyponatraemic and hypokalaemia is also seen. Urea and creatinine is normal in this patient but elevation may suggest the presence of hepatorenal syndrome. Cirrhosis is the end stage of chronic liver disease and the abdominal distension may be a sign of ascites.

Answer 483

F. Viral hepatitis A - Hepatitis A is primarily transmitted via the faecal-oral route. After the virus is consumed and absorbed, it replicates in the liver and is excreted in the bile (to be re-transmitted). Transmission usually precedes symptoms by about 2 weeks and patients are non-infectious 1 week after onset of jaundice. The history can reveal risk factors such as living in an endemic area, contact with an infected person, homosexual sex or a known food-borne outbreak. This is classically, in EMQs, associated with shellfish which is harvested from sewage contaminated water. If the patient has other liver diseases such as HBV or HCV or cirrhosis then there is a higher risk of fulminant HAV infection. The clinical course of HAV consists of a pre-icteric phase, lasting 5-7 days, consisting characteristically of N&V, abdominal pain, fever, malaise and headache. Rarer symptoms may be present such as arthralgias and even severe thrombocytopenia and signs that may be found include splenomegaly, RUQ tenderness and tender hepatomegaly as well as bradycardia. The icteric phase is characterised by dark urine, pale stools, jaundice and pruritis. When jaundice comes on, the pre-icteric phase symptoms usually diminish, and jaundice typically peaks at 2 weeks. However, a fulminant course runs in

Answer 484

G. Cholelithiasis - Gallstones, or cholelithiasis, is highly prevalent but most are asymptomatic. Symptoms occur when the cystic or bile duct get obstructed or when the gallstones erode through the gallbladder. The biliary pain here is caused by transient cystic duct obstruction or passage/obstruction through the bile duct. The typical presentation is of RUQ or epigastric pain often after food which increases in intensity and lasts for several hours. The jaundice here suggests choledocholithiasis (the presence of at least one gallstone in the CBD). Gallstones in EMQs classically involves the Fs (Fat, Forty, Female, Fertile, Fair) which reflects the risk factors such as older age, female sex, obesity, rapid weight loss, pregnancy and drugs. USS is the definitive initial investigation. HIDA scanning and MRI may help if the diagnosis remains unclear. Definitive treatment is with laparoscopic cholecystectomy for those with symptoms. Complications of gallstones include cholecystitis, cholangitis and pancreatitis and symptoms may indeed overlap between these conditions.

Answer 485

L. Hypoglycaemia - Heavy alcohol consumption decreases hepatic production of glucose, putting this person at risk of hypoglycaemia. This patient is suffering from hypoglycaemia which is present when glucose drops

Answer 486

A. Pancreatitis - This patient has acute pancreatitis. She has vomited and is describing likely epigastric pain. This classically radiates around to the back which is relieved in the fetal position and is worse with movement. This patient is likely to have gallstones as the underlying cause, which is also causing an obstructive jaundice and her previous episode. Complicated haemorrhagic pancreatitis may exhibit Cullen’s sign, Grey-Turner’s sign and Fox’s sign. Make sure you know what these are and you are familiar with the other causes of acute pancreatitis. Those caused by hypocalcaemia may display Chvostek’s sign and Trousseau’s sign. Key to diagnosis is serum amylase or lipase levels which are massively elevated. Prognostic criteria are outlined in Ranson’s criteria applied on admission and after 48 hours, or the modified Glasgow score which you can find in your Oxford Handbook. An abdominal CT is however the most sensitive and specific study and findings may include enlargement of the pancreas with irregular contours, necrosis, pseudocysts and peripancreatic fat obliteration. For interest, urinary trypsinogen-2 is now considered a better screening test than amylase but is not currently clinically used.

Answer 487

E. Atrial fibrillation - Atrial fibrillation is an irregular atrial arrhythmia with a broad list of risk factors such as coronary artery disease, heart failure, hypertension, DM and thyroid disorders. This patient has two of these risk factors. AF affects some 5% of those older than 69 years old. The presentation of SOB and palpitations is consistent with the diagnosis and the irregular heart sounds suggests AF as the cause. ECG would show absent P waves, a fibrillatory baseline and irregularly irregular complexes. Most that present with acute AF do not need immediate treatment, unless they have developed haemodynamic compromise, which requires immediate DC cardioversion. Most will require medical therapy with drugs to control the ventricular rate, restore and maintain sinus rhythm and prevent the occurence of thromboembolic events. Do you know which drugs are used and are you familiar with treatment guidelines for this common condition?

Answer 488

G. - Subdural haematoma - A subdural occurs due to blood collecting between the dura mater and the arachnoid mater surrounding the brain. It may be arterial or venous although is most often venous. The disease runs a varied course and the presentation occurs on a spectrum from asymptomatic to herniation syndromes. There is neurological deficit evident so surgery will be indicated here. The cause is trauma and this man will have suffered a head injury due to his RTA. It is important in the examination to look for signs of trauma such as scalp abrasions and bruises. Surgical options include twist-drill craniotomy with drainage (a bedside procedure where a hand drill is used to gain access to the subdural space and then a catheter is placed to act as a drain). Standard craniotomy is also an option, as is the creation of a burr hole. Remember that extradural haematomas classically have a ‘lucid interval’ and occur in younger patients, usually with an associated skull fracture, and CT of the haematoma does not cross suture lines.

Answer 489

N. Oesophageal varices - Oesophageal varices occurs as a result of portal hypertension which is a complication of cirrhosis which this patient has. Signs of chronic liver disease may be present such as spider naevi, ascites, caput medusa (vascular collaterals in the abdominal wall), jaundice etc. Splenomegaly is also commonly found and hence patents often have thrombocytopenia and anaemia as a result. The bleeding carries a significant morbidity and mortality, and beta-blockers and/or endoscopic ligation can prevent variceal bleeding prophylactically (though beta blockers are not be used in the acute setting of a variceal bleed – do not get confused here!). Oesophageal varices are basically dilated veins and these can be seen on OGD. Worldwide, HBV and HCV are also major causes of cirrhosis, leading to varices and HIV co-infection can rapidly speed up the progression to cirrhosis in chronic liver failure. The size of the varices is the key predictor of haemorrhage. Acute bleed can be managed with resuscitation, terlipression (DDAVP)/somatostatin analogues/endoscopic ligation. Additionally, a shunt can be deployed and antibiotic prophylaxis started.

Answer 490

M. Fibroadenoma - This sounds like a fibroadenoma which tends to be asymptomatic and found incidentally, typically in a patient

Answer 491

L. Breast abscess - Breast abscess presents with mastalgia and fever. Breast infection typically affects women who are lactating and the most commonly implicated pathogen is staphylococcus aureus. The painful red mass indicates the development of an abscess. Antibiotic therapy is indicated with surgical intervention such as aspiration and drainage with possible duct excision. Prompt management of mastitis when it presents will usually lead to a good timely resolution and prevent the development of complications such as an abscess. An USS can help to identify the underlying abscess which usually forms a hypoechoic lesion. Needle aspiration can be used both therapeutically and diagnostically and can be guided by ultrasound.

Answer 492

E. Carcinoma of the breast - Patient’s wth breast cancer, on examination tend to demonstrate a firm hard lump which may be associated with axillary lymphadenopathy, skin changes such as the orange peel (peau d’orange), dimpling seen here and nipple changes/discharge. Skin changes are associated with locally advanced cancer. Many breast cancers are also diagnosed on routine mammography which can show microcalcifications, in the absence of a palpable mass. MRI is more sensitive but less specific so is recommended only in patients who are at high risk, such as BRCA1/2 mutation, history of chest radiation or certain syndromes like Cowden’s or Li-Fraumeni. FNA is also useful in rapid diagnosis, although is operator dependent when it comes to how sensitive and specific it is, and a core biopsy is preferred in most cases for diagnosis as it can differentiate pre-invasive and invasive disease. Treatment is MDT involving surgeons, oncologists, radiation oncologists etc.

Answer 493

J. Fibroadenosis - Fibrocystic breasts are characterised by ‘lumpy’ breasts associated with pain which fluctuates with the menstrual cycle (it is worse during the luteal phase of menses). Risk factors include obesity, nulliparity, HRT and late onset menopause and first childbirth. It is a diagnosis of exclusion, and is considered to be an exaggerated physiological phenomenon rather than a disease (54% of clinically normal breasts are found on autopsy to have fibrocystic changes). Symptoms typically arise between the 3rd and 4th decases of life. There may also be a nipple discharge, which can be suspicious if bloody or profuse etc and may indicate the presence of an intraductal papilloma, cancer, or duct ectasia. Cysts can be aspirated if symptomatic (asymptomatic or small ones do not require intervention). If the aspirate is straw coloured and completely aspirated, there is no need for cytology, but if the aspirate is bloody, cytology or biopsy is needed to exclude cancer. There is improvement of mastalgia and cysts at menopause and until then it runs a chronic relapsing course.

Answer 494

C. Sebaceous cyst - The central punctum makes this diagnosis. This has become inflamed in this case ad is expressing a foul-smelling keratinised discharge as it has become infected. They can be caused by blockage of sebaceous glands.

Answer 495

D. Angiotensin converting enzyme inhibitor therapy - This patient has worsening LV function in line with heart failure. First line treatment is with an ACE inhibitor which reduces morbidity and mortality associated with the condition. All patients with LV dysfunction should receive ACE inhibitors, whether symptomatic or not. Caution should be taken if the patient has renal impairment, cardiogenic shock or hyperkalaemia. All patients with chronic heart failure will also receive a beta blocker such as carvedilol.

Answer 496

F. Weight reduction and increased physical activity - This woman is obese (BMI greater than or equal to 30) and needs to lose weight. Central obesity has a greater correlation with co-morbidities than peripheral obesity so arguably waist circumference is a better indicator of risk than body mass index. The mainstay of treatment is with diet and exercise. This patient is obese and drug therapy can be considered as an adjunct. This is primarily with orlistat which inhibits fat absorption by inhibiting lipases. For weight loss, the recommended intake is 1200-1500 kcal/day for men and 1000-1200kcal/day for women, producing a 500-1000kcal/day deficit.

Answer 497

A. Aspirin therapy - A TIA is colloquially called a ‘mini stroke’ with symptoms typically lasting under an hour. An antiplatelet drug such as aspirin is effective secondary prevention if the patient is not already anticoagulated. The patient will be anticoagulated if they have a likely or known cardioembolic source such as AF. Clopidogrel is an alternative in those who do not tolerate aspirin.

Answer 498

C. Weight reduction and metformin - This patient has DM. Symptomatic patients need a single random blood glucose of >11.1 or single fasting glucose of >7. Asymptomatic patients need two separate elevated readings for a diagnosis. Alternatively if there are borderline results, an OGTT can be conducted to see if plasma glucose is raised >11.1 two hours after an oral glucose load of 75g. A patient is said to have impaired fasting glucose if fasting glucose falls between 6.1-6.9. Impaired glucose tolerance is present if plasma glucose 2 hours after oral glucose load in OGTT falls between 7.8-11.0. First line intervention in this situation is diet and lifestyle advice and changes. Metformin will be added if there is no adequate response. In terms of this question, this option will reduce cardiovascular risk the most for this patient. Metformin is a biguanide and suppresses hepatic glucose production.

Answer 499

J. Corticosteroids - Side effects of corticosteroids are due to exaggerated normal physiological actions. This lady with a long history of asthma is likely either taking inhaled corticosteroids or on oral therapy. An inhaled corticosteroid is used if a patient requires their reliever more than twice a week. Smoking, current or previous, reduces the effectiveness of inhaled corticosteroids so higher doses may be needed. In asthma, they reduce airway inflammation and reduce oedema and mucus secretion into the airway. Bruising is a documented side effect. Other side effects include adrenal suppression with prolonged use of inhaled therapy, LRTIs, predisposition to osteoporosis, anxiety, depression, hyperglycaemia and cataracts. Can you classifiy the wide range of side effects of steroids?

Answer 500

L. Clopidogrel - Clopidogrel is used to prevent atherothrombotic events in patients sensitive to aspirin. This patient has had an MI. It is an antiplatelet drug which inhibits fibrinogen binding to glycoprotein IIb/IIIa receptors. It is given orally but effects are not seen until 4 days after the first dose. Use with aspirin further increases the risk of bleeding. Clopidogrel can also rarely cause a neutropenia.

Answer 501

H. Aplastic anaemia - This is aplastic anaemia characterised here with the pancytopenia (which is common, but diagnosis requires 2 cytopenias out of 3) and the presentation with infections (neutropenia), fatigue (anaemia) and bruising (thrombocytopenia). Risk factors include paroxysmal noctural haemoglobinuria, hepatitis and NSAIDs. If macrocytosis is seen, this may suggest an inherited syndrome such as Fanconi’s anaemia. The reticulocyte count here rules out haemolytic anaemia, which isn’t even an option on the list. The definitive diagnosis is made on biopsy of bone marrow which shows a hypocellular marrow with no abnormal cell populations and no fibrosis. Which conditions would there be abnormal cell populations or fibrosis on bone marrow biopsy?

Answer 502

C. Hepatic cirrhosis - Cirrhosis is the end-stage of chronic liver disease, in this case due to alcoholic liver disease. Cirrhosis results in hepatic insufficiency and portal hypertension. The bruising here is due to thrombocytopenia secondary to portal hypertension with resulting hypersplenism and sequestration. Complications of chronic liver disease include ascites, variceal bleeds, jaundice, hepatic encephalopathy, hepatorenal syndrome and the development of HCC. Signs include spider naevi, palmar erythema, telangiectasia, bruising, gynaecomastia, Dupuytren’s contracture, parotid swelling and a red tongue.

Answer 503

E. Skull fracture - This is a basilar skull fracture and a CT scan (superior to MRI), in this case with 3D reconstructions, will be useful. This patient has had a fall and clearly hit his head. Basilar skyll fractures have specific clinical features. Blood pooling from these fractures can cause periorbital bruising (raccoon eyes), brusing over the mastoid area (Battle’s sign) and bloody otorrhoea. There may also be CSF leak resulting in CSF otorrhoea or rhinorrhoea. A unilateral raccoon eye has an 85% positive predictive value for this diagnosis.

Answer 504

M. Hypothyroidism - This patient has hypothyroidism. Worldwide, the most common cause is iodine deficiency. Other causes include Hashimoto’s or secondary and tertiary hypothyroidism. It can also result from viral de Quervain’s thyroiditis or postpartum thyroiditis. Symptoms include those mentioned (depression, fatigue, weight gain, bradycardia and menstrual problems) as well as others such as slow-relaxing reflexes on examination, constipation, cold intolerance, dry skin and muscle cramps. Diagnosis is based on measurement of TSH and thyroid hormones. Treatment is by replacement of T4 with or without T3 in combination. If the patient has normal T3 and T4 but mildly elevated TSH, this is described as subclinical hypothyroidism.

Answer 505

I. Reduced activity - The examination here is unremarkable and there is no change in diet or medication. This patient has reduced exercise capacity with SOB on exertion, which has resulting in reduced mobility, accounting for her weight gain as less energy is being expended despite consuming the same amount.

Answer 506

B. Cushing's syndrome - Corticosteroids remain the mainstay of treatment for minimal change disease and is given to all patients. Minimal change disease is the most common form of nephrotic syndrome in children and it is so named as there are minimal histological changes in renal tissue. 90% are idiopathic. Long term coricosteroid treatment here has induced iatrogenic Cushing’s syndrome with associated signs of hypercorticalism (weight gain and easy bruising). Corticosteroid-sparing therapies can be added here.

Answer 507

K. Polycystic ovary syndrome - This patient’s presentation makes you think of PCOS. There is hirsutisim, amenorrhoea (or irregular and infrequent periods too) and weight gain, which are all features. Other features include acne, scalp hair loss and infertility. Hypertension is sometimes associated with this syndrome. On examination, sweaty/oily skin may be found and acanthosis nigricans may also be seen. An ultrasound is by no means the first test to order, as 1/4 of normal women and women with other problems like hyperprolactinaemia may also have polycystic ovaries, and they are present in 3/4 of those with PCOS. Serum androgens can be measured or the diagnosis can be made clinically.

Answer 508

J. Portal hypertension - HCV in this patient is causing hepatic cirrhosis which has decompensated resulting in ascites, secondary to portal hypertension. The hypoalbuminaemia is a sign of decreased hepatic synthetic function. Hyponatraemia is a common finding associated with ascites. It arises due to reduced protein synthesis and therefore a loss of colloid osmotic pressure and increased fluid loss from the intravascular compartment, stimulating ADH secretion. There is peripheral oedema here which is due to low albumin. The pruritis is due to reduced hepatic excretion of conjugated bilirubin and there may be accompanying jaundice too. The cause of his renal failure may well be hepatorenal syndrome in the context of his severe liver disease. His prognosis is poor.

Answer 509

A. Diclofenac - NSAIDS may impair renal function and provoke renal failure, especially in patients with pre-existing impairment. NSAIDs should be avoided if possible in these patients or used with caution at the lowest effective dose for the shortest possible time. The mechanism of damage involves reducing creatinine clearance. NSAIDs are also contraindicated in asthmatics as it causes bronchospasm due to the accumulation of leukotrienes.

Answer 510

B. Epidural bupivavaine and fentanyl - Epidurals are relatively contraindicated in anticoagulated patients. Insertion of the epidural needle may lead traumatic bleeding into the epidural space and with clotting abnormalities, the development of a haematoma which can lead to spinal cord compression. Coagulopathy, raised ICP and infection at the injection site are absolute contraindications. Relative contraindications include anticoagulated patients and those with anatomical abnormalities of the vertebral column. NSAIDs do not increase the risk of epidural haematoma.

Answer 511

C. Codydramol - Co-dydramol is a combination of dihydrocodeine and paracetamol and the patient is known to be allergic to dihydrocodeine.

Answer 512

A. Dicolfenac - NSAIDs inhibit COX which has the effect of reducing PGE2 levels. PGE2 plays a role in gastric cytoprotection by downregulating HCl production and increasing mucus and the production of bicarbonate. This leads to gastric irritation and ulceration. A PPI can be prescribed alongside NSAIDs or misoprostol can be used, which is a stable PGE1 analogue which mimics local PG to maintain the gastroduodenal mucosal barrier.

Answer 513

D. Cirrhosis - Cirrhosis is the end stage of chronic liver disease which results in hepatic insufficiency and portal hypertension, causing this patient’s jaundice, a sign a decompensation. Jaundice is also seen here reflecting reduced hepatic excretion of conjugated bilirubin and there may be associated pruritis. Telangiectasia is a sign of chronic liver disease. Risk factors for cirrhosis include alcohol, IVDU, unprotected sex and blood transfusion. This gives us an insight into the causes which include chronic viral hepatitis (C, B with or without D) and alcoholic liver disease. Other causes include conditions such as Wilson’s disease, NAFLD, haemochromatosis, Budd-Chiari syndrome and drug induced such as amiodarone and methotrexate.

Answer 514

L. Delerium tremens - This patient has delirium tremens from alcohol withdrawal. Delirium is an acute fluctuating change in mental status. It is worth noting that alcohol excess can also cause delirium secondary to alcoholic ketoacidosis and can also be seen in Wernicke’s or Korsakoff’s. DT is associated with hallucinations (clasically tactile and visual of insects crawling on the person), delusions and tremor. First line treatment is with chlordiazepoxide (or lorazepam if the patient has liver failure).Vitamin supplementation will also be indicated.

Answer 515

H. Wernicke's encephalopathy - Wernicke’s is due to acute thiamine deficiency, which is a problem in alcoholics. Others at risk include those with AIDS, cancer and treatment with chemotherapy, malnutrition and GIT surgery, especially bariatric procedures. It is a clinically under-diagnosed condition. The classic EMQ triad is of mental change, ophthalmoplegia and gait dysfunction, which is actually only seen in 10% of cases. In reality, the manifestations are varied and a high index of suspicion is needed. Despite there, the manifestiations typically include altered consciousness, gait disorders and eye movement abnormalities. This is an emergency and treatment is with parenteral replacement of thiamine. This avoids permanent neurological damage including later development of Korsakoff’s psychosis, which is irreversible. Note that thiamine should be given before dextrose! Magnesium deficiency also needs to be corrected as it is a co-factor in the functioning of thiamine dependent enzymes.

Answer 516

C. Rhabdomyolysis - Rhabdomyolysis is basically myocyte lysis. It may present obviously with a patient having been ‘crushed’ in some way, or may be insidious in onset and not very obvious. This patient has had too much alcohol, or drugs, and has been immobilised in one position for a long period of time. This makes a crush injury more likely to occur and indeed, alcohol abuse is the most common cause in itself, by being directly toxic to myocyte membranes, inhibiting calcium uptake and disrupting the sodium-potassium pump. Illicit drugs may cause a hyperdynamic muscle state. Other risk factors include diuretics, which can deplete potassium to such a degree as to cause this condition, toxins, statins and salicylates. This condition has also been documented in unconditioned athletes. Historically, it was those crushed during the WWII bombings of London who were affected by this condition and experience during the war has also changed the way it is now treated with regard to acid-base abnormalities. If you free someone who has been crushed for a very long time, there will be a sudden release of potassium which is life-threatening. You therefore need to take measures to protect against this, such as bicarbonate therapy beforehand. Muscle pain and discomfort is a common presentation, although this person does not appear to be conscious. The diagnosis here is confirmed by the elevated serum CK level which will be increased to at least 5 times normal. Levels correlate with severity. Expect to also see raised potassium, magnesium and phosphate, and low calcium, with elevated urea and creatinine. Mainstay of treatment is with fluid resuscitation.

Answer 517

A. Depression - Depression is characterised by low mood, loss of interest and reduced energy persistently over a long period of time. It is a common condition and there is an obvious recent stress here as her husband has left her. Patients tend to respond well to CBT, antidepressants or both. Suicidal ideation should be assessed for. There is a high lifetime chance of recurrence.

Answer 518

G. Dietary advice alone - This patient is symptomatic and has a random blood glucose of 10.2. This patient does not quite meet the diagnostic criteria for DM. Symptomatic patients need a single random blood glucose of >11.1 or single fasting glucose of >7. Asymptomatic patients need two separate elevated readings for a diagnosis. Alternatively if there are borderline results, an OGTT can be conducted to see if plasma glucose is raised >11.1 two hours after an oral glucose load of 75g. A patient is said to have impaired fasting glucose if fasting glucose falls between 6.1-6.9. Impaired glucose tolerance is present if plasma glucose 2 hours after oral glucose load in OGTT falls between 7.8-11.0. First line intervention in this situation, and in newly diagnosed DM is diet and lifestyle advice and changes.

Answer 519

F. Oral hypoglycaemic drug - Careful dietary intervention has failed to keep HbA1c in check so the patient will need to be started on an oral hypoglycaemic drug. First line is metformin. It is worth noting that HbA1c values, since June 2011, are no longer expressed as a percentage and are now given in mmol/mol. HbA1c is glycated Hb and provides an estimation of glycaemic control over the life span of red blood cells (around 60 days). Fructosamine is measured instead if there is a Hb disorder or RBC life span is decreased as HbA1c is only reliable if normal Hb is present with normal RBC life spans. Fructosamine is a glycated plasma protein which provides information on glucose levels over the previous 1-3 weeks.

Answer 520

C. Blood pressure control - BP control with an ACE inhibitor is necessary to reduce progression of diabetic nephropathy. Microalbuminuria is the earliest detectable sign and indicates trace amounts of albumin not detectable with standard urinalysis. If untreated, microalbuminuria will progress to intermittent albuminuria and then to persistent albuminuria which is 5-10 years away from ESRF. The main contributor to the development of diabetic nephropathy is hypertension. Other contributors include poor glycaemic control and renovascular disease such as renal artery stenosis. It is important to test kidney function and urine regularly.

Answer 521

A. Oral glucose or sugar - This patient has symptoms of hypoglycaemia, present when glucose drops

Answer 522

I. Insulin - This young person has presented with T1DM, a condition characterised by absolute insulin deficiency. As a result, insulin treatment is required to prevent DKA and chronic complications of hyperglycaemia. The presentation is most often with polyuria, polydipsia, weight loss and weakness. Ketonuria in the presence of hyperglycaemia suggests T1DM. Ketonuria occurs due to the fact the body has no insulin to drive glucose into the cells and hence ketone bodies are utilised by the brain (fatty acids cannot cross the BBB). Diet, exercise and insulin therapy is crucial in T1DM.

Answer 523

H. Bowel obstruction - Bowel obstruction has several causes such as adhesions or cancer. The lack of bowel motions, vomiting, distended abdomen and tinkling bowel signs are all indicative. The proximal segment of bowel dilates and distal bowel collapses. Completely obstructed patients generally require surgery. If, on AXR, air is seen to be seeping past the obstruction then the obstruction is partial. As a standard, all patients should be made NBM and given supplemental oxygen, IV fluids and NG decompression (to reduce flow/gastric contents/air towards the obstruction), unless they are rushed off for an emergency laparotomy because, for example, they have complete SBO and are peritonitic.

Answer 524

D. Uraemia - This patient has developed acute renal failure, probably associated with the longstanding CCF. Advanced heart failure will lead to depressed renal perfusion and ARF. The decreased urine output is a symptom and the vomiting here is caused by uraemia or a general build up of waste products. An acute increase in creatinine will be seen, commonly with hyperkalaemia, hyperphosphataemia and a metabolic acidosis. There may also be respiratory compensation for this. Treatment is largely supportive, managing, in this case, the heart failure, and correcting abnormalities like volume status and the metabolic acidosis. Dialysis may be required.

Answer 525

B. Pancreatitis - This patient has acute pancreatitis. He has vomited and is describing mid-epigastric pain radiating around to the back. This patient can also have nausea and vomiting too, with agitation and confusion. A pleural effusion is seen in half of patients with acute pancreatitis. Complicated haemorrhagic pancreatitis may exhibit Cullen’s sign, Grey-Turner’s sign and Fox’s sign. Make sure you know what these are and you are familiar with the causes of acute pancreatitis (GET SMASHED). Those caused by hypocalcaemia may display Chvostek’s sign and Trousseau’s sign. Key to diagnosis is serum amylase or lipase levels which are massively elevated. Prognostic criteria are outlined in Ranson’s criteria applied on admission and after 48 hours, or the modified Glasgow score which you can find in your Oxford Handbook. An abdominal CT is however the most sensitive and specific study and findings may include enlargement of the pancreas with irregular contours, necrosis, pseudocysts and peripancreatic fat obliteration. For interest, urinary trypsinogen-2 is now considered a better screening test than amylase but is not currently clinically used.

Answer 526

G. Salmonella - The only option on the list which fits is Salmonella. This could obviously be E coli or Campylobacter too, for instance. This is infectious gastroenteritis and Salmonella is a common cause, pretty much able to contaminate any food. It is commonly linked to poultry, dairy items and undercooked eggs. It is self-limiting and diagnosis is on isolating the organism from a stool culture. Treatment is supportive with fluid and electrolyte replacement and antibiotics are generally used only for patients with risk factors for severe disease or those with extra-GI complications. This is not viral gastroenteritis for the reasons below:

Answer 527

K. Viral gastroenteritis - Viral gastroenteritis often presents with mainly UGI symptoms like N&V more so than diarrhoea. It is on this basis that this is likely to be viral gastroenteritis caused by organisms such as rotavirus, norovirus and adenovirus. Staphylococcus aureus and Bacillus cereus also tend to cause mostly UGI symptoms and acts by preformed toxins, though these are not on the list of available options. The differentiation is made on studying the stool and identifying the organism.

Answer 528

C. Hydrocele - A hydrocele is a collection of serous fluid between the layers of the tunica vaginalis. Patients mainly present with a painless swollen scrotum (which can be on one or both sides) which feels like a water-filled balloon. Surgery is only performed if the hydrocele is problematic. As a result of the fluid, most hydroceles can be transilluminated. On examination, the hydrocele cannot be differentiated from the testicle. The mass can also increase in size with increased intra-abdominal pressure (such as coughing, crying or raising the arms) which causes peritoneal fluid to move into the scrotal sac. This causes the mass to vary in size during the day (smaller after lying down).

Answer 529

B. Testicular torsion - This is a urological emergency caused by twisting of the testicle on the spermatic cord which if not fixed will lead to ischaemia and necrosis of testicular tissue. The main differential is epididymo-orchitis but a high index of suspicion is necessary and if there is any doubt then surgical exploration is indicated. Boys aged 12-18 are at greater risk. There is usually sudden-onset very severe scrotal pain with N&V. There may be spontaneous de-torsion hence a history of previous episodes of pain. A history of undescended testes is a major risk factor as is the bell clapper deformity. During exploration, the contralateral testis is also fixed to the posterior wall.

Answer 530

I. Hernia - This is an inguinal hernia. It is where the abdominal contents along with its layers of peritoneum and fascia protrude through the inguinal canal. Remember that on examination, a direct hernia cannot be controlled by pressure at the midpoint of the inguinal ligament whereas an indirect hernia can be controlled by occlusive pressure in this manner. Remember also, your anatomy of the region. The hernial sac of a direct hernia lies medial to the inferior epigastric artery whereas the hernial sac of an indirect hernia lies lateral to the artery. The hernia itself can be either reducible or irreducible and may additionally be obstructed, strangulated or incarcerated.

Answer 531

F. Testicular malignancy - Testicular cancer commonly presents as a hard and painless lump on one testis although the lump can be painful and 10% present with acute pain associated with haemorrhage or infection. The supraclavicular lymph nodes here are an extratesticular manifestation – others include bone pain from metastasis, swelling of the lower extremities due to venous occlusion and gynaecomastia. Key risk factors include cryptorchidism and FH. White men have the highest incidence. The principal investigation is an ultrasound of the testis and testicular examination is vital in detecting this condition early on. Beta-hCG is raised in seminomas and teratomas however only AFP is raised in teratomas. Placental ALP can be raised in advanced disease. It is diagnostic if AFP, beta hCG and LDH are elevated. Teratomas are more common in the 20-30 age group whereas seminomas are more common after 30 and this is why the better option for this question would be a seminoma. In reality, you cannot tell from the age. Radical orchidectomy and histology is the initial treatment in most cases.

Answer 532

G. Epididymo-orchitis - Generally speaking, younger males are more likely to have an STI whereas older men are more likely to have an infection with enteric organisms such as ESBL. He is mildly febrile with tenderness on-top of urinary symptoms which points towards epididymo-orchitis. A urethral swab should be sent for Gram stain and culture of secretions. A urine dipstick is also necessary combined with urine MC+S. Treatment involves bed rest, scrotal elevation, analgesia and antibiotics if indicated.

Answer 533

J. Bronchial asthma - Progressively worsening SOB and the wheeze, which may like this case wake the patient from sleep combined with the long duration of symptions suggests asthma. This patient is having an acute exacerbation and needs to be treated for this, before commencing the appropriate stepwise chronic treatment on the BTS ladder. Whether this exacerbation is mild, moderate or severe is based on the patient’s % predicted PEFR. Examination can show an expiratory wheeze and in severe exacerbations, the chest may be silent. Night symptoms occur in more severe asthma and symptoms can be exacerbated by exercise. Diagnosis is supported by PEFR variation of at least 20% over 3 days in a week over several weeks or an increase of at least 20% to treatment. Stepwise treatment is outlined below. Look up the BTS guidelines for more information. Step 1: SABA PRN, Step 2: Plus low-dose inhaled corticosteroids (ICS) , Step 3: Plus LABA, Step 4: Increase dose of ICS or add LTRA, SR theophylline or beta agonist tablet, Step 5: Daily steroid tablet and maintain ICS with specialist care.

Answer 534

A. Pneumothorax - This is a primary pneumothorax which occurs in young people without any known lung conditions. Having a tall and slender build like this patient is a recognised risk factor. Other risks include smoking, FH, Marfan’s, young age, male and conditions like CF and TB. The examination findings of absent breath sounds and hyperresonance point to this diagnosis. The main investigation is a CXR and pneumothoraces are classified by the BTS as large (>2cm visible rim between the lung margin and the chest wall) or small (

Answer 535

D. Inhaled foreign body - This is aspiration pneumonia. There are symptoms of pneumoia with pyrexia and purulent sputum, along with risk factors for aspiration in this elderly person who may have difficulties swallowing or altered mental status from, for example, dementia. The location of the wheeze is also consistent with this diagnosis as the RLL is the most common site due to the anatomy of the bronchial tree. Complications include abscess and empyema. Treatment is predominantly with antibiotics and supportive care.

Answer 536

B. Left ventricular failure - DM is a cardiovascular risk factor. There are no expressed signs or symptoms of RVF here such as peripheral oedema, ascites, elevated JVP and hepatomegaly. Nocturia may be a symptom as fluid returns from the legs when the patient lies down flat. RVF leads to a backlog of blood and congestion of the systemic capillaries. LVF, on the other hand, causes congestion in the pulmonary circulation so the symptoms are respiratory. As seen in this patient, there is SOB and the classic cough productive of frothy sputum – a sign of pulmonary oedema. On respiratory examination, pulmonary oedema due to LVF may give audible fine late inspiratory crepitations at the bases. There may also be orthopnoea. This is why you can ask patients in a cardiac history how many pillows they sleep with. PND can also occur as well as ‘cardiac asthma’. Initial investigations should include ECG, CXR, TTE and bloods including BNP levels. First line treatment is with an ACE inhibitor which reduces morbidity and mortality. Salt and fluid restriction is also beneficial. All patients with chronic heart failure will also receive a beta blocker such as carvedilol. Other adjuncts include spironolactone, diuretics, hydralazine and a nitrate, and digoxin. The NYHA classification criteria can be used based on symptoms to describe functional limitations and ranges from Class I to Class IV with symptoms occuring at rest. Many patients are asymptomatic for long periods of time because mild cardiac impairment is balanced by compensation.

Answer 537

L. Cystic fibrosis - This man has presented with cystic fibrosis which is an autosomal recessive condition characterised by a mutation in the CFTR gene on chromosome 7 (delta-F508). CF is a genetic condition with abnormal salt and water transport due to mutations in the CFTR (an apical anion channel). Heterozygotes generally do not demonstrate disease. The age of symptom onset varies though screening newborns allows this condition to be detected early on. Serum IRT from a heel prict blood spot allows screening of newborns. This patient complains of predominantly lung disease, from a cycle of mucus retention, infection and airway inflammation. This can be treated with mucus thinners, clearance of the airway with, for instance, physiotherapy and use of antibiotics. Additionally there is infertility, which may be a presenting complaint. This is due to bilateral abscence of the vas deferens which may be obvious on examination of the patient’s scrotum. This patient is also clubbed. The most conclusive diagnostic test is the sweat test which is positive if sweat chloride is >60mmol/L. The life expectancy now is around 38 years old.

Answer 538

G. Innocent murmur - This is a functional murmur which is not caused by a structural cardiac defect. Functional murmurs tend to be systolic, occuring in an otherwise healthy individual with no symptoms. They are also characteristically position dependent and soft in nature. Ones that occur in children tend to disappear as the child grows. Benign paediatric murmurs include Still’s murmur.

Answer 539

K. Mitral stenosis - Sydenham's chorea (St Vitus Dance) are dancelike movements seen in rheumatic fever. The major criteria for rheumatic fever can be remember by CASES: carditis, arthritis, Sydenham’s chorea, erythema marginatum and subcutaneous nodules. Practically every single case of mitral stenosis is caused by rheumatic heart disease. The process tends to also cause regurgitation. This is characteristically a grade 1-2 low pitch murmur heard in mid-diastole which has a rumbling nature and there is no radiation. There can be an associated malar flush, tapping apex beat and a diastolic thrill palpable at the apex, in the 5th intercostal space in the MCL. The first heart sound is also characteristically loud and often this is the most striking feature on ascultation. It is a difficult murmur to pick up so if you are ever asked at this stage to spot this murmur, it will most likely be based on the loud S1.

Answer 540

E. Infective endocarditis - Any patient presenting with fever and a new murmur should always make you think of bacterial endocarditis. The classic new or worsening murmur is actually rare. As are splinter haemorrhages, which this patient has. Other uncommon signs you may find include Janeway lesions (painless macular haemorrhagic plaques on the palms and soles) and Osler nodes (painful nodules on the pads of the fingers and toes). Roth spots may also be seen on fundoscopy. Three sets of bood cultures are required and this patient will have to go for an echocardiogram.The Duke criteria is used for diagnosis.

Answer 541

F. Aortic stenosis - Aortic stenosis can present with chest pain, dyspnoea and syncope. It is characterised by a harsh ejection systolic murmur heard loudest at the right upper sternal edge at end expiration, which radiates up towards the carotids. The pulse pressure is narrow and there may be an associated slow-rising and plateau pulse. Doppler echo is vital for diagnosis and shows a pressure gradient across the narrowed valve orifice. 20% of cases are due to a congenital bicuspid valve. The most common cause of aortic stenosis in adults is calcification of normal trileaflet valves. Clinically stable patients may be considered for surgical repair or TAVR.

Answer 542

L. Mitral regurgitation - MR is loudest at the apex and radiates to the axilla and tends to be around grade 4. It is associated with a systolic thrill at the apex. TTE is the investigation of choice for diagnosis. Chronic MR is associated with a laterally displaced apex beat with LV dilatation. Mitral valve prolapse is a strong risk factor for development of MR.

Answer 543

H. Occupational therapist - Occupational therapists will visit the patient’s home and help determine tools and facilities the patient can benefit from for their day to day life e.g. stair-lifts, bath rails

Answer 544

I. Physiotherapists - Physiotherapists work with patients who have physical difficulties to identify and improve movement and function. This will involve, for example, encouraging movement and exercise using a range of techniques.

Answer 545

F. Speech and language therapist - Would you discharge this patient in their present state? This patient needs a swallowing assessment and speech therapy from a SALT. SALTs deal with disorders of speech, communication, language and swallowing. They are particularly useful after neurological impairments and conditions including stroke, head injury, Parkinson’s and dementia. In the film ‘The King’s Speech’ starring acclaimed actor and heartthrob Colin Firth, George VI gets help with his stammer from Logue who is a speech and language therapist.

Answer 546

A. Dietician - This patient has possible DM (needs a second test result to be diagnostic or needs to be symptomatic). The first line intervention is dietary and lifestyle changes. The most useful person this patient will benefit from is a dietician who will be able to give him advice on his eating habits – a diet high in fibre and low in fat is recommended and to also cut, to a degree, carbohydrate intake or at least resort to carbohydrates with a high glycaemic index such as lentils and sweet potato. The GI measures the effect carbohydrates have on blood sugar levels – a lower GI has more of an impact on blood sugar levels in a shorter amount of time. Advice will also be given on how to deal with hypoglycaemic episodes if the patient is being treated with medication which predisposes to this risk, such as insulin.

Answer 547

J. District nurse - District nurses visit people in their homes or in care homes and provide care in that context. They also provide teaching in the community.Always remember that an MDT ethos is needed for most cases. This is especially true for stroke patients.

Answer 548

C. Hiatus hernia - A hiatus hernia is where intraabdominal contents protrude through the oesophageal hiatus of the diaphragm. Risk factors inclyde obesity and high intra-abdominal pressure. The condition may be asymptomatic, or it may present with symptoms (which are non-specific) such as heartburn, dysphagia, pain on swallowing, wheezing, hoarseness and chest pain. A CXR is the first test done and may show an air bubble in the wrong place but barium studies are diagnostic and treatment depends on the symptoms and anatomy of the hernia. Hernias can be sliding or rolling (or mixed, or giant), uncomplicated or complicated by, for instance, obstruction and bleeding. Do you know the difference between a sliding and a rolling hiatal hernia?

Answer 549

F. Carcinoma of oesophagus - Dysphagia (in this progressive pattern) coupled with weight loss points to malignancy. Dysphagia occurs when there is obstruction of more than 2/3 of the lumen and presence indicates locally advanced disease. There may additionally be odynophagia. Men are twice as likely to develop oesophageal cancer. GORD, Barrett’s oesophagus, FH, tobacco and alcohol are all risk factors. The two main types are squamous cell carcinoma and adenocarcinoma. Tumours in the upper 2/3 of the oesophagus are SCC whereas those that lie in the lower 1/3 are adenocarcinomas. The main test to order is an OGD with biopsy. Treatment is either surgical resection or with chemo or radiotherapy alongside endoscopic ablation with or without stenting and brachytherapy.

Answer 550

I. Plummer-Vinson syndrome - Plummer-Vinson syndrome is the association of chronic IDA (shown here by the koilonychia and paleness on examination) with dysphagia due to a post cricoid web. Roughly 7% of those with IDA may complain of gradual onset dysphagia with the discomfort found in the area of the cricoid cartilage. Invasive procedures may be needed for management such as endoscopic dilation of the web but treatment is largely aimed at correcting the IDA.

Answer 551

E. Myasthenia gravis - Myasthenia gravis is an autoimmune condition with antibodies affecting the NMJ, mostly the nAChR at the post-synaptic muscle membrane. Although some have antibodies against MuSK, and there are other proteins involved. MG is characterised by muscle weakness which increases with exercise (fatigue, demonstrated here as the dysphagia gets worse as he eats, unlike Lambert-Eaton myasthenic syndrome). Commonly, presentations include diplopia and drooping eyelids like this patient, and there may also be SOB, proximal limb weakness, facial paresis and oropharyngeal weakness. MG is associated with thymic hyperplasia in 70% or thymoma in 10%, and these associations can also crop up in EMQs. There will be elevated serum AChR receptor antibody titres or MuSK antibodies. Electrophysiology will demonstrate a decremental response on repetitive nerve stimulation. Treatment includes anticholinesterases (pyridostigmine, and immunotherapy. Patients may also require a thymectomy. Some 15-20% may experience a myasthenic crisis (which needs mechanical ventilation). Do you know what the Tensilon test is and why edrophonium is given in this test?

Answer 552

G. Achalasia - This is achalasia which is a motility disorder with loss of peristalsis in the distal oesophagus and failure of the LOS to relax in response to swallowing. This presents commonly with dysphagia to both liquids and solids, regurgitation and retrosternal chest pain, which can be slowly progressive over time. In structural obstruction such as cancer, dysphagia to liquids is uncommon unless the disease is very advanced. Retrosternal pressure experienced can be precipitated by drinking liquids but is eased by continuing to drink, and the pain may be relieved by cold water. This may wake the individual from sleep. A UGI endoscopy is needed to exclude malignancy as a cause of dysphagia. The diagnosis is established on manometry or barium studies. Treatment is symptomatic.

Answer 553

F. Salmonella typhi - Typhoid (or enteric fever) is a faecal-oral illness caused by Salmonella enterica, serotype S typhi, Salmonella enterica and S paratyphi. There are over 2500 serovars for S enterica. In this country, it is mainly from people who have returned from a country where it is endemic – India, for example, which has the highest incidence of this disease. Mexico should also ring alarm bells. The water supplies are not treated and sanitary conditions are dire which prompts transmission of this infection. Humans are the only known reservoir. The vaccine only offers moderate protection and does not protect against paratyphoid infection. This person has a high fever which is a hallmark of infection (sometimes in a step-wise manner) which is not responding to paracetamol (it persists) and blood culture (you need a big sample of blood for testing as count is usually low) shows a gram negative bacilli, and he has returned from an endemic region. The fever of typhoid classically increases incrementally until a persistent fever with temperature 39-41 is established. There are also typically flu like symptoms after onset of fever and cough. Remember that characteristic findings such as bradycardia and rose spots may not be present and indeed rose spots may not be easy to spot in those with dark skin. Rose spots are blanching red lesions reported in 5-30% of cases usually occuring on the chest or abdomen. This patient needs antibiotics though the temperature will fall over about week. A third generation cephalosporin is indicated due to resistance to fluoroquinolones in the Indian sub-continent. If the sensitivity panel returns and shows that this organism is sensitive to all antibiotics then ciprofloxacin should be given.

Answer 554

B. Neisseria meningitidis type B - This patient has meningitis. A big risk factor is crowding which occurs during Hajj. Commonly there will be a headache, fever and nuchal rigidity. There may also be an altered mental status, confusion, photophobia and vomiting. Kernig’s sign is uncommon but is positive when attempts to extend the leg are met with resistance when the patient is supine with the thigh flexed to 90 degrees. Another uncommon sign is Brudzinski’s sign and a petechial/purpuric rash, typically associated with meningococcal meningitis. CT head should be considered before LP if there is any evidence of raised ICP. A LP will confirm the diagnosis with bacterial meningitis showing a low CSF glucose, elevated CSF protein and positive CSF culture/gram stain or meningococcal antigen. The CSF gram stain showing a gram negative diplococci here gives the organism away. If you know your gram stains etc you can get this one from just reading the last line.

Answer 555

C. Entamoeba histolytica - This is amoebiasis caused by E. histolytica. Again, there is history of travel to an endemic area. Additional risk factors include being male and male-male sex, both oral and anal. Diagnosis would be confirmed by the detection of antigen in stool samples, serology or PCR. As neither of these methods are 100% sensitive, it is normally good to use more than 1 test in diagnosis. The presentation is normally with diarrhoea, without blood or mucus, present for several days or longer, indicating intestinal infection. Half also report weight loss. This patient, has however got the fever more associated with hepatic infection. There may also be jaundice here and hepatomegaly on examination. The RUQ tenderness found here is indicative of hepatic infection and the mass here is likely a hepatic abscess, which itself may extend into the pleural or pericardial cavities in rare cases, causing effusions. Splenic abscesses and even brain abscesses with neurological abnormalities can also rarely occur. Treatment is with nitroimidazoles followed by agents like paromomycin.

Answer 556

A. Mycobacterium tuberculosis - It is important to have a high level of suspicion when evaluating patients with risk factors who present with suggestive symptoms. Night sweats, fever, malaise, cough, haemoptysis and erythema nodosum are all suggestive. In the first half of the 20th century, tuberculosis accounted for over 90% of cases of erythema nodosum. Other key risk factors for pulmonary TB include exposure to infection and returning from or being born in a high-risk region such as Asia, Africa and Latin America. If TB is suspected, the patient should be placed in isolation and a CXR obtained with 3 sputum samples cultured for AFB being the gold standard of diagnosis. Cavitating lesions like the one this patient has can be seen on CXR but is non-specific for TB. Culture takes several weeks so sputum smears will be done before culture results are known. Interferon-gamma release assays (IGRAs) are now used by some hospitals to rapidly determine a patient’s TB status. All patients who have TB should be tested for HIV within 2 months of diagnosis.

Answer 557

G. Falciparum malaria - In the Western world, almost all cases of malaria occurs in travellers so an adequate travel history is crucial or the diagnosis may be missed. Patients typically present with non-specific symptoms such as a fever, sweats, chills and myalgia. This student has just returned from an endemic area. Sometimes EMQs will describe patterns of fevers occuring at regular intervals of 48-72 hours associated with P. vivax, P. ovale and P. malariae infections but in most patients there is no specific pattern. Hepatosplenomegaly is a common presenting sign although not common at presentation in a first world setting. Thrombocytopenia is common with falciparum infection and a mild degree of anaemia are commonly seen. WCC can be high, low or normal. Pregnant women affected by P. falciparum are susceptible to the complications of pregnancy due to placental parasite sequestration. Treatment of malaria in pregnancy must be managed with an ID specialist and should be treated with IV antimalarial therapy. The test of choice is Giesma-stained thick and thin blood smears. Thick films sensitively detect parasites whereas thin films allow species identification and calculation of parasitaemia to guide treatment. Studies have shown that for P falciparum, the most effective treatment is artesunate which is more effective than quinine without the risk of cinchonism. Numerous studies such as the AQUAMAT study in The Lancet showing that quinine should no longer be the established treatment of choice. Dengue presents abruptly with typically headache and retrobulbar pain worsening with eye movement. There may also be a rash and leukopenia and thrombocytopenia are common.

Answer 558

Fluid replacement therapy aims to maintain physiological parameters. Systolic BP >90 is required for adequate organ perfusion. IV 0.9% saline is the first line treatment for volume depletion in almost all situations. It is worth noting that normal saline in large amounts carries a risk of inducing a metabolic acidosis due to the high chloride content. 5% dextrose is equivalent to water when given, and is not approriate for volume resuscitation since it will distribute throughout the total body water. Only 1/12 will remain in the intravascular space. 5% dextrose may be used, however, in resuscitation or replacement in diabetics on an insulin drip to prevent hypoglycaemia. The insulin and dextrose infusion should go in the same cannula so there is no risk of giving unopposed insulin. For example, if the arm you are giving the dextrose infusion were to clog up, or more likely, if the patient bends their arm if the cannula is sited in the antecubital fossa. Sodium bicarbonate solution can be used acutely to treat severe metabolic acidosis. Lactated Ringer’s solution may also be helpful, but may contribute to hyperkalaemia in the setting of renal failure. IV colloids are less preferred. These include albumin, starches, dextrans and gelatins. Colloids are used when there is a risk of tissue oedema as there is a reduced proportion of administered fluid lost into the interstitial space. However, they are expensive and have not shown to benefit mortality in many studies, and indeed a systematic review in the BMJ of 37 RCTs has shown a 4% increase in absolute mortality. Crystalloids remain first choice for fluid resuscitation in the first instance. Blood is the best intravascular volume expander (replacing like for like), especially if the patient is anaemic or is actively bleeding. It is usually given as packed red cells with saline. Mild volume depletion can be managed by ORT. Glucose is typically added to promote the sodium/glucose co-transporter. Depending on the site of loss, antiemetics and antidiarrhoeals (in non-infectious diarrhoea) may be indicated. Vasopressors are often needed in sepsis. So, having said all of this... A. IV saline - This patient is very dehydrated

Answer 559

Fluid replacement therapy aims to maintain physiological parameters. Systolic BP >90 is required for adequate organ perfusion. IV 0.9% saline is the first line treatment for volume depletion in almost all situations. It is worth noting that normal saline in large amounts carries a risk of inducing a metabolic acidosis due to the high chloride content. 5% dextrose is equivalent to water when given, and is not approriate for volume resuscitation since it will distribute throughout the total body water. Only 1/12 will remain in the intravascular space. 5% dextrose may be used, however, in resuscitation or replacement in diabetics on an insulin drip to prevent hypoglycaemia. The insulin and dextrose infusion should go in the same cannula so there is no risk of giving unopposed insulin. For example, if the arm you are giving the dextrose infusion were to clog up, or more likely, if the patient bends their arm if the cannula is sited in the antecubital fossa. Sodium bicarbonate solution can be used acutely to treat severe metabolic acidosis. Lactated Ringer’s solution may also be helpful, but may contribute to hyperkalaemia in the setting of renal failure. IV colloids are less preferred. These include albumin, starches, dextrans and gelatins. Colloids are used when there is a risk of tissue oedema as there is a reduced proportion of administered fluid lost into the interstitial space. However, they are expensive and have not shown to benefit mortality in many studies, and indeed a systematic review in the BMJ of 37 RCTs has shown a 4% increase in absolute mortality. Crystalloids remain first choice for fluid resuscitation in the first instance. Blood is the best intravascular volume expander (replacing like for like), especially if the patient is anaemic or is actively bleeding. It is usually given as packed red cells with saline. Mild volume depletion can be managed by ORT. Glucose is typically added to promote the sodium/glucose co-transporter. Depending on the site of loss, antiemetics and antidiarrhoeals (in non-infectious diarrhoea) may be indicated. Vasopressors are often needed in sepsis. So, having said all of this... B. Administer diuretics - This patient has developed pulmonary oedema. This patient should be positioned upright and given diuretics (frusemide).

Answer 560

Fluid replacement therapy aims to maintain physiological parameters. Systolic BP >90 is required for adequate organ perfusion. IV 0.9% saline is the first line treatment for volume depletion in almost all situations. It is worth noting that normal saline in large amounts carries a risk of inducing a metabolic acidosis due to the high chloride content. 5% dextrose is equivalent to water when given, and is not approriate for volume resuscitation since it will distribute throughout the total body water. Only 1/12 will remain in the intravascular space. 5% dextrose may be used, however, in resuscitation or replacement in diabetics on an insulin drip to prevent hypoglycaemia. The insulin and dextrose infusion should go in the same cannula so there is no risk of giving unopposed insulin. For example, if the arm you are giving the dextrose infusion were to clog up, or more likely, if the patient bends their arm if the cannula is sited in the antecubital fossa. Sodium bicarbonate solution can be used acutely to treat severe metabolic acidosis. Lactated Ringer’s solution may also be helpful, but may contribute to hyperkalaemia in the setting of renal failure. IV colloids are less preferred. These include albumin, starches, dextrans and gelatins. Colloids are used when there is a risk of tissue oedema as there is a reduced proportion of administered fluid lost into the interstitial space. However, they are expensive and have not shown to benefit mortality in many studies, and indeed a systematic review in the BMJ of 37 RCTs has shown a 4% increase in absolute mortality. Crystalloids remain first choice for fluid resuscitation in the first instance. Blood is the best intravascular volume expander (replacing like for like), especially if the patient is anaemic or is actively bleeding. It is usually given as packed red cells with saline. Mild volume depletion can be managed by ORT. Glucose is typically added to promote the sodium/glucose co-transporter. Depending on the site of loss, antiemetics and antidiarrhoeals (in non-infectious diarrhoea) may be indicated. Vasopressors are often needed in sepsis. So, having said all of this... C. Colloid - As mentioned, first line fluid resuscitation is with 0.9% saline.

Answer 561

A. IV saline - Initial treatment of DKA aims at correcting severe volume depletion (the main problem), again with IV saline infusion at a rate of 1-1.5L for the first hour. When glucose reaches 11.1mmol, fluid should be changed to 5% dextrose to prevent hypoglycaemia.

Answer 562

D. Blood transfusion - This patient has been given IV saline already as the initial choice for volume expansion. His anaemia and clinical state warrants a blood transfusion. 1 unit of blood raises the Hb concentration by 1g/dL. Commonly, transfusion begins with 2 units of packed RBCs and the patient reponse is monitored.

Answer 563

D. Base of skull fracture - This is a basilar skull fracture and a CT scan (superior to MRI), in this case with 3D reconstructions, will be useful. These patients have hit their head head. Basilar skull fractures have specific clinical features. Blood pooling from these fractures can cause periorbital bruising (raccoon eyes), brusing over the mastoid area (Battle’s sign) and bloody otorrhoea. There may also be CSF leak resulting in CSF otorrhoea or rhinorrhoea. A unilateral raccoon eye has an 85% positive predictive value for this diagnosis. Base of skull fractures can also affect cranial nerves leading to hearing impairment (due either to SN as a result of VII injury or conductive due to haemotympanum) and facial numbness or paralysis. These patients also need to have their GCS calculated. Treatment is primarily conservative although surgical intervention may be needed if there is associated CSF leak, intracranial pathology or CN deficit. RTAs are the second most common cause of skull fractures, the first being a fall from a height. There may also, like the first case, be a history of assault resulting in head trauma or even a gunshot to the head.

Answer 564

A. Subdural haematoma - Blood collects between the dura and arachnoid mater in a subdural haematoma. This condition can run a variable disease course. This man is at risk because he is an alcoholic and this question tells you he has fallen over and hit his head on the pavement. It is important in the examination to look for signs of trauma such as scalp abrasions and bruises. It is also important in the work up to calculate this patient’s admission GCS, which is deteriorating, possibly as a result of raised intracranial pressure from the SOL. A CT scan will also be indicated. A surgical opinion is indicated. Treatment includes twist-drill craniotomy with drainage (a bedside procedure where a hand drill is used to gain access to the subdural space and then a catheter is placed to act as a drain). Standard craniotomy is also an option, as is the creation of a burr hole. Unlike an extradural there is no ‘lucid interval’. Also extradurals tend to occur in younger patients, usually with an associated skull fracture, and CT of the haematoma does not cross suture lines. In diffuse axonal injury, there will be a history of trauma involving shear or acceleration/deceleration force.

Answer 565

B. Concussion - Concussion is a closed head injury due a blow to the head. This woman has been assaulted, which is a common cause. Typical symptoms include headache (which tends to be cause the most problems in management), mental slowness, memory difficulties, N&V and LOC, though LOC is not necessary for the diagnosis. Symptoms typically go after a week to a month but can fluctuate. CT and MRI are typically normal. For uncomplicated cases, resting is sufficient and no intervention is needed.

Answer 566

C. Extradural haematoma - Here we have the ‘lucid interval’ classically associated with an extradural haematoma. There is blood buildup this time between the dura mater and the skull. Compressive signs may also be present such as the down and out pupil due to CN III compression. The bleed here is usually from arteries, under high pressure, causing raised intracranial pressure. In this case, there is a chance the brain stem has been compressed causing his LOC and GCS

Answer 567

D. Base of skull fracture - This is a basilar skull fracture and a CT scan (superior to MRI), in this case with 3D reconstructions, will be useful. These patients have hit their head head. Basilar skull fractures have specific clinical features. Blood pooling from these fractures can cause periorbital bruising (raccoon eyes), brusing over the mastoid area (Battle’s sign) and bloody otorrhoea. There may also be CSF leak resulting in CSF otorrhoea or rhinorrhoea. A unilateral raccoon eye has an 85% positive predictive value for this diagnosis. Base of skull fractures can also affect cranial nerves leading to hearing impairment (due either to SN as a result of VII injury or conductive due to haemotympanum) and facial numbness or paralysis. These patients also need to have their GCS calculated. Treatment is primarily conservative although surgical intervention may be needed if there is associated CSF leak, intracranial pathology or CN deficit. RTAs are the second most common cause of skull fractures, the first being a fall from a height. There may also, like the first case, be a history of assault resulting in head trauma or even a gunshot to the head.

Answer 568

B. Unfractionated heparin - The mainstay of treatment for acute DVT is anticoagulation. This can be either unfractionated heparin, a LMWH or a factor Xa inhitor like fondaparinux. Fondaparinux has a higher half life than LMWH and there is no effective way of reversing it. LMWH have a shorter half life and some of it can be removed with protamine. Heparin though can be reversed quickly with protamine. Hence, if the patient is at a high risk of bleeding, they should be treated with unfractionated heparin and you should avoid fondaparinux. If they start bleeding you can just chuck them protamine. This however requires monitoring of APTT and platelet counts. If the patient has heparin-induced thrombocytopenia, you can try using fondaparinux. LMWH is recommended in those with active cancer and preferred in pragnancy, and consideration needs to be given in those with renal impairment.

Answer 569

A. Aspirin - The only antiplatelet drug here is aspirin. In those sensitive to aspirin, clopidogrel can be used instead. Aspirin irreversibly inhibits COX1 by acetylating the active site and inhibits platelet TXA2. This reduces the risk of future embolic events. A TIA is colloquially called a ‘mini stroke’ with symptoms typically lasting under an hour. An antiplatelet drug such as aspirin is effective secondary prevention if the patient is not already anticoagulated. The patient will be anticoagulated if they have a likely or known cardioembolic source such as AF.

Answer 570

D. Vitamin K - This patient has clearly been ‘over-warfarinised’. The guidelines are as follows: If there is no bleeding and INR is 6 you need to give PO vitamin K as well. If there is severe bleeding and the INR is high, then you need to stop warfarin and give parenteral vitamin K and PCC (octreotide/octaplex). PCC (prothrombin complex concentrate) is better than FFP in these situations. Remember that warfarin prevents the activation of vitamin K which is a cofactor in the synthesis of factors 2, 7, 9 and 10.

Answer 571

C. Warfarin - This patient here has two risk factors for thromboembolism – AF and valve replacement. This patient will need to be warfarinised to an appropriate INR for anticoagulation. As mentioned above, warfarin acts to inhibit synthesis of the vitamin K dependent clotting factors.

Answer 572

K. Platelet concentrates plus fresh frozen plasma - DIC is a syndrome where coagulation pathways activate resulting in intravascular thrombosis, platelet and clotting factor depletion. The underlying disorder needs to be treated and FFP with platelet concentrate needs to be given. A platelet infusion should be considered and FFP is preferred for replacement of clotting factors and clotting inhibitors. Complications of DIC include life-threatening bleed, ARF and gangrene.

Answer 573

K. Primary hyperparathyroidism - This is the most common cause of hypercalcaemia. 85% are due to a single adenoma in one of the glands while most of the rest are due to 4 gland disease. The range of disease is from very mild/asymptomatic through to severe disease with bone loss, fractures and osteitis fibrosa cystica. There is autonomous PTH production in primary HPT which causes deranged calcium metabolism. Biochemistry will show elevated serum calcium and inappropriate elevation of PTH. Depression, cognitive changes, change in sleep (possibly due to change in circadian rhythm) and myalgia are all common complaints. Osteoporosis may occur due to excess PTH causing bone resorption (osteoclasts are stimulated). Hypercalciuria may cause renal stones and nocturia. The only definitive cure is a parathyroidectomy although complications of this procedure include hypocalcaemia, injury to the recurrent laryngeal nerve, bleeding and a pneumothorax.

Answer 574

A. Myeloma - This patient has multiple myeloma. This is characteristed by clonal proliferation of plasma cells in BM and commonly presents with bony pain and symptoms of anaemia. There may also be infections present in 10%. Elevated ESR agrees with this diagnosis. The diagnostic test is serum or urine electrophoresis looking for a paraprotein spike of IgG or IgA and light chain urinary excretion (Bence Jones proteins). Bone marrow examination and skeletal survey will also need to be conducted. Bone marrow analysis will help differentiate this from MGUS and solitary plasmacytoma. Bone changes include osteopenia, osteolytic lesions and fractures. Younger patients may be candidates for high-dose chemotherapy and autologous transplantation.

Answer 575

C. Sarcoidosis - Sarcoidosis is a chronic multisystem disease with an unknown aetiology but pulmonary involvement usually dominates. Hypercalcaemia occurs in these granulomatous conditions (also including TB and leprosy) as a result of tissue being able to 1-alpha-hydroxylate 25(OH) D leading to vitamin D (the 1,25-dihydroxyvitamin D3) excess. Erythema nodosum, tender erythematous nodules and lupus pernio, indurated plaques with discoloration on the face, are typical skin manifestations of sarcoidosis. CXR will typically show bilateral hilar lymphadenopathy and CXR findings are used in the staging of disease. Additionally, serum calcium and ACE levels may be raised. A transbronchial biopsy is essential for diagnosis in most cases and shows the presence of non-caseating granulomas. Black people have a higher lifetime risk of sarcoidosis, as do those of Scandinavian origin. The mainstay of treatment for severe disease involves systemic corticosteroids.

Answer 576

F. Vitamin D intoxication - This silly lady has self-over-dosed on vitamin D. This is a fat soluble vitamin which can be toxic when taken in excess over time. OD of alfacalciol or calcitriol can easily cause hypercalcaemia and this needs to be avoided. So why is this not milk-alkali syndrome? Milk-alkali syndrome is caused by excess milk, as the name suggests, or alkali, for example due to dyspepsia. Excess intake, or calcium supplementation OTC can lead to hypercalcaemia, so granted if she is buying lots and lots of chewable calcium carbonate or something this is possible, but it is more likely in post-menopausal women on supplementation - just think about what is more likely here. Also, the patient is not completely stupid and will not be OD on vitamin A, even though is a cause of hypercalcaemia.

Answer 577

I. Immobility - Well this one is dead easy. In young people, immobilisation causes bone to demineralise causing hypercalcaemia. This is more common in young people due to larger mobile calcium pools to sustain the rise in calcium levels.

Answer 578

B. Primary hyperaldosteronism - The normal range for potassium 3.5-5mmol/l. You should really know the normal ranges for common values like sodium, potassium and urea by this stage. In Conn’s, potassium is normal or low. It is important when drawing blood to avoid haemolysing the sample, which will cause a falsely elevated potassium level. It is important for screening to calculate the aldosterone/renin ratio, with >30 being suggestive of Conn’s. In Conn’s, aldosterone is raised and renin is low due to negative feedback. This is in contrast to renal artery stenosis where both aldosterone and renin will be raised. It is important to discontinue diuretics and other interfering medications for at least 6 weeks prior to measuring the ratio. The most reliable diagnostic test is a fludrocortisone suppression test. Treatment can be surgical with excision of the adenoma (if aldosterone production is lateralised to one side) or medical with spironolactone and amiloride. There are also familial forms of primary hyperaldosteronism which show an autosomal dominant mode of inheritance. Spironolactone is an aldosterone receptor antagonist. Amiloride inhibits aldosterone-sensitive sodium channels. They are both examples of potassium sparing diuretics acting on the DCT.

Answer 579

D. Pheochromocytoma - Phaeochromocytomas presents with paroxysmal episodes of palpitations, anxiety, excessive sweating, pallor and hypertension. It can be inherited in MEN2, von Hippel-Lindau syndrome and NF1. Diagnosis is based on raised urinary and serum catecholamines, metanephrines and normetanephrines. 24 hour urinary VMA will be elevated. CT is used to localise the tumour. Treatment includes medical with the use of phenoxybenzamine, phentolamine and surgical options. Surgical excision is carried out under alpha and beta blockade to protect against the release of catecholamines into circulation when the tumour is being manipulated. The 10% rule is often quoted: 10% are bilateral, 10% malignant, 10% extraadrenal and 10% hereditary.

Answer 580

F. Metabolic syndrome (Insulin resistance/syndrome X) - Metabolic syndrome incorporates insulin resistance, IGT, central obesity, dyslipidaemia and hypertension. Multiple criteria exist to define this syndrome. This is sometimes called Reaven's syndrome. Treatment aims at lifestyle interventions with statins if these do not achieve desired LDL cholesterol levels. Other lipid-lowering drugs such a fibrates can also be considered. The risk of developing T2DM is up to 5 times higher in those with metabolic syndrome. Low dose aspirin may be indicated, particularly for those at a higher risk, due to the prothombotic state of metabolic syndrome. Other treatments that can be considered include orlistat and bariatric surgery. Those with insulin resistance may benefit from metformin, which will reduce the progression to T2DM in patients with IGT.

Answer 581

C. 'Essential' hypertension - Defined as BP >140/90 or >130/80 in those with renal disease. Typically diagnosed in screening asymptomatic patients. Treatment aims at modifying lifestyle (reducing salt intake to 160/100). Treatment differs for the stages and depends on whether there are any co-morbidities. For example, if there is AF the first choice is a beta blocker and if there is BPH the first choice agent is an alpha blocker.

Answer 582

E. Isolated systolic hypertension - Isolated systolic hypertension occurs more commonly in the elderly. It is characterised by a raised SBP >140 but a normal diastolic BP

Answer 583

D. Autonomic neuropathy - Autonomic neuropathy is a complication of diabetic neuropathy. Symptoms of autonomic neuropathy include... resting tachycardia (late findings due to vagal impairment), impaired HR variation, erectile dysfunction (affects many diabetic men though is not solely due to autonomic neuropathy), decreased libido and dyspareunia, orthostatic hypotension (measure BP supine and then standing after 1, 2, 3 and sometimes 5 minutes – an abnormal drop when standing is indicative) and urinary symptoms of frequency, urgency, incontinence, nocturia, weak stream and retention. Other symptoms include constipation, faecal incontinence and sweating dysfunction. Fludrocortisone may be helpful in this woman.

Answer 584

I. Volume depletion - Volume depletion is a reduction in ECF volume due to salt and fluid losses which exceed intake. Causes include vomiting, bleeding, diarrhoea, diuresis and third space losses. Symptoms do not occur until large losses have alrady occured. Cool peripheries are a sign of peripheral shut down. Confusion may reflect poor cerebral flow or uraemia.Volume depletion has led to the low BP. Other symptoms include postural hypotension and tachycardia, weight loss and signs of shock. Serum urea and creatinine is elevated (you need to eyeball the patient when looking at creatinine – a very big body builder will have a much higher creatinine), indicating poor renal blood flow. This patient needs IV saline fluid replacement.

Answer 585

F. Hyperaldosteronism - Hyperpigmentation in the palmar creases points towards Addison’s disease. Hyperpigmentation due to excess ACTH production can be mucosal or cutaneous and is more pronounced in the palms, knuckles and around scars. MSH is a byproduct of the production of ACTH from the cleavage of POMC. Sodium is low and potassium elevated. Vomiting is present in 75% of patients and nausea is a common finding. Additionally, postural hypotension may be present. The presence of other autoimmune diseases is also a risk factor for the development of Addison’s.

Answer 586

G. Cardiogenic shock - Cardiogenic shock is pump dysfunction. This may occur, like in this case, after MI (shock complicates just under 10% of MIs) or may be due to cardiomyopathy, valve dysfunction or arrhythmias. This cause of shock in this patient is obviously apparent. Clinical signs of shock include stress responses of tachycardia and tachypnoea, hypotension (

Answer 587

H. Drug induced - Opioid OD symptoms include CNS depression (drowsiness, sleepiness), respiratory depression and relative bradycardia. This patient needs ventilation prior to the administration of naloxone, titrated to patient response.

Answer 588

D. BNF - The BNF will tell you about indications and contraindications for drugs

Answer 589

A. Cocherane database - The Cochrane database was established by the NHS with the aim of being a place to review existing literature on a subject matter.

Answer 590

B. Medline - Medline, or pubmed, is run by the US Government and will help you search through many journals with keywords to look for new and older research on a matter

Answer 591

C. NICE - In the UK, NICE currently analyses the medical and cost-effectiveness of various treatment options and publishes guidelines based upon this

Answer 592

D. BNF - The BNF will tell you about indications and contraindications for drugs

Answer 593

A. CT chest, abdomen and pelvis - Dissecting aneurysms are either type A, which involves the ascending aorta, or type B. Type A dissections require urgent surgery whereas type B can be managed medically if it is not complicated by end organ ischaemia. A CT scan is indicated as soon as a diagnosis of aortic dissection is suspected and should be from the chest to the pelvis to see the full extent of the dissecting aneurysm. What you will see is the intimal flap. MRI is more sensitive and specific but is more difficult to obtain acutely.

Answer 594

J. MRI head - Pituitary MRI is preferred over CT and you will be able to see if the tumour has invaded, for example, the sphenoid sinus and cavernous sinuses or any compression of the optic chiasma. MRI is contra-indictaed in some cases such as those with a permanent pacemaker or those with ESRF on dialysis – in which case you would perform a CT. Both are done with contrast enhancement. A sellar mass will be seen.

Answer 595

C. Duplex ultrasound - Renal artery stenosis is basically narrowing of the renal artery. There may not be any clinical consequences of this – just because someone’s renal arteries are narrowed does not mean they are suffering worsening kidney function, although this may be the case, especially after blockade of the renin-angiotensin system, and patients may have difficult to control and accelerated hypertension. A definitive diagnosis is made on imaging, where there is some controversy on what is most appropriate to use. USS is safe and non-invasive but the sensitivity and specificity is low. CT/MR angiography has the risk of contrast nephropathy and nephrogenic systemic fibrosis. Conventional angiography (the best test available) has the risk of bleeding and emboli as well as contrast related risks already mentioned. Generally, the recommendation is to start with renal duplex ultrasound. This would not be an unreasonable approach. This can be followed by further tests. Although, in some centres in the country, the first line is CT or MR angiography and duplex USS is only done if there is a contra-indication to CT/MR angiography. However, the only reasonable option on this list is duplex USS.

Answer 596

I. Barium swallow - A barium swallow is the test you should do here. It is often done with fluoroscopy and may be normal in early disease. What will be seen is a loss of peristalsis and delayed emptying of the oesophagus. The oesophagus will be dilated proximally, and tapers distally into a ‘bird’s beak’ shape narrowing at the gastro-oesophageal junction.UGI endoscopy has a low sensitivity and is only done to exclude malignancy. However, it is an essential first line test for this very reason to evaluate the presentation of dysphagia.

Answer 597

B. Ventilation-perfusion scan - The first line recommended initial imaging test is a CT chest to directly visualise the thrombus in a pulmonary artery, which would show as a filling defect. However this option is not given in this list and the test to pick here is a V/Q scan, which offers a similarly high level of sensitivity and specificity. In a PE the area affected will be ventilated but not perfused.

Answer 598

K. Tumour lysis syndrome - There is recent diagnosis of malignancy here combined with the recent start of both chemotherapy and radiotherapy. This is tumour lysis syndrome which encompasses metabolic and electrolyte abnormalities, like hyperkalaemia, occuring after cytotoxic treatment in a patient with cancer. There is excessive cell lysis and the release of intracellular contents into the bloodstream leads to elevated levels of serum urate, potassium, phosphate and a reduction in calcium level.

Answer 599

L. Pseudohyperkalaemia -This is pseudohyperkalaemia caused by haemolysis of the sample. The medical student who has had some difficulty drawing the blood has haemolysed the sample. Potassium in serum will in this case exceed the plasma value by >0.5 mmol/L and the pink tinge when centrifuging the sample will also give this away.

Answer 600

B. Rhabdomyolysis - This is a crush injury that has caused myocyte lysis – rhabdomyolysis. The diagnosis would be confirmed by raised CK. The swelling and pain in his leg muscle is a further give away. The dark urine here is caused by urinary myoglobin. The long time it took for him to be retrieved is also an indication of this diagnosis. The mainstay of treatment is with fluid hydration.

Answer 601

E. Addison's disease - Hyperpigmentation is due to excess ACTH production and can be mucosal or cutaneous and is more pronounced in the palms, knuckles and around scars. MSH is a byproduct of the production of ACTH from the cleavage of POMC. Sodium is low and potassium elevated in this condition. Additionally, postural hypotension may be present. The presence of other autoimmune diseases is a risk factor for the development of Addison’s, such as this man’s vitiligo. Diagnosis of Addison’s can be made on an ACTH stimulation test (synacthen test) whereby serum cortisol remains low despite the administration of synthetic ACTH. In an emergency, treatment should not be delayed by diagnostic testing.

Answer 602

H. DKA - This is DKA. This girl is simply lying about her insulin and has been skipping insulin doses in order to lose weight. This is known as ‘diabulimia’. The main thing that needs to be corrected is the severe intravascular volume depletion and to restore tissue perfusion with IV saline. Insulin will of course also be needed. When glucose reaches 11.1mmol, fluid should be changed to 5% dextrose to prevent hypoglycaemia. Insulin should be held until potassium is at least 3.3 mmol/L (remember insulin moves potassium into cells) and a continuous infusion is recommended (with new DKA treatment guidelines, the ‘sliding scale’ is now a thing of the past). If interested, look up the latest DKA treatment guidelines for more information.

Answer 603

J. Upper GI endoscopy - NSAID use is a key risk factor for a peptic ulcer. Other key risks include H. pylori infection, smoking and FH of PUD. Zollinger-Ellison syndrome should be considered if there are multiple ulcers or ulcers refractory to treatment. The epigastric pain and symptoms of dark stools are consistent with a bleeding peptic ulcer. The most specific and sensitive test is an upper GI endoscopy which also allows management of the bleed. A biopsy may also be done to rule out malignant transformation. Gastric ulcers require a compulsory biopsy but duodenal ulcers rarely undergo malignant change. Management here can be aimed at discontinuing NSAIDs which are the cause in this case.

Answer 604

B. Chest X-ray - The blood being brought up here occurs at a fixed time period every day and he is not anaemic. This makes you suspect a respiratory cause of his haemoptysis such as pneumonia – particularly TB, cancer, vasculitis like Wegener’s, bronchiectasis and bronchitis.

Answer 605

A. Clotting profile - This woman is on prophylactic warfarin anticoagulation. There is an interaction here with the antibiotics this woman is taking which has resulting in enhanced anticoagulation effects of warfarin, causing her to bring up the blood. Cepahalosporins, chloramphenicol, ciprofloxacin, clarithryomycin, erythromycin and metronidazole are all examples which increase the effect of warfarin. Any P450 inducer will have this effect as warfarin is a drug metabolised by cytochrome P450 enzymes. Antibiotics can also upset the gut flora which reduces vitamin K levels.

Answer 606

C. Upper GI endoscopy - The definitive diagnosis for a Mallory-Weiss tear is made by OGD. This tear in the oesophageal mucosa occurs after a rise in abdominal pressure, causing subsequent GI bleeding. It commonly presents with haematemesis after an episode of retching/vomiting/coughing/straining. Hence, risk factors include anything which can cause vomiting like heavy alcohol use, which is commonly the case in EMQs. Also, other conditions would include food poisoning, bowel obstruction, hyperemesis gravidarum, bulimia, the chronic cough of COPD, meningitis etc... you name it really. Classically, MWT presents with a small self limiting episode of haematemesis. Treatment is supportive because most cases are self limiting and emergency treatment is not offered unless the patient is showing signs of clinical instability. If the patient is actively bleeding, treatment will be with therapeutic endoscopy in most cases, and very very few cases will require more intervention such as angiography with embolisation.

Answer 607

F. CT scan abdomen - The cause here could be an aortoenteric fistula or AVM. You have to assume with endoscopy here that upper and lower GI endoscopy were unremarkable. The next step on this list would be a CT scan with contrast to detect the GI bleed. You would expect to see the active bleeding with contrast leaking into the bowel lumen.

Answer 608

A. Renal ultrasound - A dominantly inherited cause of renal failure is likely to be polycystic kidney disease which is best noted on ultrasound

Answer 609

A. Renal ultrasound - A patient with cervical carcinoma is at risk of ureteric obstruction and then hydronephrosis. Again ultrasound is the test of choice

Answer 610

B. CVP measurement - A confused hypotensive shocked patient might be hypotensive because of cardiac disease (which will cause a raised JVP) or due to sepsis (which will cause a low JVP). If you can't see the JVP, then you should put in a central line to measure the CVP accurately to tell you if the cause of the low blood pressure here is sepsis or cardiac failure

Answer 611

C. Renal biopsy - When the cause of renal failure might be nephritis (as in nephrotic syndrome) a renal biopsy will be needed to make the diagnosis. Occasionally this is urgent, so that potent immunosuppresives can be administered

Answer 612

D. Plasma electrophoretic strip - Renal impairment in myeloma results from a combination of factors: deposition of light chains, hypercalcaemia, hyperuricaemia and (rarely) in patients who have had the disease for some time, deposition of amyloid. Serum protein electrophoresis characteristically shows a monoclonal band.

Answer 613

E. Benign oesphageal stricture - Healing of oesophageal damage inflicted in GORD involves the deposition of collagen. This causes contraction of the distal oesophagus which causes the formation of strictures. This is often associated with dysphagia for solids. Other complications of GORD include oesophageal ulcer, haemorrhage or perforation, Barrett’s oesophagus and oesophageal adenocarcinoma.

Answer 614

J. Achalasia - This is achalasia which is a motility disorder with loss of peristalsis in the distal oesophagus and failure of the LOS to relax in response to swallowing. This presents commonly with dysphagia to both liquids and solids, regurgitation and retrosternal chest pain, which can be slowly progressive over time. In structural obstruction such as cancer, dysphagia to liquids is uncommon unless the disease is very advanced. Retrosternal pressure experienced can be precipitated by drinking liquids but is eased by continuing to drink, and the pain may be relieved by cold water. This may wake the individual from sleep. A UGI endoscopy is needed to exclude malignancy as a cause of dysphagia. The diagnosis is established on manometry or barium studies. Treatment is symptomatic.

Answer 615

K. Oesophageal cancer - Dysphagia (normally in a progressive pattern) coupled with weight loss points to malignancy. Dysphagia occurs when there is obstruction of more than 2/3 of the lumen and presence indicates locally advanced disease. There may additionally be odynophagia. Lymphadenopathy is a sign of metastatic disease here. Men are twice as likely to develop oesophageal cancer. GORD, Barrett’s oesophagus, FH, tobacco and alcohol are all risk factors. The two main types are squamous cell carcinoma and adenocarcinoma. Tumours in the upper 2/3 of the oesophagus are SCC whereas those that lie in the lower 1/3 are adenocarcinomas. The main test to order is an OGD with biopsy. Treatment is either surgical resection or with chemo or radiotherapy alongside endoscopic ablation with or without stenting and brachytherapy.

Answer 616

G. Candidal oesophagitis - This is really odynophagia rather than true dysphagia. Whenever a patient complains of ‘difficulty swallowing’, you should always take a good history and explain what the patient actually means by this. Does the patient mean that there is true difficulty swallowing, or just that it is painful to swallow, or is there a feeling of a lump in the throat (globus). The white plaques here give alongside discomfort away the diagnosis. The patient also has AIDS and is therefore immunocompromised. Candidiasis here is an opportunistic infection in an immunocompromised host and is a mucosal infection caused in most cases by Candida albicans (and occasionally by other species). Patients may present like this, or with oral or vaginal infection. Oral thrush may be seen (pseudomembranous candidiasis) with altered taste sensation or indeed dysphagia, as well as odynophagia in oesophageal disease. In vaginal infection, there is erythema with a white discharge and pruritis. This patient should be started on an empirical trial of antifungals. A systemic azole can be used such as fluconazole or itraconazole.

Answer 617

D. Globus hystericus - Globus hystericus is a sensation of fullness or a lump in the neck or difficulty swallowing which is not a true case of dysphagia. Swallowing can be performed normally and there is no real lump or obstruction in the throat. In some cases the cause is unknown and is believed to be psychogenic in cause and is associated with anxiety disorders. In other cases throat inflammation can cause this sensation. The normal examination and endoscopy here in a patient with a psychiatric history is diagnostic.

Answer 618

``` In terms of karyotype analysis, the following are some conditions to be aware of: Trisomy 21: Down’s; Trisomy 18: Edwards’; Trisomy 13: Patau’s; 45 XO: Turner’s, 47 XXY: Klinefelter’s; 47 XXX: Triple X syndrome; Microdeletion at 22q11: DiGeorge; Microdeletion at 7q11: William’s; 5p-: Cri-du-chat ``` D. Klinefelter's syndrome - Klinefelter’s syndrome is the presence of an extra X chromosome in a male to give 47, XXY. Hypogonadism is a principle feature of this condition and there is reduced fertility. Hypogonadism itself does not mean ‘small testicles’ but XXY men do also have small testicles. They will also often have low testosterone levels but high LH and FSH levels due to primary hypogonadism. The only reliable method of diagnosis is with karyotype analysis and the degree to which XXY males are affected varies from person to person. Gynaecomastia is to some extent present in around a third of individuals affected by this condition. 1 in 10 will choose cosmetic surgery to fix this.

Answer 619

``` In terms of karyotype analysis, the following are some conditions to be aware of: Trisomy 21: Down’s; Trisomy 18: Edwards’; Trisomy 13: Patau’s; 45 XO: Turner’s, 47 XXY: Klinefelter’s; 47 XXX: Triple X syndrome; Microdeletion at 22q11: DiGeorge; Microdeletion at 7q11: William’s; 5p-: Cri-du-chat ``` A. Edwards syndrome - Edwards syndrome is trisomy 18 and is phenotypically similar to Patau’s. Most affected are females and most die before birth. The incidence, as with most trisomies, increases with advanced maternal age. The rate of survival is low due to cardiac abnormalities, renal malformations and other visceral disorders. Signs and symptoms include those mentioned and a whole host of other signs of this phenotype such as a small head, small jaw, widely spaced eyes and ptosis. The cardiac defects seen include VSD, ASD and PDA (all the lovely 3 letter acronyms). Classic EMQ signs include webbing of the second and third toes and the Rocker bottom feet characterised by calcaneal prominence and a convex rounded bottom to the foot, which is associated with both trisomy 13 and 18.

Answer 620

C. Fragile X syndrome - This is fragile X syndrome. History includes learning difficulties, which can range from mild to severe, social communication difficulties (patients may be autistic), hyperactivity and attention deficit and motor co-ordination difficulties. There may be a FH of learning difficulties too. Examination may reveal macrocephaly, low muscle tone, long face, high arched palate, prominent jaw, big testicles (macro-orchidism), large ears and strabismus. DNA testing is diagnostic and reveals a fragile site on Xp27.3 (FRM1 gene position). This is characterised by trinucleotide repeat expansion (CGG) to more than 200 copies.

Answer 621

F. Downs syndrome - This is the one you need to be really aware of. Down’s syndrome is trisomy 21 and the diagnosis is one which is made antenatally or perinatally. You will never have a patient with Down’s who gets diagnosed as a child unless you are in a country which is very deprived of any medical personnel and your patient was born in a rural farm away from civilisation. The patient may have a history of delayed development, congenital cardiac anomalies, epilepsy as a child, atlanto-occipital instability, GI or hearing problems and there may also be associated autism. Examination may display dysmorphism, oblique palpebral fissures, epicanthic folds, low nasal bridge and low set ears, characteristic central iris Brushfield spots, short curved 5th finger, single palmar crease and may also have cardiac murmurs. Karyotype analysis will reveal trisomy 21, robertsonian translocation, or mosaicism.

Answer 622

E. Turners syndrome - Turner’s syndrome is characterised on diagnostic karyotype analysis by 45 XO (complete or partial absence of the second sex chromosome occuring in 1 in 2500 liver female births). This may be diagnosed antenatally by amniocentesis, which is an invasive test performed after 15 weeks gestation. The patient will be phenotypically female and may give a history of amenorrhoea, infertility, visual problems and hearing loss. Specific learning difficulties are normal but there is no cognitive impairment and intelligence is normal. Examination may reveal short stature, low-set ears, webbed neck, low hairline and cubitus valgus. Obvious stigmata though such as webbed neck only affects 20-30% of patients. I wouldn’t worry too much about most of these syndromes. A brief overview of the rest for those who are interested: Prader-Willi is characterised by 15q11-13 deletion and patients can have a short stature, small hands and feet, and dysmorphism. The classic association is with hyperphagia and obesity from 3 years of age. Angelman’s is characterised by 15q11-12 deletion and there is severe general developmental delay and cognitive impairment. William’s is characterised by deletion of 7q11.23 and has classic phenotypic features. DiGeorge classically presents with a triad of cardiac abnormalities, hypoplastic thymus and hypocalcaemia but manifestations are highly variable. Tuberous sclerosis is characterised by seizures in infants and children, variable cognitive impairment and examination may reveal ash leaf patches of hypomelanotic macules. There is either mutation of TS1 or TS2 on DNA analysis.

Answer 623

B. Benzodiazepines - This woman here who is clearly distraught after her breakup has overdosed on benzodiazepines. The clue here is given when it says she is taking medication for panic attacks at the moment. BZDs are the most commonly prescribedmedication for anxiety disorders, sedation and sleep. Patients may present like this and may be intentional or accidental in nature, and may be in combination with other CNS depressants such as alcohol and opioids in older people. Occasionally overdose is due to medication error. The key feature of overdose is excessive sedation and anterograde amnesia. Vital signs are unremarkable. Larger doses can lead to coma and respiratory depression. Treatment is symptomatic and may include assisted ventilation and haemodynamic support and death is uncommon and often due to mixed overdoses with other depressants such as alcohol. Flumazenil is a BZD antagonist that can be used in first time or infrequent users to reverse CNS depression but it is contraindicated in those who are long-term of frequent users (like this patient) due to the risk of provoking seizures, which outweights the benefits.

Answer 624

M. Sympathomimetics - The symptoms described here are those of sympathetic activation and the overdose here is of sympathomimetics. This group of drugs mimic the effects of transmitter substances of the sympathetic nervous system such as adrenaline, dopamine and noradrenaline.

Answer 625

A. Salicyclates - This is salicylate overdose which is potentially fatal and can present either acutely or indolently with more chronic exposure. It is a relatively common overdose so really with anyone presenting with an unknown overdose it should be considered along with paracetamol. The unexplained acid-base disturbance should make you suspicious of this diagnosis. Tinnitis is common in the early stages of acute salicylate poisoning and reflects CNS toxicity. There may also be deafness and both are reversible. Seizures are common especially in patients with salicylate levels >80mg/dL. An ABG is also indicated in this patient which during the course of salicylate poisoning would initiually show a respiratory alkalosis and later a concomitant metabolic acidosis, potentially with a wide anion gap. GIT decontamination should be considered as an adjunct on arrival to A&E and activated charcoal can be given. The mainstay of treatment is alkaline diuresis induced by an infusion of sodium bicarbonate. In cases of severe poisoning, it is still started as a bridge to haemodialysis.

Answer 626

H. Carbon monoxide - This man who works in a petrol refinery is suffering from carbon monoxide poisoning and his co-worker who has found him lying on the ground is starting to suffer from similar symptoms due also to CO exposure. CO is a colourless and odourless gas so patients may not initially be aware of the poisoning which can cause hypoxia, cell damage and death (in approximately 1/3). CO can come from fire or non-fire sources and early symptoms are non-specific and include the symptoms seen here: headache, dizziness and nausea. Increasing exposure leads to cardiovascular effects like myocardial ischaemia, infarction and possible arrest. Neurological symptoms include the confusion seen here and may lead eventually to coma and syncope. Diagnosis is based on carboxyhaemoglobin levels and the clinical picture here is very suggestive. It is worth noting that in severe CO poisoning, pulse oximetry readings may be falsely raised. Key in treatment is the use of high-flow oxygen, hyperbaric oxygen and supportive care. Hyperbaric treatment entails complications such as barotrauma, oxygen toxicity and pulmonary oedema.

Answer 627

E. Tricyclic antidepressants - This is an overdose of tricyclic antidepressants which are a class of drugs with a narrow therapeutic index and therefore become potent toxins in moderate doses to both the CNS and cardiovascular system. This patient has reason to be depressed and is therefore on these pills. There is a sudden deterioration of mental status here and the diagnosis here is clinical. The main aim in treatment is to provide respiratory and cardiovascular support until the medicine has been fully metabolised and eliminated. The warm, dry and flushed skin is part of the anticholinergic effects (physostigmine should NOT be used to reverse this as it has been in rare cases been associated with asystole – would you rather have a patient who is flushed or flatlined?). Other anticholinergic effects include dilated pupils, urinary retention, decreased or absent bowel sounds and changes in mental status. Hypotension is common and is due to alpha 1 antagonism. Classic ECG changes are of sinus tachycardia progressing to wide complex tachycardia and ventricular arrhythmias (with increasing severity and intoxication). Condution problems and hypotension is improved with hypertonic sodium bicarbonate and if arrhythmias are present, treatment of these involves correcting the acidosis, hypoxia and electrolyte abnormalities. Anti-arrhythmics are generally avoided. If hypotension is refractory then a vasopressor can be used. BZDs can be used for any seizures.

Answer 628

E. Thyrotoxicosis - This woman has symptoms and signs of hyperthyroidism (diarrhoea, weight loss, AF and a fine tremor). In countries where sufficient iodine intake is not an issue, Graves’ disease is the most common cause of hyperthyroidism. Peripheral manifestations such as ophthalmopathy, pretibial myxoedema and hyperthyroid acropachy do not occur with other causes of hyperthyroidism. Acropachy manifests as clubbing with soft tissue swelling. Pretibial myxoedema is almost always associated with ophthalmopathy. Treatment aims to normalise thyroid function and is achieved by radioactive iodine, antithyroid medications or with surgery. They are all effective and relatively safe options. Symptomatic therapy is given with beta blockers such as propranolol.

Answer 629

H. Irritable bowel syndrome - The intermittent diarrhoea and constipation, with bloating, without symptoms suggestive of IBD make IBS a more likely diagnosis. IBS is a chronic condition with abdominal pain associated with bowel dysfunction and is a diagnosis of exclusion. The pain or discomfort may be relieved by defecation. Examination is usually unremarkable and the diagnosis is based on the patient’s history in line with the Rome Criteria. If the patient presents with any worrying symptoms, then these will warrant a more thorough investigation. Treatment depends on the patient’s predominant symptoms. Antispasmodics relieve abdominal pain or discomfort but do not affect bowel habit. Examples include peppermint oil and dicycloverine. Laxatives can also be used such as lactulose. Lifestyle and dietary modifications combined with reassurance remain the 1st line intervention for functional bowel disease. IBS is linked with stressful jobs such as working as a banker in the city and there is a female/male ratio of 2:1.

Answer 630

A. Bacterial gastroenteritis - This acute presentation in a previous fit and healthy individual and the raised neutrophils on FBCand a fever indicate an infective cause for his GI symptoms. It is self-limiting and diagnosis is on isolating the organism from a stool culture (if needed). Blood in the stool allows you to narrow down the list of potential causative organisms. Treatment is supportive with fluid and electrolyte replacement and antibiotics are generally used only for patients with risk factors for severe disease or those with extra-GI complications. It is worth noting that viral gastroenteritis often presents with mainly UGI symptoms like N&V more so than diarrhoea.

Answer 631

L. Clostridium difficile - This is infection with clostridium difficile with the major risk factor here of antibiotic exposure due to the recent chest infection. The most common ones implicated are ampicillin, second and third generation cephalosporins, clindamycin and fluoroquinolones, especially if used in the preceding 3 months (though most manifestations occur on days 4 through to 9 of antibiotic therapy). Diarrhoea may range from a few loose stools to severe diarrhoea, though absence could be related to toxic megacolon to paralytic ileus. Abdominal pain is also common as is fever. C. difficile produces 2 exotoxins which are responsible for its pathogenicity. These are called toxin A and toxin B (A is thought to be more important than B) which lead to an inflammatory response in the large bowel, increased vascular permeability and the formation of pseudomembranes. Colonic pseudomembranes look like raised yellow and white plaques against an inflamed mucosa and are composed of neutrophils, fibrin, mucin and cellular debris. The diagnostic standard is with cytotoxic tissue culture assay. Treatment involves discontinuing the implicated antibiotic and beginning oral metronidazole or vancomycin. 5-20% will have a recurrence on discontinuing treatment and will need a second course.

Answer 632

J. Ulcerative colitis - While this could be Crohn’s disease, bloody diarrhoea is more commonly a presentation of UC than Crohn’s. UC is characterised by diffuse mucosal inflammation running a relapsing and remitting course. Bloody diarrhoea is commonly experienced by patients who may also complain of other symptoms such as (lower) abdominal pain, faecal urgency and the host of extra-intestinal manifestations associated with UC. These include erythema nodosum, pyoderma gangrenosum, sacroiliitis, ankylosing spondylitis, PSC, aphthous ulcers, episcleritis, peripheral arthropathy and anterior uveitis. Another clue in this question which makes you pick UC instead of Crohn’s is the fact the patient has given up smoking. While I remain convinced this link as a risk factor is a weak one, you should try to think like an EMQ when answering EMQs (generally the information is there for a reason). There is a weak risk of UC development in non-smokers and those who were a former smoker (though it is an established link). This is based on a review paper published by some German medics in an exciting journal named ‘Inflammatory Bowel Diseases’. Should be you interested you can check it out: Inflammatory Bowel Diseases. 10(6):848-859, November 2004 (just read the abstract if you want) Diagnosis of UC requires endoscopy with biopsy and a negative stool culture to rule out infectious gastroenteritis. Flare ups are usually linked to pathogens so a stool culture will always be needed in these cases. Toxic megacolon is a complication which is associated with a risk of perforation. UC is also linked with bowel adenocarinoma and PSC. Treatment involves mesalazine (5-ASA) used to induce and maintain remission.

Answer 633

L. Pericarditis - This patient has presented with pericarditis – most likely viral following a viral infection (as suggested by the prodrome and fever). Symptoms include a sharp and severe chest pain retrosternally which is worse on inspiration and when supine, relieved by sitting forwards. The classical finding on examination is a friction rub which is said to sound like ‘walking on snow’ – a monophasic, biphasic or triphasic friction rib is pathognomic with close to 100% specificity. There may be diffuse ST elevations on ECG, an effusion on echocardiography and blood results suggesting inflammation. Complications include tamponade and constrictive pericarditis. Prior viral infection is a risk factor with the most common pericardial infection being viral. Bacterial purulent pericarditis also occurs. The inflammation is due either to direct viral attack or immune mediated damage. Other risk factors include male gender, post-MI (both ‘early’ and Dressler’s), post-pericardiotomy syndrome, neoplasm from local tumour invasion, uraemia and autoimmune conditions such as RA and SLE.

Answer 634

H. Mitral regurgitation - MR can occur as a complication of MI which may cause structural damage to the mitral valve apparatus. MR is loudest at the apex and radiates to the axilla and tends to be around grade 4. It is associated with a systolic thrill at the apex. TTE is the investigation of choice for diagnosis. Chronic MR is associated with a laterally displaced apex beat with LV dilatation. This case of acute MR in the setting of an acute MI is very serious can lead to high LA pressure and pulmonary oedema secondary to reduced LA compliance. Occasionally no murmur is heard. Note that while a VSD also gives a pansystolic murmur, which is generally easily heard, and is loudest at the left parasternal region, with no axillary radiation.

Answer 635

A. Tuberculosis - It is important to have a high level of suspicion when evaluating patients with risk factors who present with suggestive symptoms. Night sweats, fever, weight loss, malaise, cough, haemoptysis and erythema nodosum are all suggestive. In the first half of the 20th century, tuberculosis accounted for over 90% of cases of erythema nodosum. Other key risk factors for pulmonary TB include exposure to infection and returning from or being born in a high-risk region such as Asia, Africa and Latin America (highly possible in this case). If TB is suspected, the patient should be placed in isolation and a CXR obtained with 3 sputum samples cultured for AFB being the gold standard of diagnosis. Culture takes several weeks so sputum smears will be done before culture results are known. Interferon-gamma release assays (IGRAs) are now used by some hospitals to rapidly determine a patient’s TB status. All patients who have TB should be tested for HIV within 2 months of diagnosis. Specific anti-TB medication is required, not standard antibiotics for community-acquired pneumonia. The CXR in pulmonary TB is almost always abnormal in patients who are not immunocompromised. Patients with, for instance, advanced HIV, may have a normal CXR. Primary disease common presents as middle and lower zone infiltrates and ipsilateral adenopathy, atelectasis from airway compression and pleural effusion can be seen. Post-primary classically involves apical changes, spreading to other lobes/segments as the disease progresses. The CXR may be normal in normal individuals but it is rare and it is likely only part of this patient’s CXR is reported here so as not to completely give the game away in this EMQ. Or whoever was interpreted it should not have been allowed to pass finals, which is also possible.

Answer 636

I. Dilated cardiomyopathy - It is worth noting that ventricular hypertrophy due to hypertension causes concentric hypertrophy i.e. the wall of the ventricle gets thicker inwards. Hence the apex beat is not displaced unlike in DCM. DCM is characterised by LV dilation and systolic dysfunction without significant coronary artery disease or abnormal loading conditions. RV dilation is often also present. 25-35% are familial (there may be FH of sudden death). Causes are extensive and include post-myocarditis, alcohol, chemotherapy agents, haemochromatosis, AI conditions and acromegaly. This case is alcohol related DCM with a history of alcohol excess, signs of chronic liver disease on examination and signs of systolic dusfunction on examination (crackles at lung bases, JVP distension and there may also be peripheral oedema). ECG may show non-specific ST-T changes, CXR can show an enlarged cardiac shadow and echo also give consistent results (wall thickness, LV dilation). LFTs, serum albumin and clotting profile may all be abnormal here too, and GGT would especially be expected to be elevated due to alcohol abuse.

Answer 637

F. Pericardial effusion - This is a malignant effusion (one of the most likely to lead to tamponade) caused by lung cancer. Other prevalent malignant causes include breats cancer, lymphomas and leukaemias. This may also be the first sign of metastatic disease. The history of lung cancer here should make you suspicious. Other causes of a pericardial effusion include hypothyroidism (high protein content and accumulate very slowly due to capillary leak), cardiac causes such as CHF and dissection of the proximal aorta, trauma, radiation-related, uraemia, immune-mediated such as SLE, Dressler’s, amyloidosis and Wegener’s, infectious or idiopathic (which is generally assumed to be viral). Symptoms may coexist with those of pericarditis sometimes. ECG and CXR are indicated here. Most patients also get an echocardiogram whic is the preferred test to establish the diagnosis. On ECG there may be diffuse ST elevation and PR depression with epicardial inflammation. If the effusion is large enough there may be electrical alternans, which is beat-to-beat variation of the ventricular axis (find an image of this to cement it in your memory). The cardiac shadow on CXR is said to be ‘water-bottle shaped’. Pericardiocentesis may be necessary depending on the clinical case.

Answer 638

E. Gastric ulcer - The patient has a bleeding peptic ulcer (the black tarry stools from the UGI bleed). Epigastric pain and tenderness related to eating a meal is typical of a peptic ulcer. 80% are duodenal and 20% are gastric. Ulcers may cause iron deficiency anaemia and associated symptoms may feature. Key risk factors are NSAID use, like in this patient, H. pylori infection, smoking and a family history of PUD. Zollinger-Ellison syndrome should be considered if there are multiple ulcers or ulcers refractory to treatment. Gastric ulcers classically cause pain which is exacerbated by eating and immediately relieved on vomiting. There is usually also weight loss due to a fear of food and its association with pain. Duodenal ulcers are classically made worse by hunger and are relieved by eating and the patient may wake at night with the pain. As a result, weight gain is typically a feature. In reality, it is difficult to differentiate the site of the ulcer based on these features. The most specific and sensitive test is an upper GI endoscopy which is initially ordered if the patient has ‘red flag’ symptoms, is >55 years of age or fails to respond to treatment. Duodenal ulcers rarely undergo malignant transformation so do not require a compulsory biopsy but gastric ulcers require biopsies to rule this out. In patients who are 55 or younger without ‘red flags’, testing for Helicobacter pylori (breath testing with radiolabelled urea or stool antigen testing) is necessary. Management is aimed at correcting the underlying cause such as discontinuing NSAIDs. H. pylori eradication should be started if the organism is present with triple therapy. Otherwise, a PPI is indicated.

Answer 639

B. Chrones disease - This patient gives a history of IBD. This could well be UC where the mainstay of treatment is with 5-ASA. A colonoscopy is required to assess the extent of disease and for a definitive diagnosis. Biopsy in CD will show transmural granulomatous inflammation. CD can affect the whole GIT but favours the TI (RLQ pain) and proximal colon and is macroscopically characterised by skip lesions. UC on the other hand is characterised by the presence of crypt abscesses, which is pathognomic. CD risk is increased 3-4 fold by smoking whereas smoking seems protective in UC. The mainstay of treatment in CD is with steroids and azathioprine to revent relapses and for those suffering side effects of steroid treatment. TNF-alpha inhibitors also have a role. Surgery in CD is only indicated in a small number of patients who bleed, for bowel perforation and cases of complete obstruction. The aim is to rest distal disease by temporarily diverting faecal flow.

Answer 640

F. Mallory-Weiss tear - This occurs after a rise in abdominal pressure which induces a tear in the oesophageal mucosa, causing subsequent GI bleeding. It commonly presents with haematemesis after an episode of retching/vomiting/coughing/straining. Hence, risk factors include anything which can cause vomiting like heavy alcohol use, which is commonly the case in EMQs. Also, other conditions would include food poisoning like in this case, bowel obstruction, hyperemesis gravidarum, bulimia, the chronic cough of COPD, meningitis etc... you name it really. Classically, MWT presents with a small self limiting episode of haematemesis. Definitive diagnosis is made by OGD. Treatment is supportive because most cases are self limiting and emergency treatment is not offered unless the patient is showing signs of clinical instability. If the patient is actively bleeding, treatment will be with therapeutic endoscopy in most cases, and very very few cases will require more intervention such as angiography with embolisation.

Answer 641

H. Oesophageal malignancy - Such levels of extreme weight loss over a short period of time with GI symptoms here points to GI malignancy which is bleeding. The only option on the list is oesophageal. EMQs normally mention dysphagia, which occurs when there is obstruction of more than 2/3 of the lumen and presence indicates locally advanced disease however this is absent here. There may additionally be odynophagia. Men are twice as likely to develop oesophageal cancer. GORD, Barrett’s oesophagus, FH, tobacco and alcohol are all risk factors. The two main types are squamous cell carcinoma and adenocarcinoma. Tumours in the upper 2/3 of the oesophagus are SCC whereas those that lie in the lower 1/3 are adenocarcinomas. The main test to order is an OGD with biopsy. Treatment is either surgical resection or with chemo or radiotherapy alongside endoscopic ablation with or without stenting and brachytherapy.

Answer 642

A. Duodenal ulcer - The patient has a duodenal ulcer (the black tarry stools from the UGI bleed). Epigastric pain and tenderness related to eating a meal is typical of a peptic ulcer. 80% are duodenal and 20% are gastric. Ulcers may cause iron deficiency anaemia and associated symptoms may feature. Key risk factors are NSAID use, like in this patient, H. pylori infection, smoking and a family history of PUD. Zollinger-Ellison syndrome should be considered if there are multiple ulcers or ulcers refractory to treatment. Gastric ulcers classically cause pain which is exacerbated by eating and immediately relieved on vomiting. There is usually also weight loss due to a fear of food and its association with pain. Duodenal ulcers are classically made worse by hunger and are relieved by eating and the patient may wake at night with the pain. As a result, weight gain is typically a feature. In reality, it is difficult to differentiate the site of the ulcer based on these features. The most specific and sensitive test is an upper GI endoscopy which is initially ordered if the patient has ‘red flag’ symptoms, is >55 years of age or fails to respond to treatment. Duodenal ulcers rarely undergo malignant transformation so do not require a compulsory biopsy but gastric ulcers require biopsies to rule this out. In patients who are 55 or younger without ‘red flags’, testing for Helicobacter pylori (breath testing with radiolabelled urea or stool antigen testing) is necessary. Management is aimed at correcting the underlying cause such as discontinuing NSAIDs. H. pylori eradication should be started if the organism is present with triple therapy. Otherwise, a PPI is indicated.

Answer 643

H. Laryngitis - Laryngitis, as the name would suggest, is inflammation of the larynx, which can lead to oedema of the true vocal folds. It has both infectious and noninfectious causes such as vocal strain. Symptoms of acute disease are most commonly hoarseness, generally over a period of less than a week, usually preceded by viral URTI and usually self limiting. The pain on swallowing and sore throat is common of URTIs. An exudate or cervical lymphadenitis would suggest bacterial infection instead. Treatment begins, as always, with ABC and airway assessment. Chronic laryngitis presents with hoarseness lasting more than 3 weeks and this needs investigating due to the fact that symptoms may be similar to cancer of larynx. Antibiotics are given in bacterial cases or otherwise voice rest and hydration is sufficient.

Answer 644

E. Angioedema - Angio-oedema is swelling involving the deeper layers of the subdermis (occuring in association with urticaria in around 40% of cases). In this case it involves the face/neck and is dangerous – the risk being airway compromise and this requires rapid treatment with adrenaline. The food trigger here is obvious – this person has just consumed peanuts and this is allergic in nature, causing an IgE mediated reaction. Common triggers aside from nuts include eggs and shellfish although any food can be implicated. As mentioned, airway management and adrenaline is crucial here and you would also give antihistamines, IV corticosteroids and tell the patient to avoid the trigger.

Answer 645

A. Laryngeal nerve palsy - This patient has just had neck surgery and the hoarseness here results from damage to the recurrent laryngeal nerve, which is a branch of the vagus nerve which supplies motor function and sensation to the larynx. This nerve runs posterior to the thyroid and results in hoarseness when damaged. Bilateral damage is even worse and the patient could have difficulty breathing and the complete inability to speak. The right recurrent laryngeal is more prone to damage as it is located relatively more medial than the left.

Answer 646

G. Carcinoma of the larynx - Laryngeal cancer is frequently associated with two big risk factors: smoking and alcohol use. Major signs are persistent (>3 weeks) of hoarseness, dysphonia, sore throat, dysphagia, referred otalgia (seen here) and neck masses/adenopathy. These patients need to be evaluated by a specialist and as mentioned this can resemble chronic laryngitis in presentation. An MDT approach is taken to try and preserve the organ as much as possible, with salvage surgical resection offered in advanced disease.

Answer 647

B. Hypothyroidism - This woman is in a hypothyroid state. The hoarseness may be a symptom of a goitre (enlarged thyroid). Worldwide, the most common cause is iodine deficiency. Other causes include Hashimoto’s or secondary and tertiary hypothyroidism. It can also result from viral de Quervain’s thyroiditis or postpartum thyroiditis. Symptoms include those mentioned as well as depression, bradycardia, sluggish reflexes, cold intolerance and muscle cramps. Diagnosis is based on measurement of TSH and thyroid hormones. Treatment is by replacement of T4 with or without T3 in combination. If the patient has normal T3 and T4 but mildly elevated TSH, this is described as subclinical hypothyroidism.

Answer 648

L. Endomysial antibodies - This is a common condition in the US and Europe. Coeliac disease most commonly presents with IDA, although it can also lead to a macrocytic anaemia with mainly folate deficiency (though B12 is also affected but hepatic stores last several years). The mouth ulcers are a sign of this. There are also GI symptoms resulting from malabsorption. It is an autoimmune condition (the presence of another autoimmune condition here is a risk factor) triggered by gluten peptides found in wheat, rye and barley. The ultimate best test is duodenal biopsy and histology to show intra-epithelial lymphocytes, villous atrophy and crypt hyperplasia. Macroscopic changes may be present but endoscopy is generally unhelpful. The test of choice before performing such an invasive confirmatory test is to look for elevated anti-gliadin antibodies. Anti-tissue transglutaminase is less accurate and endomysial antibody is more expensive and has lower sensitivity, though is the only option on this list specific for coeliac. It is worth knowing about the Schilling test as it is frequently examined. However, it is no longer routinely done in clinical practice. In this test, IM vitamin B12 is given to saturate stores. Then oral radiolabelled B12 is given and urine is collected over 24 hours. The amount excreted is lower in B12 malabsorption. If this is not corrected by IF the problem is with the ileum and not inadequate IF.

Answer 649

K. HIV test - Molluscum contagiosum is an umbilicated, pearl like, smooth papules and at least a third of patients will also develop symptoms of local erythema, swelling or pruritis. HIV is a strong risk factor for MC. It is transmitted skin-to-skin or fomite in children and sexually transmitted in adulthood. Oral candidiasis is also an opportunistic infection associated with the immunocompromised state of HIV. Weight loss is also seen in HIV and may result from malnutrition, TB or HIV wasting syndrome. Diarrhoea is also common. An HIV test is needed in this patient and contact tracing will be necessary.

Answer 650

B. Sweat test - CF is autosomal recessive and the mean age of death is around 40. There is currently no cure for this condition. The reccurent chest infections and greasy stools (fat malabsorption due to pancreatic insufficiency) should make you think of CF. A persistent cough which is productive would also raise suspicions. Additionally, you may find nasal polyps and hepatomegaly and/or splenomegaly and a congenital absence of the vas deferens in males. There may also be failure to thrive in infants. The most conclusive diagnostic test is the sweat test which is positive if sweat chloride is >60mmol/L. Serum IRT from a heel prict blood spot allows screening of newborns. CF is a genetic condition with abnormal salt and water transport due to mutations in the CFTR (an apical anion channel). Heterozygotes generally do not demonstrate disease.

Answer 651

D. ERCP - This is chronic pancreatitis which is most commonly associated with chronic alcohol abuse. Features include the epigastric pain here, which classically radiates to the back, and steatorrhoea from malabsorption (pale, foul-smelling and difficult to flush stools). There may additionally be DM due to pancreatic failure and the patient may be malnourished. The diagnosis is based on findings and imaging – your options are USS which is less sensitive, or CT, which is more sensitive but involves radiation exposure. AXR is not a sensitive enough test. However, this question is looking for the best test which is ERCP, commonly considered the most accurate test with high sensitivity and specificity. It is limited in use though due to cost and the risk to the patient. Characteristically ERCP would show beading of the main pancreatic duct as well as irregularities in the side branches. Faecal elastase-1 is inaccurate for diagnosing mild to moderate pancreatic insufficiency, and anyway has unacceptably low sensitivity. There is no real definitive treatment, which is mainly symptomatic and the underlying and precipitating factors are treated – in this case, this man’s alcohol excess. Complications of chronic pancreatic imflammation include the development of pseudocysts, calficiation, DM and malabsorption.

Answer 652

H. Colonoscopy - This is a history of inflammatory bowel disease. A colonoscopy is required to assess the extent of disease and for a definitive diagnosis. Biopsy in CD will show transmural granulomatous inflammation. CD can affect the whole GIT but favours the TI (RLQ pain) and proximal colon and is macroscopically characterised by skip lesions. UC on the other hand is characterised by the presence of crypt abscesses, which is pathognomic.

Answer 653

H. Primary biliary cirrhosis - Primary biliary cirrhosis (PBC) is a chronic condition where the intrahepatic small bile ducts are progressively damaged (and eventually lost) occuring on a background of portal tract inflammation. Fibrosis develops, ultimately leading to cirrhosis (which is defined as fibrosis with nodular regeneration). It is widely believed to be autoimmune in aetiology as almost all patients have AMA (present here). The pointers in this question which would raise your suspicion, is xanthelasma around the eyes, pruritis in the absence of an obvious dermatological cause, fatigue and the features of liver disease typical of cirrhosis (obstructive jaundice) and splenomegaly as a feature of portal hypertension.

Answer 654

B. Gilbert's syndrome - Gilbert’s occurs in an asymptomatic patient, often as an incidental finding or mild jaundice occuring in adolescence/young adult age. There is elevated unconjugated BR with other liver tests being normal. The blood smear is also normal with normal reticulocyte count, and normal Hb indicating that this is not due to haemolysis. It is a common syndrome and is not really a disease, more a physiological variant. No treatment is needed and this condition is due to decreased UDPGT activity leading to decreased conjugation of unconjugated bilirubin, leading to elevated levels. Positive FH is common as this condition is most likely transmitted in an autosomal recessive pattern.

Answer 655

C. Cancer of the pancreas - Pancreatic cancer (of the head) typically presents with painless obstructive jaundice and weight loss and generally presents late. There is however epigastric pain in this case, which is a possible presentation. Whipple’s procedure or Traverso-Longmire procedure (pancreaticoduodenectomy) offers the only hope of a cure but only a small minority are elegible for these procedures. The first tests to order are an abdominal USS and LFTs. Note Courvoisier’s law: Jaundice and a palpable painless gallbladder is unlikely to be caused by gallstones. The tumour marker for pancreatic cancer is CA19-9 which is useful in preoperative staging.

Answer 656

F. Hepatitis - This is likely hepatitis A which is primarily transmitted via the faecal-oral route. After the virus is consumed and absorbed, it replicates in the liver and is excreted in the bile (to be re-transmitted). Transmission usually precedes symptoms by about 2 weeks and patients are non-infectious 1 week after onset of jaundice. The history can reveal risk factors such as living in an endemic area, contact with an infected person, homosexual sex or a known food-borne outbreak. This is classically, in EMQs, associated with shellfish which is harvested from sewage contaminated water. If the patient has other liver diseases such as HBV or HCV or cirrhosis then there is a higher risk of fulminant HAV infection. The clinical course of HAV consists of a pre-icteric phase, lasting 5-7 days, consisting characteristically of N&V, abdominal pain, fever, malaise and headache. Rarer symptoms may be present such as arthralgias and even severe thrombocytopenia and signs that may be found include splenomegaly, RUQ tenderness and tender hepatomegaly as well as bradycardia. The icteric phase is characterised by dark urine, pale stools, jaundice and pruritis. When jaundice comes on, the pre-icteric phase symptoms usually diminish, and jaundice typically peaks at 2 weeks. However, a fulminant course runs in

Answer 657

G. Haemolytic anaemia - Africans have higher incidence of sickle cell anaemia. This is a presentation of bone pain here with dactylitis, consistent with hand-foot syndrome which can be what young infants and children present with (it is often a child’s first presentation of disease). The jaundice here is due to haemolysis and so while this is sickle cell anaemia, the options are trying to get you to think a bit about the best fit here which would be haemolytic anaemia. About 8% of black people carry the gene and the prevalence is high in sub-Saharan Africa. The condition is autosomal recessive and therefore occurs in 1 in 4 pregnancies where both parents carry the sickle gene. Sickling occurs when RBCs containing HbS become distorted into a crescent shape. Patients with sickle cell anaemia have no HbA at all. If both parents carry the sickle cell gene, there is a 1 in 4 chance of giving birth to a child with sickle cell anaemia. Sickle cell disease also includes other conditions such as HbS from one parent with another abnormal Hb or beta thalassaemia from the other parent such as HbS-Beta thal and HbSC. Treatment goals here include fluid replacement therapy, pain management and symptomatic control.

Answer 658

B. Extensive necrosis - Paracetamol OD can occur after a single large OD or repeated ODs. Often, the patient is asymptomatic at initial presentation but if untreated may cause liver injury over the 2-4 days after ingestion, including fulminant liver failure. Massive hepatic necrosis will occur and will be the cause of fulminant liver failure. Paracetamol is the most frequent intentional OD drug in this country. The risk of liver damage is increased after taking drugs which induce CYP 450. Inducers include St John’s wort, barbiturates, phenytoin, tetracycline, chronic alcohol use and carbamazepine. A serum paracetamol level is important to order as early as possible, but at the earliest 4 hours post-ingestion.Treatment if indicated is with N-acetylcysteine with the level based on a paracetamol treatment graph.

Answer 659

L. Hepatocellular carcinoma - Patients with cirrhosis, especially those with alcoholic liver disease, are at a high risk of developing HCC. Cirrhosis is irreversible so despite stopping drinking, he is still at risk of HCC (hepatoma). Patients with cirrhosis should be screened for HCC with serum AFP and USS at 6 month intervals.

Answer 660

E. Cholecystitis - Cholecystitis is acute GB inflammation caused by an obstruction at the cystic duct. It occurs as a major complication of gallstones and classically presents with RUQ pain and fever. Gallstones in EMQs classically involves the Fs (Fat, Forty, Female, Fertile, Fair). USS is the definitive initial investigation. HIDA scanning and MRI may help if the diagnosis remains unclear. Treatment is with cholecystectomy.

Answer 661

B. Extensive necrosis - This is a case of haematemesis secondary to oesophageal varices. Oesophageal varices are a direct result of portal hypertension, which occurs as a progressive complication of cirrhosis, which is what liver biopsy will show. Diagnosis and surveillance by endoscopy is an important part of management of this condition and in terms of prophylaxis against variceal bleeding before it has occured, non-selective beta blockers and/or endoscopic ligation can be used.

Answer 662

A. Hepatitis B - Hepatitis B is a DNA virus which is transmitted percutaneously and permucosally. It is also a STI. HCV is an RNA virus and RNA-PCR will be positive. A brief bit about hepatitis B markers: HBsAb appears several weeks after HBsAg disappears and in most patients suggests a resolved infection and life-long immunity (it is also detectable and titres are measured in those immunised with the HBV vaccine). HBsAg on the other hand appears 2-10 weeks after exposure to HBV and usually, in self-limiting acute cases, becomes undetectable after 4-6 months of infection. Persistence for >6 months implies chronic infection. Core antibody (IgM) appears within weeks of acute infection and remains detectable for 4-8 months and can be the only way to diagnose acute infection during the period when surface antigen disappears but before surface antibody has appeared. Chronic infection is indicated by IgG core antibody. The best single test to screen household contacts of infected individuals to determine the need to vaccinate is still HBcAb. E antigen is a soluble viral protein in serum which is part of the early acute infection and disappears soon after peak ALT levels. Presence >3 months indicates chronic infection is likely. E antigen being present in those with surface antigen indicates greater infectivity and a high level of viral activity and replication.

Answer 663

``` D. Activated charcoal - This is an overdose of tricyclic antidepressants which are a class of drugs with a narrow therapeutic index and therefore become potent toxins in moderate doses to both the CNS and cardiovascular system. The main aim in treatment is to provide respiratory and cardiovascular support until the medicine has been fully metabolised and eliminated. GI decontamination should be considered in those presenting with early overdose (under 2 hours after ingestion) provided that the airway can be protected. There is no shown clear benefit to repeated doses of activated charcoal. The warm, dry skin is part of the anticholinergic effects (physostigmine should NOT be used to reverse this as it has been in rare cases been associated with asystole – would you rather have a patient who is flushed or flatlined?). Other anticholinergic effects include dilated pupils, urinary retention, decreased or absent bowel sounds and changes in mental status. Hypotension is common and is due to alpha 1 antagonism. Classic ECG changes are of sinus tachycardia progressing to wide complex tachycardia and ventricular arrhythmias (with increasing severity and intoxication). Condution problems and hypotension is improved with hypertonic sodium bicarbonate and if arrhythmias are present, treatment of these involves correcting the acidosis, hypoxia and electrolyte abnormalities. Anti-arrhythmics are generally avoided. If hypotension is refractory then a vasopressor can be used. BZDs can be used for any seizures. ```

Answer 664

K. N-acetylcysteine - Paracetamol OD can occur after a single large OD or repeated ODs. Often, the patient is asymptomatic at initial presentation but if untreated may cause liver injury over the 2-4 days after ingestion, including fulminant liver failure. Paracetamol is the most frequent intentional OD drug in this country. The risk of liver damage is increased after taking drugs which induce CYP 450. Inducers include St John’s wort, barbiturates, phenytoin, tetracycline, chronic alcohol use and carbamazepine. A serum paracetamol level is important to order as early as possible, but at the earliest 4 hours post-ingestion.Treatment if indicated is with N-acetylcysteine with the level based on a paracetamol treatment graph.

Answer 665

E. IV nalaxone - Signs of opiate OD include CNS depression, miosis (pinpoint pupils) and apnoea. Naloxone is indicated both therapeutically and diagnostically. If there is a response, then it is diagnostic. Another diagnosis should be sought if the patient is unresponsive. IV is the preferred route of administration although naloxone can be given IM or SC if IV access cannot be established. Ventilatory support is key with 100% oxygen.

Answer 666

J. IV glucagon - Glucagon stimulates adenyl cyclase which acts to increase intracellular cAMP and to therefore increase cytosolic calcium and cardiac contractility. Hypotension and bradyarrhythmias are the most common initial findings of beta blocker toxicity. If IV glucogon is not available then high-dose insulin can be used instead with co-administration of dextrose to maintain blood glucose levels.

Answer 667

I. IV ethanol - Antifreeze is ethylene glycol. It is a sweet-tasting, odourless and colourless liquid and the substance itself is non-toxic and initially causes inebriation. Toxicity appears within 12-24 hours and is due to metabolic acidosis and the formation of calcium oxalate from one of the metabolites. Oxalate deposits in the lungs, myocardium and kidneys leading to organ damage and renal failure, and hypocalcaemia may also occur due to the consumption of circulating calcium. Ethylene glycol is not absorbed by activated charcoal and gastric decontamination is pointless regardless of time since consumption. The first line treatment is fomepizole (4-methylpyrazole) which is a competitive inhibitor of alcohol dehydrogenase, an enzyme involved in catalysing the initial steps in metabolism of ethylene glycol and methanol into toxic metabolites. However, this is not on the list, and oral ethanol (loading dose or infusion) can be used in this case (have a think about why ethanol would work as an antidote, if you think back to how ethanol is metabolised). Dialysis may well be required. Other specific antidotes to be aware of (check out TOXBASE if interested): • Calcium channel blocker OD can be treated with calcium chloride. Glucagon can be tried if calcium replacement alone is insufficient. • Sodium channel blocker toxicity can be treated with sodium bicarbonate depending on the QRS length. • Salicylate or phenobarbital OD can be treated with urine alkalinisation with IV sodium bicarbonate. Phenobarbital responds well to multidose charcoal. • Cholinesterase toxicity can be treated with IV atropine if there is symptomatic bradycardia. Severe cases can benefit also from IV pralidoxime. • Cyanide toxicity can be treated with hydroxocobalamin. • Warfarin toxicity can be treated with FFP/PCC and vitamin K. • Digoxin toxicity is treated with digoxin-specific antibody fragments (digoxin immune Fab). • Sulphonylurea OD can be treated with glucose infusions in combination with IV octreotide if hypoglycaemia is problematic. • Heavy metal toxicity can be treated with the appropriate chelating agent.

Answer 668

D. Diagnostic laprotomy - An abdominal and pelvic CT scan would normally be ordered in situations like this, with possible appendicitis, but it is assumed here that the doctors were thinking of pregnancy as a possibility so a sonogram was done instead, which turns out to be inconclusive. Now, in this situation, you could do an abdominal MRI (especially in early pregnancy) or go ahead with a CT scan anyway, but a diagnostic laparotomy is the best option here to diagnose and treat at the same time. The main differential here is either obstetric, such as a ruptured ectopic pregnancy, or acute appendicitis. You would have imagined they would have done the usual important tests like FBC and a urinary pregnancy test... but these results are not available, nor are they an option, but a prudent doctor would have ordered them in the diagnostic work up. However, given USS does not show a mass in the fallopian tubes, this may push you away from an ectopic pregnancy... however, USS is operator dependent, this is a female of childbearing age (this age is getting lower and lower in the UK) and the doctor has failed to obtain either serum or urine HCG levels or asked about any missed menstrual periods. This patient, should be made NBM with maintenance IV fluids like lactated Ringer’s, and have a laparotomy which can be both diagnostic and therapeutic. You can take the appendix out if this is the problem or deal with the ectopic, if that is the problem, or deal with whatever it could be.. say for instance, a rare Meckel’s diverticulitis.

Answer 669

C. CT scan - This patient has renal colic which classically presents with severe flank pain radiating to the groin. Infection may complicate renal calculi. Microscopic haematuria is present in up to 90% of cases. Up to 85% of stones are visible on a plain KUB although urate stones are radiolucent. If the stone is radio-opaque, calcification will be seen within the urinary tract. In pregnancy though, a renal USS is first line. The IVP has now been replaced by the CT scan which is the new diagnostic standard. A non-contrast helical (or spiral) CT is preferred due to high sensitivity and specificity and acurately determines presence, site and size of stones. Stones are analysed after they are extracted or when they are expelled to check their composition. In reality the first test to order is urinalysis – it is quick and simple. You’d also always exclude ectopic pregnancy with a urine pregnancy test if this were a female of childbearing age, look for hypercalcaemia and hyperuricaemia and perform a FBC to look for infection.

Answer 670

E. Oral antibiotics - This patient obviously has diverticulitis and does not need further investigation. Symptomatic diverticulitis presents with fever, high WCC and LLQ pain. Risk factors for diverticular disease include low dietary fibre and advanced age. Oral antibiotic therapy and analgesia is indicated. If there is no improvement in 72 hours after oral antibiotics then IV antibiotics are indicated. Make sure you understand the differences in the terms: diverticulosis, diverticulitis and diverticular disease.

Answer 671

A. Ultrasound scan - Abdominal ultrasound is ordered when a patient presents with biliary pain and is the single best test for cholelithiasis (though has a low sensitivity for choledocholithiasis). Note that cholelithiasis refers to stones in the gallbladder and choledocholithiasis refers to stones in the bile duct. If stones are found then this would give weight to a diagnosis of acute cholecystitis. There are symptoms here of obstructive jaundice due to gallstone obstruction of bile outflow. Serum amylase would also be ordered in any patient presenting with pain located in the epigastric region, to rule out acute pancreatitis. This has been done here. In this patient you would also order LFTs, FBC looking for evidence of inflammation. MRCP, ERCP and EUS can be considered if necessary.

Answer 672

N. IV naloxone - Signs of opiate OD include CNS depression, miosis (pinpoint pupils) and apnoea. Naloxone is indicated both therapeutically and diagnostically. If there is a response, then it is diagnostic. Another diagnosis should be sought if the patient is unresponsive. IV is the preferred route of administration although naloxone can be given IM or SC if IV access cannot be established. Ventilatory support is key with 100% oxygen.

Answer 673

E. IV lorazepam - This is a tonic-clonic, generalised seizure. It is characterised by LOC and widespread motor tonic contractions followed by clonic jerking movements. There will characteristically be a suppressed level of arousal following the event. This may either reflect a primary generalised episode or a focal seizure with secondary generalisation. The main aim of acute treatment is to terminate the seizure and to protect the airway. Management always starts with basic life-support (like every acute emergency) and your ABCs. IV access needs to be established (bloods sent to the lab too and serum glucose measured to test for reversable causes of seizure activity – thiamine should also be given to the patient if there is any concern about deficiency and hypoglycaemia, for instance in alcohol abuse). The following are needed: ECG, pulse oximetry, ABG. IV lorazepam is the preferred initial therapy, though rectal diazepam can be used if there is no IV access. If BZDs fail to stop the seizure then phenytoin or fosphenytoin can be tried. After the episode, MRI and EEG are essential in diagnosing an epilepsy syndrome. During the episode of generalised tonic-clonic activity, the EEG will show bilateral synchrony in the epileptiform activity. If this is a one-off seizure in which a provoking factor, such as electrolyte disturbance or hypoglycaemia, has been identified then there is no need for therapy for epilepsy. In unprovoked cases, this depends on history, examination, EEG and MRI. Treatment may not be needed the first time but after a second unprovoked instance, therapy is generally recommended.

Answer 674

J. Elevate legs - This is a case of vasovagal syncope. Elevation of the legs will help move blood towards the head. The other options on the list are not necessary.

Answer 675

E. IV lorazepam - This patient is in status epilepticus defined by 30 minutes or more of continuous seizure activity, or repetitive seizures with no intervening recovery of consciousness. SE can be either generalised convulsive or non-convulsive (simple or complex partial). In children, seizures of a shorter duration may also be considered to be status epilepticus. The initial treatment is as 2) and should start with BLS measures, continuous monitoring and benzodiazepines as first line therapy – with IV lorazepam. Securing the patient’s airwar may prove difficult due to the convulsions and neuromuscular blockade with a short-acting drug such as vecuronium may be necessary. Unresponsive cases can get anticonvulsants or phenobarbitone. It is worth noting that phenytoin infusions may lead to venous irritation and tissue damage if the undiluted drug is given through a small bore cannula. If SE persists, the next step to take is to intubate and start general anaesthesia. The best initial agents to use are midazolam and propofol though other options include pentobarbital and thiopental, the former being an active metabolite of the latter. GA should be tapered off after a minimum of 12 hours, and if the seziure recurs then the infusion should be restarted for another 12-24 hours.

Answer 676

M. IM benzylpenicillin - This is suspected bacterial meningitis and in the community setting the patient should receive immediate IM benzylpenicillin before being transferred to hospital as an emergency case.

Answer 677

F. X-linked recessive Here is a list of the important conditions you should be aware of: Autosomal dominant: Achrondroplasia, APKD, dystrophia myotonica, familial hypercholesterolaemia, Huntington’s, Marfan’s, NF, tuberous sclerosis, osteogenesis imperfecta Autosomal recessive: CF, hereditary haemochromatosis, sickle cell disease, Wilson’s, Friedreich’s ataxia, CAH X-linked recessive: DMD, Fragile X syndrome, Haemophilia A and B, G6PDH deficiency, Alport’s You should be aware that Down’s is trisomy 21, Edwards’ is trisomy 18 and Patau’s is trisomy 13.

Answer 678

C. Autosomal recessive - Here is a list of the important conditions you should be aware of: Autosomal dominant: Achrondroplasia, APKD, dystrophia myotonica, familial hypercholesterolaemia, Huntington’s, Marfan’s, NF, tuberous sclerosis, osteogenesis imperfecta Autosomal recessive: CF, hereditary haemochromatosis, sickle cell disease, Wilson’s, Friedreich’s ataxia, CAH X-linked recessive: DMD, Fragile X syndrome, Haemophilia A and B, G6PDH deficiency, Alport’s You should be aware that Down’s is trisomy 21, Edwards’ is trisomy 18 and Patau’s is trisomy 13.

Answer 679

C. Autosomal dominant- Autosomal dominant: Achrondroplasia, APKD, dystrophia myotonica, familial hypercholesterolaemia, Huntington’s, Marfan’s, NF, tuberous sclerosis, osteogenesis imperfecta Autosomal recessive: CF, hereditary haemochromatosis, sickle cell disease, Wilson’s, Friedreich’s ataxia, CAH X-linked recessive: DMD, Fragile X syndrome, Haemophilia A and B, G6PDH deficiency, Alport’s You should be aware that Down’s is trisomy 21, Edwards’ is trisomy 18 and Patau’s is trisomy 13.

Answer 680

E. Chromosomal abnormality - Here is a list of the important conditions you should be aware of: Autosomal dominant: Achrondroplasia, APKD, dystrophia myotonica, familial hypercholesterolaemia, Huntington’s, Marfan’s, NF, tuberous sclerosis, osteogenesis imperfecta Autosomal recessive: CF, hereditary haemochromatosis, sickle cell disease, Wilson’s, Friedreich’s ataxia, CAH X-linked recessive: DMD, Fragile X syndrome, Haemophilia A and B, G6PDH deficiency, Alport’s You should be aware that Down’s is trisomy 21, Edwards’ is trisomy 18 and Patau’s is trisomy 13.

Answer 681

A. None/unknown - Here is a list of the important conditions you should be aware of: Autosomal dominant: Achrondroplasia, APKD, dystrophia myotonica, familial hypercholesterolaemia, Huntington’s, Marfan’s, NF, tuberous sclerosis, osteogenesis imperfecta Autosomal recessive: CF, hereditary haemochromatosis, sickle cell disease, Wilson’s, Friedreich’s ataxia, CAH X-linked recessive: DMD, Fragile X syndrome, Haemophilia A and B, G6PDH deficiency, Alport’s You should be aware that Down’s is trisomy 21, Edwards’ is trisomy 18 and Patau’s is trisomy 13.

Answer 682

K. Acute urinary retention - This is acute urinary retention caused by anticholinergics. Unwanted effects of this class of drugs include urinary retention, constipation, erectile dysfunction, CNS disturbance, cycloplegia, dry mouth and decreased sweating. Poisoning in severe cases can be treated with anticholinesterases such as physostigmine. This man will need to be catheterised first to relieve the urinary retention and prevent any further complications from occuring.

Answer 683

C. Labour - Abdominal pain throughout pregnancy is common although reproductive organs share the name visceral innvervations as the lower ileum, sigmoid colon and rectum so it may be difficult to differentiate GI pain from gynaecological pain. This acute abdomen would require some consideration as the clinical picture may be distorted by the fact that pregnancy stretches the anterior abdominal wall so peritoneal signs are often different due to a lack of contact with the underlying inflammation, if an inflammatory process were to be present. The case described here could well be labour and the already overweight woman has simply been unaware of the fact she is pregnant (which although uncommon, can occur). The gross abdominal distension (which she may not attribute to anything abnormal if she is already fat) and symptoms are pointing to labour and there may also be uterine contractions. Blood or mucus on vaginal examination may be seen and you would expect the cervix to be soft, effaced and possibly dilated. However, this could be another complication of pregnancy or even a non-gynaecological condition and these need to also be considered during the diagnostic work-up. An ultrasound scan, which is safe to use in pregnancy, should be done here.

Answer 684

D. Bladder cancer - Gross haematuria is the primary symptom of bladder cancer. Over 80% present with haematuria which is the primary presenting complaint in this condition. The gross haematuria is classically painless and present throughout the entire urinary stream, however while painful the rest of the symptoms fit. It is also worth noting that carcinoma in situ commonly presents with dysuria and frequency and can easily be confused with prostatitis. Risk factors include smoking, exposure to carcinogens such as the aromatic amines used in rubber and dye industries, age >55, pelvic radiation and Schistosomiasis resulting in SCC (related to chronic inflammation – so other risks also include UTI, stones etc). Bladder cancer is the most common cancer in Egypt, for the latter reason. Cystoscopy and urinary cytology are key in diagnosis. Low grade tumours are papillary and easy to see on cystoscopy whereas high grade tumours and carcinoma in situ are often difficult to visualise. Resection provides diagnosis and primary treatment in one step.

Answer 685

F. Urinary tract infection - This young woman has presented with a UTI (dysuria, frequency, and foul smelling urine). Sexual activity the strongest risk factor for UTIs in women. It is diagnosed with a urine dipstick and microscopic analysis for bacteria, WBC and RBC and urine culture and antibiotic sensitivities. Antibiotic selection should be based on local guidelines or known sensitivities. Uncomplicated UTIs with no known antibiotic resistance can be treated with co-trimoxazole or nitrofurantoin. If there is resistance, a quinolone can be considered such as ciprofloxacin.

Answer 686

E. Urinary tract stones: ureteric colic/stricture - This patient has renal colic which classically presents with severe flank pain radiating to the groin. Microscopic haematuria is present in up to 90% of cases. Up to 85% of stones are visible on a plain KUB although urate stones are radiolucent. If the stone is radio-opaque, calcification will be seen within the urinary tract. In pregnancy, a renal USS is first line. The IVP has now been replaced by the CT scan which is the new diagnostic standard otherwise. A non-contrast helical (or spiral) CT is preferred due to high sensitivity and specificity and acurately determines presence, site and size of stones. Stones are analysed after they are extracted or when they are expelled to check their composition. It is worth noting that in all females of child bearing age, a urine pregnancy test is necessary to exclude an ectopic pregnancy.

Answer 687

A. Endometriosis - Endometriosis is a chronic inflammatory condition defined by endometrial stroma and glands located outside of the uterine cavity – the most common sites being the pelvic peritoneum and ovaries. It may present as an incidental finding in asymptomatic patients but more commonly it presents in women of reproductive age with chronic pelvic pain and/or subfertility. This woman has symptoms which make this diagnosis likely. There is dyspareunia which is pain during sexual intercourse, particularly on deep penetration, and may be caused by a distortion in the pelvic anatomy and rectovaginal involvement. There is also well documented genetic predisposition so a positive FH may be found. Additionally, nulliparous women are more likely to be diagnosed with endometriosis than parous women. The diagnosis can be confirmed on visualising the ectopic tissue directly and focused biopsies during laparoscopy but this is not generally necessary as clinical suspicion is enough to start treatment. Options for treatment include NSAIDs, COCPs, GnRH agonists, danazol or related androgens and surgical destruction of lesions. Those who present with subfertility may be considered for ovarian hyperstimulation and IVF.

Answer 688

F. Endometrial cancer - This is a common malignancy and is usually an adenocarcinoma. Obesity is associated with an increased incidence of endometrial cancer and also poorer outcome. Risk factors to consider aside from obesity include HRT, tamoxifen use, age over 50, unopposed oestrogen and radiotherapy. FH and a history of other cancers are also risks. A good history is important to establish that PV bleeding does not have another obvious cause such as intercourse or that associated with HRT, and that is is unlikely to be related to another malignancy like cervical cancer. Examination tends to be challenging due to the prevalence of obesity in those with endometrial cancer. The presentation is typically with post-menopausal bleeding PV and often the disease is surgically curable. Diagnosis will need to be confirmed by biopsy and histology with histopathology showing adenocarcinoma.

Answer 689

G. Adenomyosis - Adenomyosis, as you can probably figure out from the name, is the presence of ectopic glandular tissue in muscle. The first test to order is a pelvic USS which shows a normal or enlarged uterus. It is of minimal diagnostic value, especially if the clinical history and examination findings are suggestive, but can be used to rule out endometriosis, where the USS may show the presence of ovarian endometriomas. Examination may reveal an enlarged globular uterus and uterine tenderness on palpation, particularly during the menses. The history is usually of a parous woman, symptoms commonly occuring after childbirth, with heavy menstrual flow or an abnormal bleeding pattern being seen. Adenomyosis is a condition which can be diagnosed clinically, although if a costly MRI pelvis is done, then abnormal signal intensities within the myometrium can be seen.

Answer 690

D. Ectopic pregnancy - Ectopic pregnancy usually presents between 6-8 weeks after the last normal menstrual period but it can present later on. The risk increases if the woman has had a previous ectopic, surgery on the tubes, genital infections, smokes or uses an IUD. The classic symptoms and signs are pain, vaginal bleeding and amenorrhoea. If the patient is haemodynamically unstable or there is cervical motion tenderness, this may indicate that a rupture has occured or is imminent. Rupture, which is a complication, can present with shock from blood loss and with unusual patterns of referred pain from the presence of intraperitoneal blood. The positive urine pregnancy test here confirms pregnancy. Once the patient is confirmed to be pregnant, a transvaginal USS is used to determine the location of the pregnancy. If an intrauterine gestation is visible on USS regardless of whether it is viable, then the chances of having an ectopic pregnancy are incredibly low. Occasionally, an ectopic pregnancy itself can be seen, either as a ‘doughnut sign’ (adnexal mass separate from two clearly seen ovaries) or ‘ring of fire’ (increased blood flood to the ectopic seen on colour Doppler). A transabdominal ultrasound is less sensitive than a TVUS. Treatment approaches can include expactant, medical (methotrexate) or surgical (salpingectomy, salpingostomy).

Answer 691

C. Pelvic inflammatory disease - Pelvic inflammatory disease is an acute ascending infection of the female tract that is often associated with Neisseria gonorrhoeae or Chlamydia trachomatis. Key risk factors include prior infection with chlamydia or gonorrhoea or PID, young age of onset of sexual activity, unprotected sex with multiple partners and IUD use. Signs and symptoms vary and can include tenderness of the lower abdomen, adnexal tenderness and cervical motion tenderness. Fever and cervical or vaginal discharge may also be present. Complications include tubo-ovarian abscess and subsequent infertility or ectopic pregnancy due to scarred or obstructed fallopian tubes.

Answer 692

B. Caecal carcinoma - The weight loss, fatigue and RIF discomfort point to caecal carcinoma. Right sided colorectal cancer tends to present with anaemic symptoms. Almost 90% are anaemic at diagnosis.

Answer 693

D. Haemorrhoids - Haemorrhoids are vascular rich cushions in the anal canal and presents, typically, as painless bright PR bleeding or with sudden onset pain in the area associated with a palpable mass. Pruritus ani is common and there is often perianal pain or discomfort. Diagnosis is made visually. Grade 1 is limited to within the anal canal. Grade 2 protrudes but spontaneously reduces when the patient stops straining. Grade 3 protrudes and reduces fully on manual pressure. Grade 4 is irreducible. Treatment includes fibre, ligation, photocoagulation, sclerotherapy or surgical haemorrhoidectomy. Haemorrhoidectomy is the treatment of choice of choice for patients with grade 4 haemorrhoids or for any patient who has failed with more conservative treatment such as sclerotherapy.

Answer 694

G. Duodenal ulcer - There is no reason why this cannot be a gastric ulcer but it is not an option on the list. The history of dyspepsia and black tarry stools over the past few days suggests a bleeding peptic ulcer. The dyspepsia is often centred in the upper abdomen and is related to eating. Contrary to popular lay health beliefs there is no good evidence of alcohol as an important risk factor. Key risk factors are NSAID use, H. pylori, smoking and FH of PUD. The most specific and sensitive test is an upper GI endoscopy which will also allow intervention to stop the bleeding ulcer from bleeding. Duodenal ulcers rarely undergo malignant transformation so do not require a compulsory biopsy but gastric ulcers require biopsies to rule this out. Management is aimed at correcting the underlying cause such as discontinuing NSAIDs. H. pylori eradication should be started if the organism is present with triple therapy. Otherwise, a PPI is indicated.

Answer 695

J. Carcinoma of the rectum - This a rectal carcinoma. Tenesmus, blood PR alongside weight loss are all highly suggestive. Treatment of rectal carcinoma involves surgical excision where possible. This can either be an anterior resection (tumours in the upper 1/3 of the rectum) or an abdominoperineal resection (if the tumour lies lower down). APER involves the formation of a permanent colostomy and has a high incidence of sexual and urinary dysfunction. Anterior resection involves a colo-anal anastamosis.

Answer 696

H. Inflammatory bowel disease - This woman has inflammatory bowel disease, which by the history is probably more likely to be UC than CD, whereby the mainstay of treatment is with 5-ASA. A colonoscopy is required to assess the extent of disease and for a definitive diagnosis. Biopsy in CD will show transmural granulomatous inflammation. CD can affect the whole GIT but favours the TI and proximal colon and is macroscopically characterised by skip lesions. UC on the other hand is characterised by the presence of crypt abscesses, which is pathognomic. CD risk is increased 3-4 fold by smoking whereas smoking seems protective in UC. The mainstay of treatment in CD is with steroids and azathioprine to prevent relapses and for those suffering side effects of steroid treatment. TNF-alpha inhibitors also have a role. Surgery in CD is only indicated in a small number of patients who bleed, for bowel perforation and cases of complete obstruction. The aim is to rest distal disease by temporarily diverting faecal flow.

Answer 697

J. Addison's disease - Hyperpigmentation in the palmar creases points towards Addison’s disease. Hyperpigmentation due to excess ACTH production can be mucosal or cutaneous and is more pronounced in the palms, knuckles and around scars. MSH is a byproduct of the production of ACTH from the cleavage of POMC. Anorexia, fatigue and weight loss is observed in all patients. Sodium is low and potassium elevated. Vomiting is present in 75% of patients and nausea is a common finding. Additionally, postural hypotension may be present. The presence of other autoimmune diseases is a risk factor for the development of Addison’s. Diagnosis of Addison’s can be made on an ACTH stimulation test (synacthen test) whereby serum cortisol remains low despite the administration of synthetic ACTH. In an emergency, treatment should not be delayed by diagnostic testing.

Answer 698

A. Gladular fever - This is infectious mononucleosis or glandular fever and is caused by EBV. It is characterised by fever, pharyngitis and lymphadenopathy. Enlargement of the spleen begins in the first week and lasts 3-4 weeks, occuring in half of all cases. Risk factors for EBV transmission include kissing and sex. The fact her boyfriend has had similar symptoms should raise your suspicion. A FBC will show an atypical lymphocytosis. Confirmation of IM involves detection of the existence of heterophile antibodies using the Paul Bunnell monospot. A more accurate test is a serological test detecting EBV specific antibodies. Treatment is usually symptomatic but IM can carry rare but potentially life threatening complications.

Answer 699

H. Diabetes mellitus - Polyuria, polydipsia, nocturia in a boy who is tired all the time should make you think of T1DM. Insulin is needed alongside dietary changes and exercise. Insulin regimes aim to mimic physiological insulin release with a basal-bolus dosing. There is an option between using a pump and having multiple daily injections. It is worth noting that there is a high incidence of diabulimia among young people with T1DM who give themselves less insulin than they need in order to lose weight (they lose weight, ‘look good’ but trash their bodies).

Answer 700

F. AIDS - AIDS (acquired immunodeficiency syndrome) is caused by HIV, which is a retrovirus. To give you an indication of risk here, there is a risk of 50 infections per 10,000 exposed to an infected source in unprotected receptive anal intercourse. The risk with receptive vaginal intercourse is 10 infections per 10,000 exposures. Obviously, people have sex more often than the one off, as is human nature, so what seems like a small risk per sexual encounter adds up. IVDU needle sharing has a risk of 67 per 10,000, a needle-stick is 30 per 10,000 (equal to having receptive vaginal intercourse 3 times with an HIV positive man, so be careful on the wards, though this statistic does depend on factors like the size of the needle) and the risk associated with vertical transmission is associated with maternal viral load (the risk goes if you can suppress the viral load with anti-retrovirals). The thing to note is that the association with homosexuality is based on the increased risk of transmission from receptive anal compared to receptive vaginal, and if you happen to be a homosexual male and contract HIV, you’re unlikely to pass it on with vaginal intercourse. There should not be negative stigma attached to HIV. There are two types, HIV 1 which is the main virus responsible and HIV 2 which is restricted to parts of West Africa. Weight loss is common in HIV and if more than 10% body weight is lost or BMI reduces to 18.5, this is an indication of more severe immunocompromise. Weight loss in HIV may result from malnutrition, co-existent TB infection or HIV wasting syndrome, the latter being an AIDS defining illness. The purple lesions seen here are due to Kaposi’s sarcoma, which is a neoplasm derived from mesenchymal tissue, associated with HHV-8 infection. This is an AIDS defining infection. There are WHO (stage 1-4) and CDC criteria used in clinical staging of HIV. This patient needs to have a CD4 count, HBV and HCV screen, VDRL (syphilis), tuberculin skin test (TB) and CXR. HIV viral load will also be assessed. Prophylaxis and immunisations should be considered against infections such as hepatitis, influenza, PCP and TB. HAART needds to be initiated as he has developed AIDS. Classes of antiretrovirals include NRTIs, NNRTIs, protease inhibitors, fusion inhibitors and integrase inhibitors.

Answer 701

I. Hypothyroidism - There is weight gain, cold intolerance, fatigue and constipation as well as bradycardia which all point to hypothyroidism. Worldwide, the most common cause is iodine deficiency. Other causes include Hashimoto’s or secondary and tertiary hypothyroidism. It can also result from viral de Quervain’s thyroiditis or postpartum thyroiditis. Symptoms include those mentioned as well as depression, bradycardia, sluggish reflexes, constipation, cold intolerance and muscle cramps. Diagnosis is based on measurement of TSH and thyroid hormones. Treatment is by replacement of T4 with or without T3 in combination. If the patient has normal T3 and T4 but mildly elevated TSH, this is described as subclinical hypothyroidism.

Answer 702

M. Sickle cell anaemia - Africans have higher incidence of sickle cell anaemia. The jaundice here is due to haemolysis and accounts for the raised unconjugated bilirubin. The splenomegaly here is further suggestive. About 8% of black people carry the gene and the prevalence is high in sub-Saharan Africa. The condition is autosomal recessive and therefore occurs in 1 in 4 pregnancies where both parents carry the sickle gene. Sickling occurs when RBCs containing HbS become distorted into a crescent shape. Patients with sickle cell anaemia have no HbA at all. If both parents carry the sickle cell gene, there is a 1 in 4 chance of giving birth to a child with sickle cell anaemia. Sickle cell disease also includes other conditions such as HbS from one parent with another abnormal Hb or beta thalassaemia from the other parent such as HbS-Beta thal and HbSC. Treatment goals include fluid replacement therapy, pain management where necessary and symptomatic control.

Answer 703

E. Gilbert's syndrome - Gilbert’s occurs in an asymptomatic patient, often as an incidental finding or mild jaundice occuring in adolescence/young adult age, or during times of physiological stress (this patient has a cold). There is elevated unconjugated BR with other liver tests being normal. The blood smear is also normal with normal reticulocyte count, and normal Hb indicating that this is not due to haemolysis. It is a common syndrome and is not really a disease, more a physiological variant. No treatment is needed and this condition is due to decreased UDPGT activity leading to decreased conjugation of unconjugated bilirubin, leading to elevated levels. Positive FH is common as this condition is most likely transmitted in an autosomal recessive pattern.

Answer 704

H. Hepatitis A - IgM anti-hepatitis A virus is positive here which is highly sensitive and specific combined with the typical symptoms this student displays. IgM antibodies are detectable typically 5-10 days before symptom onset and remain raised for 4-6 months. It can be ordered alongside IgG anti-HAV and is a cheap and simple test. IgG rises soon after IgM and stays elevated for life so a positive IgG can mean prior infection or recent disease. Again, a cheap and simple to carry out test. Hepatitis A is primarily transmitted via the faecal-oral route. After the virus is consumed and absorbed, it replicates in the liver and is excreted in the bile (to be re-transmitted). Transmission usually precedes symptoms by about 2 weeks and patients are non-infectious 1 week after onset of jaundice. The history can reveal risk factors such as living in an endemic area, contact with an infected person, homosexual sex or a known food-borne outbreak. This is classically, in EMQs, associated with shellfish which is harvested from sewage contaminated water. If the patient has other liver diseases such as HBV or HCV or cirrhosis then there is a higher risk of fulminant HAV infection. The clinical course of HAV consists of a pre-icteric phase, lasting 5-7 days, consisting characteristically of N&V, abdominal pain, fever, malaise and headache. Rarer symptoms may be present such as arthralgias and even severe thrombocytopenia and signs that may be found include splenomegaly, RUQ tenderness and tender hepatomegaly as well as bradycardia. The icteric phase is characterised by dark urine, pale stools, jaundice and pruritis. When jaundice comes on, the pre-icteric phase symptoms usually diminish, and jaundice typically peaks at 2 weeks. However, a fulminant course runs in

Answer 705

J. Primary sclerosing cholangitis - Primary sclerosing cholangitis is a cholestatic liver disease which causes bile duct destruction, cirrhosis and end-stage liver disease. It predominantly affects young and middle-aged men, often with underlying inflammatory bowel disease. There is an association of PSC with UC (typically) and IBD diagnosis tends to precede that of PSC with a mean time from onset of IBD to PSC of 9 years. This patient has evidence of obstructive jaundice and pruritis. The diagnosis involves laboratory tests in combination with cholangiography. There are no specific auto-antibodies specific to or diagnostic of PSC but a number of serum autoantibodies such as ANCA are often present in PSC patients. There is currently no effectively medical therapy and the only treatment option in those with advanced disease is to have a liver transplant. The leading cause of death in these patients is liver failure and cholangiocarcinoma. The latter is a relatively common complication and should be suspected in patients who present with rapidly progressing jaundice, weight loss or abdominal pain on a background of PSC.

Answer 706

I. Carcinoma of head of the pancreas - Pancreatic cancer (of the head) typically presents with painless obstructive jaundice and weight loss and generally presents late. Whipple’s procedure or Traverso-Longmire procedure (pancreaticoduodenectomy) offers the only hope of a cure but only a small minority are elegible for these procedures. The first tests to order are an abdominal USS and LFTs. Note Courvoisier’s law: Jaundice and a palpable painless gallbladder is unlikely to be caused by gallstones. The tumour marker for pancreatic cancer is CA19-9 which is useful in preoperative staging.

Answer 707

G. Nebulised salbutamol - This patient is having an asthma attack. The best option here is nebulised salbutamol initially. Early systemic corticosteroids and supplemental oxygen should be considered and the patient’s status needs to be monitored regularly.

Answer 708

F. Oral clarithromyin - This is a CAP which is confirmed by CXR shadowing, which would show airspace shadowing with air bronchograms. The history is also consistent with respiratory symptoms and pyrexia. The most sensitive test for CAP is a CXR. The treatment is initially empirical with antibiotics and management would be guided by this patient’s CURB-65 score. A macrolide is the first choice in adults with no contra-indications without a recent cours eof antibiotics or risk of drug resistance, but always check local prescribing policies. Azithromycin, clarithromycin or erythromycin are all valid choices here.

Answer 709

J. IV cefuroxime - This is an acute infective exacerbation of COPD and the patient has already received nebulised salbutamol and corticosteroids. The next important thing to do is to give IV antibiotics. Inpatient therapy for severe infections can be with IV ceftriaxone with azithromycin. High risk patients should receive piperacillin and tazobactam, or meropenem, to cover for pseudomonas.

Answer 710

I. Diuretic - This patient has developed pulmonary oedema which accounts for the history and examination findings. The CXR indicates the cause is heart failure. He will require diuretics and fluid restriction to deal with his overloaded state. Patients need to be sat upright to improve the SOB and IV access needs to be established. Oxygen, morphine, diuretics (frusemide or another loop diuretic) and nitrates will be given. Once stable, medical treatment of heart failure should be started which involves in the first instance, an ACE inhibitor followed by beta blockade.

Answer 711

E. Opiates - Opiates are commonly prescribed for analgesia and can also be used as an anti-tussive in this case (suppresses ACh and NK release needed for cough activation and inteferes with serotonin receptors in the DRN). They are also abused for their euphoric effects mediated by their action on dopamine release at the nucleus accumbens. They have a host of side effects: GI effects (receptors present on the myenteric and submucosal plexus) of decreased gastric motility, emptying and increased gut water reabsorption... in short, constipation; Respiratory depression (desensitises central chemoreceptors to lessen the response to PaCO2); N&V by stimulating the CTZ; Pupillary constriction via the Edinger-Westphal nucleus; And causes symptoms like pruritis, urticaria, hypotension through histamine release via a direct effect on mast cells.

Answer 712

B. Methadone programme - This patient is a regular recreational opiate user and should be educated about the dangers, particularly of overdose. Needle sharing puts the patient at risk of infections such as HIV and HCV and advice should also be given on the use of clean needles or techniques on how to clean needles. This patient should be commended on wanting to take part in a detoxification programme for his chronic problem. Methadone is a drug which is used in opioid dependence as substitution therapy. Buprenorphine is another drug which can be used for this purpose and both are recommended in NICE guidelines. This medication can be started with a short period of stabilisation followed by either a withdrawal regimen or maintenance treatment, which reduces drug use and crime and improves health. Methadone is a long-acting opioid agonist, usually given as a single daily oral solution. It has a long half life and takes 3-10 days for plasma levels to reach a steady state. Buprenorphine is a partial opioid receptor agonist (i.e. it has both opioid agonist and antagonist properties) and some patients may prefer this drug as it is less sedating than methadone so may be more suitable for people who drive around, for instance. Adjunctive therapy may be needed to manage withdrawal symptoms. Loperamide can be used for diarrhoea, mebeverine for stomach cramps, paracetamol and NSAIDs for muscular pains and headaches, and metaclopramide or prochlorperazine for N&V. Lofexidine is an alpha-2 agonist which can be used to alleviate physical symptoms of opioid withdrawal. Remember that naloxone is an opioid-receptor antagonist used to reverse opioid overdose (those dependent can be given a supply in case they ‘accidentally’ overdose) and naltrexone is prescribed to prevent relapse in formerly dependent patients.

Answer 713

E. Disulfiram - Disulfiram is used as an adjunct in the treatment of alcohol dependence. It leads to an unpleasant systemic reaction after taking even the smallest amount of alcohol as it causes acetaldehyde to accumulate in the body. Disulfiram blocks the metabolism of alcohol. It is only effective if taken daily and symptoms can occur within 10 minutes of ingesting any alcohol and include facial flushing, throbbing headache, palpitations, tachycardia, N&V and possible arrhythmias, hypotension and collapse with large doses of alcohol. These reactions can last several hours. Even the smallest amounts of alcohol in medications and even mouthwash can lead to a reaction. After stopping treatment, alcohol still should be avoided for at least a week. Other medications that can be used to prevent relapse and support abstinence include acamprosate (which stabilises glutamate and GABA systems) and naltrexone which is an opioid antagonist.

Answer 714

D. Physician advice - This (likely medical) student is not dependent nor does he have any physical symptoms but is concerned and aware that his drinking is harmful. At this stage he needs advice on the dangers of excessive alcohol consumption which is the first line intervention for those with patterns of problematic alcohol use but are not dependent (as well as those with mild dependence). The consultations can consist of one or more sessions with a doctor during which feedback can be given about the patient’s alcohol use and the consequences in a supportive and empathic manner. The doctor and patient can work together to develop an action plan for cutting down or eliminating alcohol use and then assessing how effective this is with subsequent visits. Motivational interviewing is a technique which can also be deployed in this situation.

Answer 715

H. Inpatient detoxification with chlordiazepoxide - Chlordiazepoxide is a long-acting benzodiazepine which is used to attenuate alcohol withdrawal symptoms. People with severe dependence should undergo withdrawal in an inpatient setting – withdrawal in those who are severely dependent without medical support can lead to seizures, DT and death. A symptom triggered flexible regimen is typically used in hospital and continued assessment and monitoring is carried out for 24-48 hours, usually followed by a 5 day reducing dose schedule. When BZDs are contraindicated, carbamazepine can be used as an alternative. Clomethiazole can also be used in the acute withdrawal setting although BZDs are preferred. Patients with marked agitation or hallucinations, and those at risk of DT can be given antipsychotic drugs such as haloperidol as an adjunct. This patient should also be given parenteral thiamine (as Pabrinex) due to the risk of developing Wernicke’s encephalopathy from chronic alcohol abuse.

Answer 716

F. Alcoholics anonymous - Alcoholics Anonymous is a help group where people share their experiences and help each other recover from alcoholism. http://www.alcoholics-anonymous.org.uk

Answer 717

L. Anorexia nervosa - Anorexia nervosa often occurs in women which is a key risk factor. Puberty/adolescence and obsessive personality traits are additional risks. There is a higher incidence reported in western cultures (presumably skinny models on TV, adverts etc...) and studies on immigrants moving to a western culture exhibited a higher incidence. There is also postulated to be a genetic contribution from twin studies.Those who in higher socioeconomic classes are more affected. BMI is usually

Answer 718

G. Hyperthyroidism - TSH is the initial screening test and if supressed, T4/T3 levels are measured. Treatment aims to normalise thyroid function and is achieved by radioactive iodine, antithyroid medications or with surgery. They are all effective and relatively safe options. Symptomatic therapy is given with beta blockers such as propranolol.

Answer 719

B. HIV - HIV is a retrovirus and there are two types, HIV 1 which is the main virus responsible and HIV 2 which is restricted to parts of West Africa. Weight loss is common in HIV and if more than 10% body weight is lost of BMI reduces to 18.5, this is an indication of more severe immunocompromise. Weight loss in HIV may result from malnutrition, co-existent TB infection or HIV wasting syndrome, the latter being an AIDS defining illness. Generalised lymphadenopathy is also common and is characterised by the painless enlargement of 2 more more non-contiguous sites of >1cm for >3 months. Neutropenia is also seen due to CD4 deficiency and thrombocytopenia may also be seen along with an anaemic picture. There are WHO (stage 1-4) and CDC criteria used in clinical staging. This patient needs to have a CD4 count, HBV and HCV screen, VDRL (syphilis), tuberculin skin test (TB) and CXR. HIV viral load will also be assessed. Prophylaxis and immunisations should be considered against infections such as hepatitis, influenza, PCP and TB. When to initiate HAART depends on the clinical stage, CD4 and co-morbidities. This patient will need to be started on HAART. Classes of antiretrovirals include NRTIs, NNRTIs, protease inhibitors, fusion inhibitors and integrase inhibitors.

Answer 720

J. Malignancy - This person likely has lung cancer which has resulted in the significant weight loss of 10kg. This is post-obstructive pneumonia which is common in lung cancer patients and is caused, most of the time, by a large and centrally obstructing tumour. It is essential to relieve this obstruction in this case and many techniques can be tried. There is also bone pain here in the lower spinal column which is due to metastases. The prognosis here is not good.

Answer 721

E. Malabsorption - There is frequent loose stools here and abdominal pain. Combined with the IDA, this points to malabsorption. This could well be a presentation of coeliac disease – IDA is one of the most common clinical presentations and abdominal pain and diarrhoea are common. Coeliac disease is a systemic autoimmune condition triggered by dietary gluten peptides found in grains. It is a relatively common condition. The only treatment is a strict gluten-free diet for life.

Answer 722

I. Asterixis - Asterixis is a flapping tremor, or a liver flap, which is a coarse tremor of the hand when the wrist is held extended. It is caused in this case by liver failure and can be a sign of hepatic encephalopathy. The signs found on examination point to decompensated chronic liver disease.

Answer 723

E. Sydenham's chorea - Chorea features as part of the acute presentation in 5-10% of patients with rheumatic fever. It can also occur as an isolated event up to 6 months after the initial GABHS infection. It is named Sydenham chorea after the doctor who described St Vitus Dance in the 17th century. Choreiform movements can affect the whole body or just one side of the body, in which case it is referred to as hemi-chorea. The head is often involved with erratic facial movements that resemble grimaces, grins and growns, and the tongue may be affected to resemble a bag of worms when protruded, and protrusion cannot be maintained. In severe cases the patient may have an impaired ability to eat. Chorea disappears with sleep and is made worse by purposeful movements. When the patient is asked to grip the doctor’s hand, the patient will be unable to maintain grip and rhythmic squeezing occurs. There are two signs to look out for in these patients. The first is the spooning sign, which is a flexion at the wrist with finger extension when the hand is held extended. The pronator sign is the second which is when the palms turn outwards when held above the head. Both are consistent with chorea. Remember that the 5 major manifestations of acute rheumatic fever are carditis, polyarthritis, chorea, erythema marginatum and SC nodules – the most common of which are carditis and polyarthritis. Primary episodes occur mainly in children aged 5-14 and are rare in those over 30. The greatest burden of disease remains in the developing countries and in populations of people living in poverty.

Answer 724

B. Exaggerated physiological tremor - The tremor seen in hyperthyroidism is usually fine and is a exaggerated physiological phenomenon. Treatment aims to normalise thyroid function and is achieved by radioactive iodine, antithyroid medications or with surgery. They are all effective and relatively safe options. Symptomatic therapy is given with beta blockers such as propranolol.

Answer 725

C. Cerebellar tremor - A cerebellar tremor can be seen in MS, trauma or stroke. The history may feature complaints of incoordination and imbalance, a possible FH of cerebellar ataxia or PMH of MS, head trauma, stroke or cerebellar haemorrhage. The tremor itself is a coarse and irregular kinetic tremor which is generated proximally. There may also be abnormal finger-to-nose testing and heel-to-shin testing, dysdiadochokinesia, wide-based ataxic gait (like the patient is drunk) as well as dysarthria (speech problems). The first test to order is an MRI of the patient’s head which may show signs of cerebellar atrophy or may suggest changes of demyelination seen in MS or changes of stroke, trauma or haemorrhage. You may also want to perform other tests such as thyroid function tests to exclude hyperthyroidism.

Answer 726

H. MS - MS is a demyelinating CNS condition which is characterised by 2 or more episodes of neurological dysfunction which are separated in both time and space. MS classically presents in white women aged 20-40 with temporary visual/sensory loss although any presentation can occur. MRI is a sensitive test but less specific than spinal MRI, however, spinal MRI is abnormal in fewer cases. Treatment aims at treating the attack, preventing future attacks and symptomatic treatment of problems like bladder dysfunction, pain and fatigue.

Answer 727

J. Dressler's syndrome - This is percarditis again, likely to be Dressler’s syndrome. This is believed to be an autoimmune process with myocardial neo-antigens implicated in the aetiology and occurs typically 2-3 weeks post-MI. Typical treatment is with aspirin. It tends to subside in a few days and raised ESR is a lab finding which can be seen. See above for more information on angina.

Answer 728

A. Aortic regurgitation - Aortic regurgitation is the leakage of blood back into the LV in diastole. The collapsing pulse is also known as a water hammer or Corrigan’s pulse and describes the rapid rise and quick ‘collapse’ of the arterial pulse resulting in a wide pulse pressure. The murmur in AR is early diastolic in mild cases and increases to pansystolic in severe cases. Risk factors include a bicuspid valve, rheumatic fever, endocarditis, anklylosing spondylitis and Marfan’s. Other commonly seen signs in EMQs, although uncommon in clinical practice include Traube’s (pistol shot sounds over the femoral arteries when compressed), Quincke’s (subungal capillary pulsations), Duroziez’s (systolic and diastolic murmurs over the femoral arteries) and de Musset’s (head bobbing with each heart beat) sign. Other equally uncommon signs (there are many for AR) include Muller’s, Mayen’s, Lighthouse, Hill’s, Becker’s, Landolfi’s, Rosenbach’s, Gerhardt’s, Lincoln’s and Sherman’s sign. The visible carotid pulsation here is Corrigan’s sign, with the pulse characteristic as described previously. Occasionally although uncommon, an Austin Flint murmur may be heard which is a rumbling mid-diastolic murmur best heard at the apex, produced by the regurgitant jet hitting the LV endocardium. Its presence indicates severe AR and the absence of a loud S1 or an opening snap distinguishes this from the murmur of mitral stenosis.

Answer 729

C. Infective endocarditis - Any patient presenting with fever and a new murmur should always make you think of bacterial endocarditis. The weakness here may be consistent with a stroke and systemic emboli may be the cause here. The classic new or worsening murmur is actually rare. As are splinter haemorrhages, which EMQs often mention. Other uncommon signs you may find include Janeway lesions (painless macular haemorrhagic plaques on the palms and soles) and Osler nodes (painful nodules on the pads of the fingers and toes). Roth spots may also be seen on fundoscopy. Three sets of bood cultures are required and this patient will have to go for an echocardiogram.The Duke criteria is used for diagnosis.

Answer 730

B. Aortic stenosis - Aortic stenosis can present with chest pain, dyspnoea and syncope. It is characterised by a harsh ejection systolic murmur heard loudest at the right upper sternal edge at end expiration, which radiates up towards the carotids. The pulse pressure is narrow and there may be an associated slow-rising and plateau pulse. Doppler echo is vital for diagnosis and shows a pressure gradient across the narrowed valve orifice. 20% of cases are due to a congenital bicuspid valve. The most common cause of aortic stenosis in adults is calcification of normal trileaflet valves. Clinically stable patients may be considered for surgical repair or TAVR.

Answer 731

F. Mitral regurgitation - MR is loudest at the apex and radiates to the axilla and tends to be around grade 4. It is associated with a systolic thrill at the apex. TTE is the investigation of choice for diagnosis. Chronic MR is associated with a laterally displaced apex beat with LV dilatation. Mitral valve prolapse is a strong risk factor for development of MR. The alcohol history here is hinting at dilated cardiomyopathy as a possible cause.

Answer 732

G. Innocent murmur - This is a functional murmur which is not caused by a structural cardiac defect. They often reflect hyperdynamic states such as that seen in pregnancy. Functional murmurs tend to be systolic, occuring in an otherwise healthy individual with no symptoms. They are also characteristically position dependent and soft in nature. Ones that occur in children tend to disappear as the child grows. Benign paediatric murmurs include Still’s murmur.

Answer 733

D. Subarachnoid haemorrhage - SAH (bleeding into the subarachnoid space) presents with sudden severe headache patients will often describe as the worst headache of their life, and can often be so bad that they feel like they’ve been kicked in the back of the back. Half of all patients lose consciousness and eye pain with exposure to light can also be seen. Altered mental status is common. SAH occurs most commonly in the 50-55 age group and affects women and black people more than men and white people. The most common cause of non-traumatic SAH is an aneurysm which ruptures. Conditions which predispose to aneurysm formation and SAH include adult PKD, Marfan’s, NF1 and Ehlers-Danlos. Cerebral aneurysms arise around the circle of Willis. A CT scan is indicated, and if unrevealing, this should be followed by an LP. Cerebral angiography can confirm the presence of aneurysms. The patient should be stabilised and this followed by surgical clipping or endovascular coil embolisation, the choice is subject to much current controversy sparked by relatively recent research. Complications can commonly occur and include rebleeding, hydrocephalus and vasospasm.

Answer 734

L. Meningitis - This patient has meningitis. Universities are common sites of outbreaks due to crowding. Commonly there will be a headache, fever and nuchal rigidity. There may also be an altered mental status (this patient has LOC), confusion, photophobia and vomiting. Kernig’s sign is uncommon but is positive when attempts to extend the leg are met with resistance when the patient is supine with the thigh flexed to 90 degrees. Another uncommon sign is Brudzinski’s sign and the petechial/purpuric rash seen here, typically associated with meningococcal meningitis. CT head should be considered before LP if there is any evidence of raised ICP. An LP will confirm the diagnosis with bacterial meningitis showing a low CSF glucose, elevated CSF protein and positive CSF culture/gram stain or meningococcal antigen. Investigations should not delay treatment in this very urgent situation and antibiotics need to be promptly administered. IM benzylpenicillin can be given on the way to the hospital with empirical antibiotic therapy being started on arrival before the causative organism is identified. This can be, for example, with vancomycin and ceftriaxone (or meropenem, cefotaxime, chloramphenicol). Dexamethasone should also be given before the first dose of antibiotics and continued for 4 days as this has been shown to improve outcome and overall mortality in community-acquired bacterial meningitis (though NICE guidelines state it should not be given to those aged

Answer 735

B. Normal pressure hydrocephalus - This is normal pressure hydrocephalus – a diagnosis you should suspect in any patient who presents with a gait apraxia (the falls here from loss of balance) and cognitive impairment. There may also be urinary symptoms such as urgency, frequency or urge incontinence. These symptoms tend to be insidious in onset over months or years.There is not significantly raised CSF pressure though perhaps this should not be named ‘normal pressure’ as elevated CSF pressure may be seen but just not significantly so. CT head is the first test to order, although MRI head can also be done, and could be normal but could show mild to moderate ventricular enlargement, periventricular leukomalacia, cerebral infarction, relative preservation of the cortical gyri and sulci and reduced diameter of the corpus callosum and widened callosal angle. This diagnosis can be excluded if an obstructive lesion is seen. If you suspect Parkinson’s then a levodopa challenge should be ordered.

Answer 736

A. Wernicke's encephalopathy - Wernicke’s is due to acute thiamine deficiency, which is a problem in alcoholics. Others at risk include those with AIDS, cancer and treatment with chemotherapy, malnutrition and GIT surgery, especially bariatric procedures. It is a clinically under-diagnosed condition. The classic EMQ triad is of mental change, ophthalmoplegia and gait dysfunction, which is actually only seen in 10% of cases. In reality, the manifestations are varied and a high index of suspicion is needed. Despite there, the manifestiations typically include altered consciousness, gait disorders and eye movement abnormalities. This is an emergency and treatment is with parenteral replacement of thiamine. This avoids permanent neurological damage including later development of Korsakoff’s psychosis, which is irreversible. Note that thiamine should be given before dextrose! Magnesium deficiency also needs to be corrected as it is a co-factor in the functioning of thiamine dependent enzymes.

Answer 737

F. Hepatic failure - This patient has decompensated chronic liver disease (he is in liver failure) which has resulted in neurological symptoms associated with hepatic encephalopathy. The brain is exposed to ammonia which bypasses the liver by portosystemic shunting. It is a diagnosis of exclusion and tests will need to be conducted to rule out other potential causes of confusion. The findings of jaundice and fetor hepaticus (liver failure) are signs of liver disease. Think about the other signs you might see like spider naevi and palmar erythema. This patient may also have asterixis which is a coarse flapping tremor. HE is likely caused by a host of factors. This patient’s LFTs will be abnormal and she is likely to have coagulopathy too (PT will be elevated).

Answer 738

J. Myeloma - This patient has multiple myeloma. This is characteristed by clonal proliferation of plasma cells in BM and commonly presents with bony pain and symptoms of anaemia. There may also be infections present in 10%. Elevated ESR agrees with this diagnosis. 30% have hypercalcaemia and half have renal impairment, which is associated with a worse prognosis. This explains the elevated urea and creatinine here. The diagnostic test is serum or urine electrophoresis looking for a paraprotein spike of IgG or IgA and light chain urinary excretion (Bence Jones proteins). Bone marrow examination and skeletal survey will also need to be conducted. Bone marrow analysis will help differentiate this from MGUS and solitary plasmacytoma. Bone changes include osteopenia, osteolytic lesions and fractures which this person’s XR indicates with the punched out lytic lesions. Younger patients may be candidates for high-dose chemotherapy and autologous transplantation.

Answer 739

D. Aplastic anaemia - This is aplastic anaemia characterised here with the pancytopenia (which is common, but diagnosis requires 2 cytopenias out of 3) and the presentation with infections (neutropenia), fatigue (anaemia) and bruising (thrombocytopenia). Risk factors include paroxysmal noctural haemoglobinuria, hepatitis and NSAIDs. If macrocytosis is seen, this may suggest an inherited syndrome such as Fanconi’s anaemia. The reticulocyte count here rules out haemolytic anaemia. The definitive diagnosis is made on biopsy of bone marrow which shows a hypocellular marrow with no abnormal cell populations and no fibrosis. Which conditions would there be abnormal cell populations or fibrosis on bone marrow biopsy?

Answer 740

F. Chronic myeloid leukaemia - This is CML which tends to present in the 30-60 age group. At presentation 1/3 may be asymptomatic though if symptomatic, it presents with symptoms including fever, weight loss and night sweats. There is myeloid stem cell proliferation and presents with raised neutrophils, metamyelocytes and basophils. The patient may also describe LUQ discomfort or fullness due to the feeling of a mass due to splenomegaly. There are also symptoms of anaemia here due to BM infiltration of leukaemic cells. Bruises are common and are either spontaneous or from minor trauma. All patients have raised WCC. CML is associated with the philadelphia chromosome characterised by t(9;22) of bcr-abl. There tends to be massive splenomegaly which is the most common physical finding on examination. This conditon may transform to AML or ALL in what is known as a ‘blast crisis’. CML responds to imatinib, which is an anti-bcr-abl antibody and gives long term remission in most patients.

Answer 741

C. Sickle cell anaemia - The reticulocytosis here is as a result of the haemolytic anaemia this patient is suffering due to sickle cell anaemia. The bone pain in the chest and hip is an acute symptom of vaso-occlusive crises. It is generally unusual for someone to reach this age without being aware that they have sickle cell anaemia so in real life, this would be an uncommon way to present. The diagnosis should however be suspected due to the episodes of pain (vaso-occlusive crises) and the findings of anaemia with reticulocytosis. Sickling occurs when RBCs containing HbS become distorted into a crescent shape. Patients with sickle cell anaemia have no HbA at all. If both parents carry the sickle cell gene, there is a 1 in 4 chance of giving birth to a child with sickle cell anaemia.

Answer 742

E. Iron deficiency anaemia - IDA is a microcytic hypochromic anaemia characterised by low serum iron, high TIBC and low transferrin saturation and serum ferritin. Bleeding is the principle cause of IDA. IDA is not an end diagnosis and has many causes and this is something that is important to bear in mind at all times. The cause here is from chronic blood loss from what would appear to be possible right sided bowel cancer. Worldwide, the most common cause is hookworm infection, and in the UK, menstrual losses in women. Causes of IDA can be broadly divided into 4 categories: decreased intake, increased loss, increased requirements (such as in pregnancy), and unknown.

Answer 743

B. Legionella pneumoniae - Legionella is a gram negative rod. Legionella infecting the lungs is legionnaires’ disease or Legionella pneumonia whereas non-lung infection is known as Pontiac fever. This bacteria is found in aqueous environments such as lakes and almost all cases are from contaminated water systems, which relates to the risk factors of getting Legionella (recent water exposure like a hot tub). Smoking is also a risk factor. It can cause confusion as well as hyponatraemia, abdominal pain, diarrhoea and bradycardia. Legionella does not grow on routine culture media and diagnosis relies on urine antigen detection, serology or culture on special media.

Answer 744

H. Pseudomonas aeruginosa - Pseudomonas has virulence factors for lung colonisation and is noted for its type III injection apparatus, which you may remember from your microbiology lectures. This organism is one of the main 5 organisms responsible for hospital acquired (or nosocomial) infections in the UK. Remember that a nosocomial infection is defined as one acquired >48 hours after admission. It is a gram negative bacilli causing hospital acquired pneumonia and UTI. It particularly affects immunocompromised hosts such as those on chemotherapy, those with cystic fibrosis, burns and wounds. Biofilms are antibiotic resistant. Key risks also include urinary catheters and central venous lines etc... I would not worry too much about MacConkey agar as lots of things grow on it. It is a culture medium designed to grow gram negative bacteria, so organisms ranging from E. coli, Klebsiella and Salmonella, to Proteus, Shigella and Enterobacter will all grow on it. This just rules out Streptococcus pneumoniae. Pseudomonas is also a special little organism which is not sensitive to many antibiotics originally used for gram negatives. You need to prescribe a drug here which has specific anti-pseudomonal activity such as ceftazidime (a third generation cephalosporin), tazocin, ciprofloxacin, imipenem or gentamicin (usually used with one of the others). This organism acquires resistance genes very quickly so two antibiotics are given. It is, as mentioned, inherently resistant to many drug classes. Some are even resistant to all antibiotics.

Answer 745

J. Streptococcus pyogenes - The ‘strawberry’ tongue, or a red swollen tongue, is a sign of Scarlet fever (along with Kawasaki disease and toxic shock syndrome which is caused by bacteria such as staphylococcus aureus). Scarlet fever is caused by an exotoxin released by Streptococcus pyogenes. The history is characteristically a child

Answer 746

K. Leptospira interrogans - Leptospirosis is a zoonosis, which is transmitted by contact with urine of infected animals (also possible sources include blood and fluids). The history may reveal someone swimming in rat infested canal water, for instance, or in this case, a triathlon – which involves some swimming in perhaps not too clean water. Outbreaks of this are associated with flooding and natural disasters, as can be expected. Affected patients can present with an extensive spectrum of clinical manifestations ranging from subclinical illness in 90% to renal and hepatic failure and pulmonary haemorrhage. The important factor in diagnosis is a high index of suspicion based on epidemiological exposure. There is an acute phase with fever, headaches, myalgia and then an immune phase with additional pulmonary symptoms and potential organ damage (leading to the symptoms seen here such as jaundice). The rash is maculopapular and non-pruritic, lasting 1 or 2 days, present during the acute phase. It is rarely seen. Treatment is with benzylpenicillin or amoxicillion and/or doxycycline and supportive care. Those with severe disease carry a poor prognosis.

Answer 747

E. Campylobacter jejuni - This is what we colloquially refer to as ‘food poisoning’ – infective gastroenteritis. The most common bacterial cause in the UK is Campylobactor jejuni. This organism is also a major cause of traveller’s diarrhoea. The second most common cause of acute enteritis is an organism known as Campylobacter coli. There is also Campylobacter fetus which is a rare cause of extra-intestinal infection mainly in immunocompromised patients. Campylobacters are small, gram negative rods with a curved spiral shape. 11 Campylobacters are pathogenic to humans. Most Campylobacter jejuni infections come from infected poultry. The diarrhoea is usually self limiting and resolves in a week – bacteraemia is very rare. Fluid and electrolyte replacement is essential. Only a small number of patients will benefit from antimicrobials. Note that C. jejuni is a commonly identified organism in the aetiology of Guillain-Barre syndrome.

Answer 748

G. MEN II - MEN (Multiple Endocrine Neoplasia) syndromes are hereditary autosomal dominant tumour syndromes with distinct patterns of organ involvement. At this stage, the purely simplified patterns (classification is actually more complicated) you need to be familiar with are: MEN1 consists of parathyroid adenomas, pancreatic tumours and pituitary adenomas. MEN2A consists of parathyroid, medullary thyroid cancer and phaeochromocytoma. And MEN2B or 3 consists of what is seen in 2A plus the addition of a marfanoid phenotype and ganglioneuromas (intestinal and visceral). Prophylactic thyroidectomy in childhood is indicated in MEN2. Medical management is aimed at controlling hormone hypersecretion. Surgery is often done on tumours. If you’re really interest, you can look MEN syndromes up in more detail. MEN1 is typically caused by mutations in the MEN1 gene whereas MEN2 is typically caused by mutations in the RET proto-oncogene.

Answer 749

A. Nelson's syndrome - Nelson’s syndrome is the enlargement of a pituitary adenoma which occurs after bilateral adrenalectomy. Once you know this fact, the diagnosis is clear. Bilateral adrenalectomy is an operation which can be done for Cushing’s syndrome in order to completely eliminate the production of cortisol. However, this removes cortisol’s negative feedback response which allows any pre-existing pituitary adenoma to grow without negative feedback. As a result, this rapid enlargement of the pituitary adenoma has caused this man’s symptoms of increased pigmentation due to raised MSH (a by product of POMC cleavage to give ACTH), headaches and visual disturbances (due to the space-occupying lesion). This is now rare as the operation is now only used in extreme cases. Sometimes pituitary surgery will be performed.

Answer 750

J. Sheehan's syndrome - Sheehan’s syndrome is panhypopituitarism which occurs due to necrosis of the pituitary gland following post-partum haemorrhage and hypovolaemia. Blood loss leads to a vasoconstrictor spasm of the hypophysial arteries leading to ischaemia and necrosis of the pituitary. This is a rare complication of pregnancy. Symptoms include those seen and are due to deficiency of a host of hormones. Treatment is aimed at replacing the missing hormones (hydrocortisone, thyroxine etc).

Answer 751

B. Pseudo-Cushings syndrome - Pseudo-Cushing’s syndrome is where a patient has all of the signs and symptoms and even abnormal hormone levels seen in Cushing’s syndrome however there is no problem to be found with the hypothalamo-pituitary-adrenal axis. It is hence idiopathic. Whole body CT here is normal so rules out causes from organs such as the lungs (ectopic ACTH), adrenals and MRI has also ruled out a pituitary cause.

Answer 752

F. Kallmann's syndrome - Kallmann’s syndrome (hypogonadotrophic hypogonadism) is a cause of primary amenorrhoea. The history tends to be of delayed development of secondary sexual characterisitcs with anosmia. There is either a missing olfactory bulb or one which is not fully developed (this may be seen on MRI) so there may be a lack of a sense of smell or a sense of smell which is severely reduced (hyposmia seen here). It may be diagnostically challenging as it is difficult to distinguish pathological developmental delay from constitutional delay. Normal but pre-pubertal external and internal genitalia are seen, and normal final adult height if treated. You would expect serum FSH to be low (though FSH assays have very very very wide ranges) and LH if done. Low oestradiol can also be expected.

Answer 753

J. Guillian-Barre syndrome - This previous history of influenza-like illness weeks before the onset of neurological symptoms indicates Guillain-Barre syndrome which is a demyelinating polyneuropathy. Classic neurology is a progressive symmetrical muscle weakness affecting lower extremities before upper extremities, and proximal muscles before distal muscles, accompanied by paraesthesias in the hands and feet which often precedes onset of weakness. The paralysis is typically flaccid with areflexia and progresses acutely over days, with an ensuing plateau phase followed by recovery. Two thirds of patients have a history of either prior influenza-like illness or gastroenteritis. Weak risks include immunisation, cancer and lymphoma, older age, HIV infection and male gender. Up to 30% will develop respiratory muscle weakness requiring ventilation so spirometry should be carried out at 6 hour intervals initially (and may show reduced vital capacity). AST and ALT may be elevated though the cause is unclear. LP is useful and the classic finding is of elevated CSF protein with normal cell count (known as albuminocytological dissociation). Treatment is with supportive and disease modifying treatment (plasma exchange or high dose Ig). GBS is classified by symptoms and variants exist such as Miller-Fisher syndrome, which occasionally crop up in EMQ books but will probably never crop up in clinical practice.

Answer 754

D. Milroy's disease - Milroy’s is primary lymphoedema with a familial autosomal dominant pattern of inheritance. It often presents in infancy. There is unfortunately no cure and treatment involves compression, with surgery reserved for those refractive to conservative measures and/or with major morbidity.

Answer 755

E. Tietze's syndrome - This is costochondritis, or Tietze’s syndrome (which describes constochondritis accompanied by chest wall swelling), which presents with insidious onset of anterior chest wall pain which is made worse by certain movements of the chest and deep inspiration. The key sign here is that there is pain when palpating the costochondral joints, particularly the 2nd to the 5th and the diagnosis is clinical. Tests are done to exclude other diagnoses here such as breast pathology. First line treatment is with NSAIDs. Oral NSAIDs are preferred in a primary care setting and a beneficial response confirms the diagnosis. If NSAIDs or local corticosteroid injection (usually performed by a specialist) fail to make the symptoms better than you should seek further investigations and consider a wider differential diagnosis which include conditions like pleuritis, ACS, PE, rib fracture and GORD.

Answer 756

H. Felty's syndrome - Felty’s syndrome is a rare extra-articular manifestation of rhematoid arthritis characterised by persistent and idiopathic neutropenia and in some cases splenomegaly. It occurs in

Answer 757

F. Osler-Weber Rendu syndrome - This man has the facial telangiectasia of OWR, also called hereditary haemorrhagic telangiectasia. This causes abnormal blod vessels pretty much everywhere which are prone to bleed. It is an autosomal dominant condition so a positive FH can often be found.

Answer 758

H. Subarachnoid haemorrhage - SAH (bleeding into the subarachnoid space) presents with sudden severe headache patients will often describe as the worst headache of their life, and can often be so bad that they feel like they’ve been kicked in the back of the back. Half of all patients lose consciousness and eye pain with exposure to light can also be seen. Altered mental status is common. SAH occurs most commonly in the 50-55 age group and affects women and black people more than men and white people. The most common cause of non-traumatic SAH is an aneurysm which ruptures. Conditions which predispose to aneurysm formation and SAH include adult PKD, Marfan’s, NF1 and Ehlers-Danlos. Cerebral aneurysms arise around the circle of Willis. The family history of kidney disease here points to adult PKD. A CT scan is indicated, and if unrevealing, this should be followed by an LP. Cerebral angiography can confirm the presence of aneurysms. The patient should be stabilised and this followed by surgical clipping or endovascular coil embolisation, the choice is subject to much current controversy sparked by relatively recent research. Complications can commonly occur and include rebleeding, hydrocephalus and vasospasm.

Answer 759

G. Pyoderma gangerosum - This is pyoderma gangrenosum, which presents with multiple lesions, most commonly affecting the lower extremity and is linked to UC although it can be seen less commonly in Crohn’s, and is also seen in conditions such as RA and the myeloid dyscrasias. These lesions start as tender papules or vesicles which develop into painful ulcers with a dusky purple edge and surrounding induration and erythema. The base may contain granulation tissue and lesions heal with atrophic scars.

Answer 760

D. Sickle cell anaemia - Adults with sickle cell anaemia may present with leg ulcers although it has to be said that it is very unusual for a person with sickle cell disease to reach adulthood without being aware of their diagnosis. The adult patient could present with unexplained haemolysis, possible intermittent episodes of pain due to vaso-occlusive crises, avascular necrosis and retinal haemorrhage too. The doppler derived ankle-brachial pressure index is normal here which points away from peripheral arterial disease, which in this relatively young person is unlikely in any case. The typical constellation of findings of venous ulcers are not seen (oedema, lipodermatosclerosis). In real medicine, as it is rare for sickle cell anaemia to present this late, I would consider other possibilities like pyoderma gangrenosum (which is arguably more likely in this case, given the location of the ulcer and age of the patient, so if this was your answer then you can give yourself a ‘real life medicine’ mark), but this is an EMQ (key point!) and the fact this lady is from the sub-Saharan Africa is a key fact. From an epidemiological perspective, the prevalence is 10-30% in sub-Saharan Africa. Between 25 and 30% of newborns in western Africa are carriers of sickle cell trait. This patient here will need to have their peripheral blood film reviewed following by haemoglobin electrophoresis and HPLC. Sickle solubility is a rapid test which can also be done but will not differentiate sickle trait from sickle disease.

Answer 761

I. Squamous cell carcinoma - SCC is a malignant tumour of keratinocytes arising in the epidermis of the skin. Risk factors include UV exposure, older age, immunosuppression, fair skin, HPV and exposure to ionising radiation. The skin changes here are characteristic. Most frequently however, SCC often presents with multiple actinic keratoses, with a change in the lesion such as bleeding, induration, change in size and shape or inflammation. Aks and SCCs lie on a spectrum so it may be difficult to clinically differentiate the two. Skin biopsy will confirm the diagnosis. This can either be a shave biopsy, punch biopsy, incisional biopsy or excisional biopsy depending on the size and location.

Answer 762

A. Porhyria - There are many types of porphyria. This patient has acute intermittent porphyria, which is characterised by symptoms like the ones this patient describes – abdominal pain, peripheral motor neuropathy, mental symptoms like confusion. These symptoms, certainly in EMQs, can be trigged by the use of certain drugs which are known to provoke AIP attacks. In reality, the list of drugs is pretty vast and include most CYP450 inducers, but in EMQs, alcohol and the OCP are common. It is worth noting that alcohol also induces an enzyme called delta-aminolevulinic acid synthase, which can exacerbate AIP. It is probably not worth learning the pathways unless you plan on sitting USMLE. AIP is a genetic disorder where there is a partial deficiency of PBGD (the third enzyme in the haem biosynthetic pathway). Treatment of acute attacks involves IV haem arginate with adjunctive dextrose IV. The pain is thought to be neuropathic in origin. The patient may complain of red/browny urine due to increased urinary excretion of intermediates in the haem pathway.

Answer 763

B. Beri-beri - Beriberi is vitamin B1 (thaimine) deficiency. Deficiency is the cause of several clinical syndromes including wet and dry beriberi and Wenicke’s encephalopathy. The presentation depends on the chronicity of B1 deficiency. Dry beriberi is a distal peripheral polyneuropathy characterised by parasthesia, reduced knee jerks and other tendon reflexes, and progressive severe weakness with muscle wasting secondary to chronic deficiency. Wet beriberi, which this person also has is secondary to either acute or chronic deficiency and is characterised by high-output heart failure with peripheral vasodilation, peripheral oedema and orthopnoea or low-output heart failure with lactic acidosis and peripheral cyanosis. The latter is also referred to as Shosin beriberi. Remember that Wernicke’s is an acute neuropsychiatric syndrome which classically presents with the triad of acute confusion, ataxia and ocular abnormalities (such as nystagmus and strabismus) secondary to acute B1 deficiency.

Answer 764

H. Diabetic amyotrophy - Diabetic amyotrophy, more common in T2DM, is an uncommon peripheral diabetic neuropathic complaint. It presents with severe muscle weakness and pain with proximal thigh muscle atrophy. Weak knee flexion and quadriceps wasting is typical of diabetic amyotrophy.

Answer 765

I. HIV - HIV is a retrovirus and there are two types, HIV 1 which is the main virus responsible and HIV 2 which is restricted to parts of West Africa. Strong risk factors include needle sharing with IVDU, unprotected receptive intercourse, needle stick injury and high maternal viral load (mother to child). Kaposi’s sarcoma may present as a pink or violaceous patch on the skin or in the mouth and it is an AIDS-defining condition. Peripheral neuropathy is common and may be related either to HIV or some other medicine or toxin (some HAART can cause PN). There are WHO (stage 1-4) and CDC criteria used in clinical staging. This patient needs to have a CD4 count, HBV and HCV screen, VDRL (syphilis), tuberculin skin test (TB) and CXR. HIV viral load will also be assessed. Prophylaxis and immunisations should be considered against infections such as hepatitis, influenza, PCP and TB. When to initiate HAART depends on the clinical stage, CD4 and co-morbidities. This patient will need to be started on HAART if he has not already. Classes of antiretrovirals include NRTIs, NNRTIs, protease inhibitors, fusion inhibitors and integrase inhibitors.

Answer 766

D. Vitamin B12 deficiency - This is a macrocytic anaemia with elevated MCV. The classical peripheral blood smear would show megalocytes (RBC precursors whose numbers increase) and hypersegmented polymorphonuclear cells although the film may be normal in early deficiency. You would also want to order a serum B12 level in this patient and would expect the corrected reticulocyte index to be low due to decreased production (opposed to high in haemolytic anaemia due to increased production). This peripheral sensory neuropathy would likely be a loss of proprioception and vibration indicating degeneration of the dorsal column-medial lemniscus pathway. The patient may also have a positive Romberg as a result. Parasthesias are also common in B12 deficiency indicating peripheral neuropathy. Pallor and signs of frank anaemia are late signs. It is important to remember that hepatic B12 stores last for many years so B12 deficiency depends on chronic deficiency over a long period of time. In general, deficiency is caused by decreased intake, decreased gastric breakdown from food or malabsorption from the GIT. Serum B12 is a useful investigation to initially conduct and will guide further investigations and treatment. You should consider an underlying malabsorptive process such as coeliac or Crohn’s as a possible cause.

Answer 767

D. Neurogenic shock - This is a thoracolumbar spine fracture. Neurogenic shock is not to be confused with spinal shock which is not circulatory in nature (and is characterised by hypotonia or flaccidity that resolves within 24 hours). Neurogenic shock is a form of distributive shock due to spine or braintem injury and there is resulting failure of vasoregulation. As a result there is a fall in systemic vascular resistance with vasodilation, leading to low BP as blood pools in the extremeties where sympathetic tone is low. This is occasionally associated with bradycardia which is due to autonomic disruption.

Answer 768

H. Urinary retention - This is clearly urinary retention. Urine output is gradually deteriorating in a step-wise manner and he is now anuric

Answer 769

G. Pulmonary oedema - This patient has been fluid overloaded and has pulmonary oedema, causing her dyspnoea. She will require diuretics and fluid restriction to deal with his overloaded state. The findings of bibasal fine creptitions, heard at the end of expiration, is characteristic. Infiltrates may also be seen on CXR. Type 1 respiratory failure is hypoxic respiratory failure, which occurs when PaO2 is low. Type 2 respiratory failure is known also as hypercapnic respiratory failure and occurs when there is hypoxia associated with a high PaCO2. As a general note for your exams. Common causes of type 1 respiratory failure include pulmonary oedema, pneumonia and PE. Common causes of type 2 include COPD and respiratory muscle weakness.

Answer 770

E. Haemorrhagic shock - This is a history of a ruptured AAA. He has key risk factors of obesity, smoking and T2DM. There is abdominal pain radiating to the back here and the low BP and compensatory tachycardia is due to the blood loss. This patient is in haemorrhagic shock. As this AAA has ruptured, this man will need urgent surgical repair, with of course standard resuscitation measures. The airway will needed to be managed with supplemental oxygen and ET intubation, a central venous catheter will need to be inserted, an arterial catheter and urinary catheter will also be needed for monitoring, and the target systolic BP is 50-70. Infusing too many gluids may increase the risk of death. The most effective form of surgical repair is an EVAR (endovascular AAA repair), anatomy permitting, otherwise traditional open repair is performed. Open repair has a mortality of 48%. Antibiotics will also be needed to cover bacteria to prevent graft infection. This will be prescribed in line with local protocols.

Answer 771

J. Percutaneous jejunostomy - This patient needs nutritional support to prevent malnutrition and starvation. Enteral nutrition here is not possible as a chunk of the oesophagus has just been removed and we have to wait for an intact anastomosis. During surgery, a percutaneous jejunostomy can be placed to provide a temporary route of nutrition until oral feeding can resume. A surgeon at operation cannot place a percutaneous gastrostomy, and it is more suitable for prolonged feeding. Furthermore, in the immediate post-operative period, there may be gastric stasis so it is preferred to deliver the feed via a post-pyloric placement. If you have seen a PEG tube (percutaneous gastrostomy), you will know why it is not preferred. It is not a pleasant sight (and I don’t mean cosmetically – just have a look at the needle). Some younger people may ask for it to be converted to a button gastrostomy for cosmetic reasons. A PEG tube is placed laparoscopically. A percutaneous jejunostomy is an alternative to parenteral (IV) nutrition in this post-operative patient. Parenteral nutrition is really a last resort and this is not an indication for it. Only when the GIT is either unavailable or function is indequate should you consider it. This can be delivered via a venflon, PICC line or centrally. There are a host of complications, both nutritionally, related to the catheter e.g. infection, thrombosis and the effect on organ systems e.g. biliary disease. Note that you should also be aware of the phenomenon known as refeeding syndrome.

Answer 772

H. IgA nephropathy - About 50% of those with IgA nephropathy will present with recurrent episodes of macroscopic haematuria after a URTI or gastroenteritis. About a third will have microscopic haematuria and mild proteinuria. Less than 10% present with nephrotic syndrome or acute rapidly progressive GN. Fever and myalgia are systemic findings. The oedema here may indicate nephrotic syndrome. Definitive diagnosis is made on renal biopsy. Light microscopy shows focal or diffuse mesangial proliferation and extracellular expansion, and IF shows diffuse mesangial IgA deposition in a granular pattern. ACE inhibitors are prescribed to reduce proteinuria, particularly in the setting of hypertension but they have not been shown to preserve renal function. Early treatment with corticosteroids has been shown to delay renal decline in those with moderate proteinuria. A urine dipstick would be the first test to order here.

Answer 773

D. Goodpasture's syndrome - Goodpasture’s syndrome is defined by autoantibodies to the alpha-3 chain of type IV collagen which leads to progressive renal dysfunction. It is one of the few causes of pulmonary renal syndrome, which is characterised by pulmonary haemorrhage with rapidly progressive GN. Diagnosis is by early renal biopsy and serology. Around 30-50% of patients with anti-GBM disease will have a positive ANCA result, which changes subsequent management of this condition. Positive ANCA usually suggests a diagnosis of Wegener’s granulomatosis, Churg-Strauss or microscopic polyarteritis instead of Goodpasture’s. Note that MPO-ANCA is also known as pANCA and ANCA stands for anti-neutrophil cystoplasmic antibodies. Anti-GBM serology is a confirmatory diagnostic test in addition to renal biopsy and this can also be used to monitor response to treatment and to gauge when to stop plasmapheresis (i.e. when the antibody titre turns negative). Biopsy of the kidneys will show a crescentic GN and characteristic linear IgG staining on immunofluorescence. The renal function tests here are abnormal, which is the hallmark of Goodpasture’s syndrome. If it is diagnosed before renal dysfunction becomes severe then aggressive treatment can lead to an excellent prognosis. Plasma exchange can be used to remove preformed antibodies.

Answer 774

I. Post-streptococcal glomerulonephritis - Nephritic syndrome is typically defined by acute kidney injury, hypertension and an active urinary sediment (RBCs and RBC casts). This is post-infectious glomerulonephritis caused by group A beta-haemolytic streptococcus with renal endothelial cell damage. Serological markers would expect to show antibodies to streptococcus and low complement and treatment here is with antibiotics. The high ASOT (antistreptolysin O antibody titres) indicates post-streptococcal GN. There may also be positive anti-Dnase and antihyaluronidase in post-streptococcal GN. As a note, causes of nephritic syndrome (with sub-nephrotic range proteinuria) include: IgA nephropathy, post-infectious GN and rapidly progressive GN. Nephrotic syndrome causes include minimal change disease, focal and segmental glomerulosclerosis, membranous nephropathy, deposition diseases and membranoproliferative GN.

Answer 775

A. Henoch-Schonlein purpura - Henoch-Schonlein purpura is the most common vasculitis in childhood and in all cases there is a rash of palpable purpura which are typically non-blanching. If there is no rash, then it is not HSP. They are normally 2-10mm in diameter and are due to the extravasation of blood into the skin. They can occur anywhere on the body but are usually concentrated on the lower extremities. Half of all patients have abdominal pain and arthralgias are commonly present (found in about 80%) and often associated with oedema. The joints most often affected are the knees and ankles. About half will show signs of renal disease such as proteinuria or haematuria. Risk factors for this condition include being male, age 3-15 and history of prior UTI. Complications can occur and the most common cause of death is renal failure. While serum IgA levels may be elevated, this is not a specific test for HSP.

Answer 776

G. Retroperitoneal fibrosis - Retroperitoneal fibrosis is a rare disorder where there is proliferation of fibrous tissue in the retroperitoneum which here has caused the ureters to become obstructed. This has led to oliguria, back pain and the palpable kidneys. The cause could be an effect of antihypertensives, or due to malignancy or some other cause. A CT scan is the test to order here and treatment depends on severity and can be either surgical or medical, the latter generally with glucocorticoids followed by DMARDs.

Answer 777

J. Chvostek's sign - Trousseau’s sign is carpal spasm when a blood pressure cuff is used for several minutes. Carpopedal spasm that occurs with hypocalcaemia is a painful spasm and could be the presenting sign. Chvostek’s sign is twitching of the perioral muscles in response to tapping over the facial nerve at the ear. If urgent replacement is necessary, calcium gluconate can be given IV. It is preferred over calcium chloride as it causes less tissue necrosis if it leaks out. It is worth noting that digoxin may be ineffective until serum calcium is restored to normal.

Answer 778

D. Raccoon eyes - Basilar skull fractures have specific clinical features. Blood pooling from these fractures can cause periorbital bruising (raccoon eyes), brusing over the mastoid area (Battle’s sign) and bloody otorrhoea. There may also be CSF leak resulting in CSF otorrhoea or rhinorrhoea. A unilateral raccoon eye has an 85% positive predictive value for this diagnosis.

Answer 779

E. Grey-Turner's sign - Complicated haemorrhagic pancreatitis may exhibit Cullen’s sign, Grey-Turner’s sign and Fox’s sign. Grey-Turner’s sign refers to bruising of the flanks and can take 24-48 hours to occur. It is due to retroperitoneal haemorrhage.

Answer 780

F. Murphy's sign - A positive Murphy’s sign may commonly be seen in cholecystitis. This is where palpation of the right subcostal region reveals tenderness. During deep inspiration, the tenderness suddenly becomes worse and there is inspiratory arrest as a result. This sign can also be elicited during ultrasound examination when pressure is applied using the ultrasound probe.

Answer 781

H. Quincke's sign - Quincke’s sign is an uncommonly seen sign where there is subungal or lip capillary pulsations caused by the large stroke volume seen in AR. This is a peripheral sign associated with a bounding pulse and the systolic hypertension of chronic severe aortic regurgitation.

Answer 782

B. Abdominal ultrasound - This sounds like ADPKD. There may be a FH of PKD or ESRF or cerebrovascular events (intracranial berry aneurysms in the circle of Willis and SAH). Patients may have haematuria, palpable kidneys and symptoms of a UTI. Hypertension and flank pain are also commonly seen. Hepatosplenomegaly may also be found. A renal ultrasound is the first test to order when the diagnosis is suspected. If the ultrasound is equivocal, a CT scan can be done of the abdomen and pelvis.

Answer 783

D. MSU: microscopy antigen blood test - This young woman has presented with a UTI (dysuria, frequency, haematuria). Sexual activity the strongest risk factor for UTIs in women and she’s just come back from ‘holiday’. It is diagnosed with a urine dipstick (already done), and microscopic analysis for bacteria, WBC and RBC and urine culture and antibiotic sensitivities. Antibiotic selection should be based on local guidelines or known sensitivities. Uncomplicated UTIs with no known antibiotic resistance can be treated with co-trimoxazole or nitrofurantoin. If there is resistance, a quinolone can be considered such as ciprofloxacin.

Answer 784

A. Cystoscopy - "Warts in the bladder" is the way of some sneaky urologist avoiding telling this man he had cancer. He was treated, and like all cancer patients, was followed up. Unfortunately, this sounds like a recurrence. Gross haematuria is the primary symptom of bladder cancer. Cystoscopy and urinary cytology are key in diagnosis. Low grade tumours are papillary and easy to see on cystoscopy whereas high grade tumours and carcinoma in situ are often difficult to visualise. Resection provides diagnosis and primary treatment in one step.

Answer 785

G. Helical CT - This patient has renal colic which classically presents with severe flank pain radiating to the groin. Microscopic haematuria is present in up to 90% of cases. Up to 85% of stones are visible on a plain KUB although urate stones are radiolucent. If the stone is radio-opaque, calcification will be seen within the urinary tract. In pregnancy, a renal USS is first line. The IVP has now been replaced by the CT scan which is the new diagnostic standard. A non-contrast helical (or spiral) CT is preferred due to high sensitivity and specificity and acurately determines presence, site and size of stones. Stones are analysed after they are extracted or when they are expelled to check their composition. It is worth noting that in all females of child bearing age, a urine pregnancy test is necessary to exclude an ectopic pregnancy.

Answer 786

I. CT scan - This patient has a dissecting aortic aneurysm which can be diagnosed with a CT scan showing the presence of an intimal flap. The CT scan should include chest, abdomen and pelvis to visualise the extent of the aneurysm. Dissecting aneurysms are either type A, which involves the ascending aorta, or type B. Type A dissections require urgent surgery whereas type B can be managed medically if it is not complicated by end organ ischaemia. The aortic dissection has led to a cholesterol embolism. This can be diagnosed histopathologically with the finding of cholesterol crystals. The phenomenon where cholesterol is released from an atherosclerotic plaque is called ‘trash foot’. A highly technical medical term. This results in the mottled appearance of distal embolism associated with livedo reticularis. You can search the internet for some case reports of this phenomenon.

Answer 787

G. Venous duplex scan - This patient has a DVT. Other risk factors include recent major surgery, active malignancy, pregnancy and malignancy. A Wells score is determined in all patients with a suspected DVT with the condition being likely if the score is 2 or greater. The most definitive test is venography but it is invasive. Compression USS of the proximal deep venous system is preferred but Doppler venous flow testing can be used if other tests are unavailable and will demonstrate low flow in affected veins. This requires a trained technician. Anticoagulation is the mainstay of treatment with unfractionated heparin, a LMWH or an anti FXa agent such as fondaparinux.

Answer 788

F. Blood cultures - This patient has sepsis. Sepsis is the presence of SIRS with a likely infectious cause. This patient’s profound arterial hypotension means he has severe sepsis (dysfunction of one or more organ systems). The patient being a young university student most likely has meningitis even though symptoms of headache, photophobia and neck stiffness are not mentioned. Hence, a LP would be performed but it is not the 1st test to order in a patient who presents with sepsis. It is important in the first instance to obtain a blood culture immediately, and preferably before antibiotics are started. If this is bacterial meningitis, you would expect to see a raised WCC on the LP with elevated protein, normal/reduced glucose and predominantly neutrophils in the white cell differential. Early blood cultures allows you to either broaden your empirical antibiotic spectrum or narrow it in those with sensitive organisms. It is worth noting that in sepsis, the patient may have a low temperature

Answer 789

H. Anti-neutrophil cytoplasmic antibody - This patient has Wegener’s granulomatosis, a systemic vasculitis affecting small and medium sized vessels. The classic triad includes upper and lower respiratory tract involvement and GN. Musculoskeletal manifestations such as arthralgia and signs of thromboembolism are commonly seen. A positive cANCA (antigen being proteinase 3) in the setting of the classic triad is sufficient to diagnose Wegener’s. Urinalysis and microscopy is also indicated to reveal renal involvement and a CT chest may reveal lung involvement, particularly in those who are asymptomatic for pulmonary involvement. This may show nodules or infiltrates.

Answer 790

B. Arteriogram - Diabetic neuropathy has lead to the painless ulcer developing over pressure points in the foot such as on the ball of this man’s foot. This may have resulted from an object becoming lodged in the shoe and eroding through the skin with walking. This is why it is important for diabetics to check their feet regularly and to wear specialised footwear. The black toes on his right foot are likely due to peripheral vascular disease. It is worth noting that peripheral vascular disease refers specifically to peripheral arterial disease. The first line study is an ABPI although an arteriogram can be done to identify areas of stenoses which can be treated surgically. The black mummified toes may have to be amputated.

Answer 791

A. Thick blood film - In the Western world, almost all cases of malaria occurs in travellers so an adequate travel history is crucial or the diagnosis may be missed. Patients typically present with non-specific symptoms such as a fever, sweats, chills and myalgia. This Sikh may have just arrived from an endemic area. Sometimes EMQs will describe patterns of fevers occuring at regular intervals of 48-72 hours associated with P. vivax, P. ovale and P. malariae infections but in most patients there is no specific pattern. Hepatosplenomegaly is a common presenting sign, although in the Western world, it is not common on initial presentation. The test of choice is Giesma-stained thick and thin blood smears. Thick films sensitively detect parasites whereas thin films allow species identification and calculation of parasitaemia to guide treatment. In this case, if you had to pick one, then the thick film would be more useful in establishing the diagnosis. Studies have shown that for P falciparum, the most effective treatment is artesunate which is more effective than quinine without the risk of cinchonism. However, not all hospitals are currently licensed to use it in the UK (in London, only the Hospital for Tropical Diseases and Northwick Park). Artesunate is manufactured by a pharmaceutical company in China and there are doubts over the quality of the product. However, there have now been numerous studies such as the AQUAMAT study in The Lancet showing that quinine should no longer be the established treatment of choice.

Answer 792

B. Haemaglobin electrophoresis - This is beta-thalassaemia, either major or intermedia due to the findings of abdominal distension, skeletal changes and hepatosplenomegaly which is not seen in heterozygoud beta-thalassaemia trait. The pallor is highly suggestive of moderate to severe anaemia, confirmed by the test results. Beta-thalassaemia major (or Cooley anaemia) is due to a complete abscence of HbA and often presents at a few months of age with pallor and abdominal distension, both described by parents as being progressive in nature. HSM and bony abnormalities (most often the frontal and parietal bossing mentioned, as well as ‘chipmunk facies’) are often present at presentation. Intermedia has a similar presentation but in a toddler or older child, with less pronounced symptoms and a more insidious course. Blood transfusions are required though stem cell transplant offers a hope of a cure. Hb electrophoresis will reveal minimal or no HbA and elevated HbF and HbA2.

Answer 793

B. Haemoglobin electrophoresis - Africans have higher incidence of sickle cell anaemia. This is a presentation of bone pain. About 8% of black people carry the gene and the prevalence is high in sub-Saharan Africa. Sickling occurs when RBCs containing HbS become distorted into a crescent shape. Patients with sickle cell anaemia have no HbA at all. If both parents carry the sickle cell gene, there is a 1 in 4 chance of giving birth to a child with sickle cell anaemia. Sickle cell disease also includes other conditions such as HbS from one parent with another abnormal Hb or beta thalassaemia from the other parent such as HbS-Beta thal and HbSC.

Answer 794

C. Upper GI endoscopy - This patient is anaemic due to a bleeding peptic ulcer which has resulted from prolonged NSAID use. Dyspnoea is a symptom here and the ankle oedema could be due to high output heart failure. The most specific and sensitive test is an upper GI endoscopy. Duodenal ulcers rarely undergo malignant transformation so do not require a compulsory biopsy but gastric ulcers require biopsies to rule this out. Management is aimed at correcting the underlying cause such as discontinuing NSAIDs in this case. H. pylori eradication should be started if the organism is present with triple therapy. Otherwise, a PPI is indicated.

Answer 795

D. Colonoscopy - Anaemia in this case is a sinister sign which could point to possible malignancy, particularly given the patient’s advanced age. There is GI bleeding here, presumed to be lower GI, which needs to be investigated with a colonoscopy.

Answer 796

E. Anti-gliadin antibodies - This man is being investigated for a microcytic IDA here. The cause is not dietary iron intake given the history. So this could still be due to inadequate absorption such as coeliac disease or loss from GI bleeding. The unremarkable endoscopy results and barium studies indicate the absence of GI bleeding. This leaves coeliac disease, or potentially idiopathic, but the next thing to do here is to investigate the possibility of coeliac disease. Coeliac most common presents with IDA, although it can also lead to a macrocytic anaemia with mainly folate deficiency (though B12 is also affected but hepatic stores last several years). It is an autoimmune condition triggered by gluten peptides found in wheat, rye and barley. The ultimate best test is duodenal biopsy and histology to show intra-epithelial lymphocytes, villous atrophy and crypt hyperplasia. Macroscopic changes may be present but endoscopy is generally unhelpful, as in this case. The test of choice before performing such an invasive confirmatory test is to look for elevated anti-gliadin antibodies. Anti-tissue transglutaminase is less accurate and endomysial antibody is more expensive and has lower sensitivity.

Answer 797

E. Abdominal ultrasound - This is clubbing due to cirrhosis as a result of chronic alcohol excess. Ultrasound here is the only feasible option on the list and can detect advanced cirrhosis and possible signs of portal hypertension, such as ascites, splenomegaly and increased portal vein diameter. The ultrasound may show a nodular liver surface, the liver may itself be shrunk due to cirrhosis and the left hand side of the liver may be hypertrophied. USS findings with a good clinical suspicion is enough for diagnosis.

Answer 798

G. Chest x-ray - Idiopathic pulmonary fibrosis (previously known as Cryptogenic fibrosing alveolitis) progresses over several years and is characterised by pulmonary scar tissue formation and dyspnoea. Patients complain of a non-productive cough and typically reproducible and predictable SOB on exertion. This man’s work means he comes into contact with small organic or inorganic dust particles which is thought to be implicated in the cascade of events leading to IPF. Another risk factor is cigarette smoking which significantly increases the risk of IPF. The mean age of diagnosis is 60-70. CXR here will show reticular opacities. A high resolution CT scan can also be done if it was an option on this list.

Answer 799

B. Echocardiography - The only cardiac investigation here is an echocardiogram. Cardiovascular causes of clubbing include cyanotic congenital heart disease, infective endocarditis and atrial myxoma. This could well be Eisenmenger’s syndrome caused by shunt reversal.

Answer 800

F. Bronchoscopy - The history of smoking and weight loss point to a bronchial carcinoma. Whilst the initial investigation is with a CXR, diagnosis relies on pathological confirmation from a tissue sample, often obtained from bronchoscopy. Non-small cell lung cancer is more often associated with clubbing.

Answer 801

H. Polycystic kidney disease - This sounds like autosomal dominant polycystic kidney disease. There may like in this case be a FH of PKD or ESRF or cerebrovascular events (intracranial berry aneurysms in the circle of Willis and subsequent SAH). Patients may have haematuria (which can be gross), palpable kidneys and symptoms of a UTI which is common in those with the condition. Hypertension and flank pain are also commonly seen. Hepatosplenomegaly may also be found. A renal ultrasound is the first test to order when the diagnosis is suspected. If the ultrasound is equivocal, a CT scan can be done of the abdomen and pelvis.

Answer 802

J. Obstructive uropathy - This is obstructive uropathy caused by a block in urinary flow by the cervical carcinoma affecting the urinary tract. The history here is highly suggestive of acute retention with pain, distension and the dull percussion note of a full bladder. The patient also has been unable to pass urine for many hours. The diagnosis should already be obvious from this. Other more common conditions which often can give rise to an obstructive uropathy are stones and BPH. Treatment is initially directed at relieving pressure on the kidneys to prevent nephropathy and irreversible damage. This may involve insertion of a catheter, stent or nephrostomy tube, depending on what has caused the obstruction and where it is. Further treatment will be aimed at the cause.

Answer 803

F. Acute interstitial nephritis - Acute interstitial nephritis classically presents with acute renal failure associated with oliguria and the ‘hypersensitivity triad’ of rash, fever and eosinophilia triggered by a drug. This can commonly be antibiotics, especially beta-lactams, and NSAIDs, though the range of triggering medications is vast. Oliguria can be present in more severe cases. There is inflammation of the renal interstitium, as suggested by the name, and this is likely a hypersensitivity reaction. It can also occur in the setting of a chronic inflammatory disease instead of being drug triggered. It will usually resolve once you stop the offending drug and treatment is supportive, though corticosteroids can be given to dampen the reaction. Most patients recover but have some residual impairment.

Answer 804

E. Shock - In shock there is inadequate organ perfusion and when this includes the kidneys you get renal hypoperfusion (evidenced by this man’s oliguria) which can progress to acute renal failure. The reason here is hypovolaemia due to loss of intravascular volume from this man’s haematemesis. The cool peripheries (hands and feet) indicate poor peripheral perfusion. There is low BP and reflex tachycardia here too. Treatment here will be with volume replacement with IV saline and to treat the underlying cause, ideally in ICU. Do you know some causes of haematemesis?

Answer 805

G. Pre-eclampsia - This is a syndrome characterised by new onset hypertension and proteinuria in pregnant women after 20 weeks gestation. The only definitive treament is to deliver the placenta and a risk-benefit to the mother and baby should be carried out to determine the best clinical step. The doctor starts her on an antihypertensive here so while the question does not directly tell you she is hypertensive, this can be inferred. If the patient doesn’t have either hypertension or proteinuria, then they do not have pre-eclampsia. The woman may be asymptomatic, or like this case, may present with symptoms indicating a more severe disease such as a headache which is usually frontal and upper abdominal pain usually in the RUQ (a clinical symptom of HELLP syndrome which is a subtype of severe disease characterised by Haemolysis, ELevated liver enzymes and Low Platelets). Oedema is very common but quite non-specific as a presentation. Oliguria may be present and again is a sign that the pre-eclampsia is severe. The raised creatinine indicates underlying renal impairment and renal failure can be a rare complication, often occuring as ATN with either sepsis or placental abruption.

Answer 806

L. ECG - This patient’s crushing central chest pain sounds like an MI. Chest pain is classically severe and heavy in nature, located centrally with possible radiation to the left arm or jaw and lasts for >20 minutes. SOB due to pulmonary congestion and sweating due to high sympathetic output are common symptoms. Risk factors incorporate the standard set of cardiovascular risks such as smoking, high BP, DM, obesity and dyslipidaemia. An ECG is indicated. If ECG shows STEMI, new LBBB or confirmed posterior MI then PCI/thrombolysis is indicated. It is worth noting that RV infarction is present in 40% of inferior infarcts so if ST elevation is seen in II, III and aVF, right sided ECG leads should be obtained.

Answer 807

J. Transthoracic echo - This patient has likely HOCM. The patient’s young age makes it unlikely to be atherosclerotic coronary artery disease (unstable angina). HOCM is the most likely cardiomyopathy and the most frequent cause of sudden cardiac death in younger people. The FH of sudden death is suggestive of this diagnosis. This has a benign prognosis is most people though symptomatic patients are treated medically with beta blockade, CCBs or disopyramide. Chest pain on exertion (playing squash) is a common presentation in those who are symptomatic, as is dyspnoea on exertion, palpitations (such as due to AF) and a history of either pre-syncope or syncope (due to LV outflow obstruction). Inheritance is autosomal dominant with a variable penetrance. Examination findings may be normal or may reveal an ejection systolic murmur which is positionally responsive and a double carotid or apex pulsation due to the transient interruption of CO. A fourth heart sound may also be heard due to hypertrophy. Echocardiography must be performed to establish a diagnosis, though ECG and CXR will also be done and may also show changes. Echo will show septal hypertrophy. There may also be MR.

Answer 808

F. CT scan abdomen - This patient has acute pancreatitis. He has vomited and is describing mid-epigastric pain radiating around to the back which is relieved in the fetal position and is worse with movement. He is an alcoholic and alcoholic pancreatitis is seen more frequently in men usually after an average of 4-8 years of alcohol intake. Binge drinking also increases the risk. This patient also has nausea and may describe vomiting too, with agitation and confusion. The examination findings described here allude to a pleural effusion which is seen in half of patients with acute pancreatitis. Complicated haemorrhagic pancreatitis may exhibit Cullen’s sign, Grey-Turner’s sign and Fox’s sign. Make sure you know what these are and you are familiar with the causes of acute pancreatitis. Those caused by hypocalcaemia may display Chvostek’s sign and Trousseau’s sign. Key to diagnosis is serum amylase or lipase levels which are massively elevated. Prognostic criteria are outlined in Ranson’s criteria applied on admission and after 48 hours, or the modified Glasgow score which you can find in your Oxford Handbook. An abdominal CT is however the most sensitive and specific study and findings may include enlargement of the pancreas with irregular contours, necrosis, pseudocysts and peripancreatic fat obliteration. For interest, urinary trypsinogen-2 is now considered a better screening test than amylase but is not currently clinically used.

Answer 809

D. Caecal carcinoma - The weight loss, fatigue and RIF discomfort point to caecal carcinoma. Right sided colorectal cancer tends to present with anaemic symptoms. Almost 90% are anaemic at diagnosis.

Answer 810

E. Acute bowel ischaemia - Untreated AF can lead to a thrombus forming inside the heart which can then embolise like this case to the mesenteric vasculature. This person is also old, which is an additional risk due to comorbidities like atherosclerosis. This patient has the symptoms and signs of ischaemic bowel disease (which encompasses acute mesenteric ischaemia, chronic mesenteric ischaemia and colonic ischaemia). This is likely acute mesenteric ischaemia – something that in a person who presents like this, you should maintain a high index of suspicion for as the presentation can be quite non-specific but the condition can be deadly. You would likely in this case opt for surgical intervention without delay although you can cnsider some form of imaging.

Answer 811

H. Rectal carcinoma - This a rectal carcinoma. Tenesmus, blood PR alongside weight loss are all highly suggestive. Treatment of rectal carcinoma involves surgical excision where possible. This can either be an anterior resection (tumours in the upper 1/3 of the rectum) or an abdominoperineal resection (if the tumour lies lower down). APER involves the formation of a permanent colostomy and has a high incidence of sexual and urinary dysfunction. Anterior resection involves a colo-anal anastamosis

Answer 812

I. Diverticular disease - Symptomatic diverticulitis presents with fever, high WCC and LLQ pain. There may uncommonly be rectal bleeding which is usually painless, profuse and arterial in nature. Risk factors for diverticular disease include low dietary fibre and advanced age. Oral antibiotic therapy and analgesia is indicated. If there is no improvement in 72 hours after oral antibiotics then IV antibiotics are indicated. Make sure you understand the differences in the terms: diverticulosis, diverticulitis and diverticular disease.

Answer 813

H. Paracetamol - A tension headache is commonly triggered by stress and mental tension (also, fatigure and missing meals), hence the name. It is more common in females and those in middle age, and there is a link with lower socioeconomic status, although this does not necessarily represent causation. Symptoms include a dull, non-pulsatile and constricting bilateral pain, which is often described as a band across the patient’s head. It is not severe or disabling but classically worsens as the day progresses. This headache normally responds well to simple analgesics.

Answer 814

F. Re-breathing - A headache is a common presentation of someone suffering from an anxiety disorder. She is clearly hyperventilating here which is causing a respiratory alkalosis and accounting for her subjective experience of SOB. Paraesthesia is also seen in panic attacks with hyperventilation, associated with carpopedal spasm. Tell the patient to slow the breathing but breathing into a bag will help to raise PCO2. If it is an emergency setting you can consider using benzodiazepines. Long term treatment should be aimed at treating the anxiety disorder with for example CBT.

Answer 815

A. Intravenous antibiotics - Obviously meningitis and likely meningococcal due to the petechial rash. You all got that bit. Right? Now CT head should be considered before LP if there is any evidence of raised ICP, which we can presume this person here has. An LP will confirm the diagnosis with bacterial meningitis showing a low CSF glucose, elevated CSF protein and positive CSF culture/gram stain or meningococcal antigen. BUT this is clearly a very severe case and an emergency and LP here is going to be delayed. You should start this person on IV broad spectrum antibiotics and consider getting blood culture results.

Answer 816

D. CT head - This question is alluding to lung cancer which has metastasized to the brain and is causing the headache and seizure. The best option from the list is a head CT.

Answer 817

K. Aspirin - Migraine is a chronic condition, with genetic determinants, which usually presents in early to mid life. The typical migraine aura this patient describes (which can be visual, sensory or speech symptoms) which can occur during or before the headache, is pathognomic, but is not seen in the majority of patients. The aura can be positive phenomena (for example this patient seeing flashing lights) or negative phenomena (for example visual loss). Nausea, photophobia and disability (the headache gets in the way with the patient’s ability to function) accompanying a headache also suggest a migraine diagnosis. The headache of a migraine tends to be prolonged if untreated, and tends to be unilateral and pounding (but does not have to be). Tests aim to rule out other differentials, although if the history is compatible and neurological examination is unremarkable, further testing is not needed. Treatment of this chronic condition aims at treating acute attacks to restore function. Triptans can be used in specialist care for severe symptoms. These are 5HT1 agonists. Effective initial treatment in a primary care setting can involve NSAIDs, which are available OTC and include aspirin, as well as indometacin and naproxen. Paracetamol is less effective than NSAIDs but is still better than placebo – it is first line in those who are pregnant. Treatment should be taken as soon as a patient realises they are having an attack and may need to be repeated after the attack. A few patients who have frequent, severe or disabling headaches may require daily prophylaxis such as anticonvulsants, TCAs and beta blockers.

Answer 818

G. Lumbar puncture - SAH (bleeding into the subarachnoid space) presents with sudden severe headache patients will often describe as the worst headache of their life, and can often be so bad that they feel like they’ve been kicked in the back of the back. Half of all patients lose consciousness and eye pain with exposure to light can also be seen. Altered mental status is common. SAH occurs most commonly in the 50-55 age group and affects women and black people more than men and white people. The most common cause of non-traumatic SAH is an aneurysm which ruptures. Conditions which predispose to aneurysm formation and SAH include adult PKD, Marfan’s, NF1 and Ehlers-Danlos. Cerebral aneurysms arise around the circle of Willis. A CT scan is indicated, and if unrevealing, this should be followed by an LP. Cerebral angiography can confirm the presence of aneurysms. The patient should be stabilised and this followed by surgical clipping or endovascular coil embolisation, the choice is subject to much current controversy sparked by relatively recent research. Complications can commonly occur and include rebleeding, hydrocephalus and vasospasm.

Answer 819

C. Hydronephrosis - This patient has BPH which has caused hydronephrosis. This is an example of bilateral obstructive uropathy. Acute presentations are often painful whereas chronic presentations are more insidious in onset. Blockage of urinary flow by the enlarged prostate has led to urinary retention and overflow incontinence. Initial treatment aims to relieve the pressure on the kidneys. This involves catheterisation as the first line treatment. The patient should be started on alpha blockers at the time of catheterisation.

Answer 820

E. Acute prostitis - Acute prostatitis is the most frequently made urological diagnosis in men under 50. It is commonly caused by E. coli and can cause lower abdominal, perineal, rectal and ejaculatory pain. It is associated with the presence of a UTI and BPH. As a result there may be dysuria and frequency. The prostate gland may feel warm and boggy to touch and DRE will reveal an intensely tender gland.This patient does not appear septic and first line treatment is with an oral quinolone such as ciprofloxacin for 10 days with an NSAID for pain relief. A prolonged course of antibiotics is needed if chronic prostatitis follows the acute occurence.

Answer 821

F. Renal function tests - ACE inhibitors inhibit the conversion of angiotensin I to angiotensin II and are used for a range of conditions such as heart failure, hypertension and diabetic nephropathy. Renal monitoring is needed due to hyperkalaemia and side effects of ACE inhibitors being more common in impaired renal function, necessitating a reduction in dose. They can also cause impaired renal function, particularly in old people. Using it alongside NSAIDs increases this risk and using it with potassium sparing diuretics increases the risk of hyperkalaemia. In those with renal artery stenosis, ACE inhibitors can also reduce or even remove glomerular filtration and result in renal failure and are therefore not recommended in these patients.

Answer 822

D. Liver function tests - Pravastatin is a statin. Other statins include the mighty atorvastatin, simvastatin and rosuvastatin. They inhibit HMG-CoA reductase, which is an enzyme involved in the cholesterol synthesis pathway. They hence lower LDL cholesterol levels very effectively to reduce the cardiovascular disease. Statins can cause altered LFTs and rarely even hepatitis and jaundice. There have also been rare case reports of liver failure. NICE have a guideline (which we must listen to...) which state that liver enzymes should be measured before treatment and repeated within 3 months and at 12 months of starting treatment (unless there are obvious signs and symptoms of hepatotoxicity).

Answer 823

A. Cyclophosphamide - This drug is used mainly with other drugs for treating malignancies including leukaemias, lymphomas and solid tumours. The rare and serious complication is haemorrhagic cystitis as a result of the urinary metabolite acrolein and mesna can be given as prophylaxis. It is an alkylating agent which damages DNA and interferes with cell replication. The only feasible option on this list is WCC where we have to assume this drug is being used for a leukaemia or lymphoma and we want to measure whether it is working.

Answer 824

H. INR -Warfarin needs to be monitored with INR, at first daily or on alternate days then at longer intervals (depending on patient response). INR is the international normalised ratio, which is the prothrombin time as a value which is standardised to take into account different lab techniques etc. The higher the INR, the more anticoagulated a patient is. As a rough guide, the aim is 2.5 for DVT/PE, AF, DCM and MI and 3.5 for recurrent DVT/PE.

Answer 825

K. Serum drug level - Phenytoin is useful for tonic-clonic and focal seizures but it has a narrow therapeutic index and a non-linear dose-plasma concentration relationship. Hence, small dose increases can lead to huge increases in plasma concentration and acute toxicity. Conversely if the patient skips a dose, there may also be marked change and the levels may be sub-therapeutic. You therefore need to monitor plasma drug concentration to improve the adjustment of dose. Parenterally, you can also consider fosphenytoin.

Answer 826

B. Graves disease - This woman has symptoms of hyperthyroidism (weight loss and palpitations) and a goitre. In countries where sufficient iodine intake is not an issue, Graves’ disease is the most common cause of hyperthyroidism. Graves’ gives a diffuse goitre. Peripheral manifestations such as ophthalmopathy, pretibial myxoedema and hyperthyroid acropachy do not occur with other causes of hyperthyroidism. Ophthalmopathy includes lid retraction, exophthalmos and eye movement restriction leading to diplopia. Acropachy is an uncommon manifestation presenting as clubbing with soft tissue swelling. Pretibial myxoedema is almost always associated with ophthalmopathy. Treatment aims to normalise thyroid function and is achieved by radioactive iodine, antithyroid medications or with surgery. They are all effective and relatively safe options. Symptomatic therapy is given with beta blockers such as propranolol.

Answer 827

G. De Quervain's thyroiditis - This girl has de Quervain’s thyroiditis (which can also be called subacute granulomatous thyroiditis) which is inflammation of the thyroid characterised by a triphasic course where there is transient thyrotoxicosis followed by a hypothyroid phase before a return to euthyroidism. The thyrotoxic phase (symptoms of hyperthyroidism may be present) is characterised by pain and tenderness of the thyroid, which tends to be larger, firm and tender to touch. This girl also has a fever and neck pain which is making it painful for her to swallow. This is a self-limiting condition and no specific treatment is needed though NSAIDs and beta blockers can be used for symptomatic relief. Roughly 30-40% describe a prior viral infection.

Answer 828

C. Hodgkin's disease - This is a case of lymphoma. Reed-Sternberg cells are binucleate cells characteristically seen in Hodgkin’s lymphoma. Hodgkin’s is localised to a single group of nodes (normally the cervical and/or supraclavicular) and extranodal involvement is rare. Mediastinal involvement is common. Spread is contiguous and B symptoms may be present such as a low grade fever, weight loss and night sweats. Pruritis may be found in approximately 10% of cases but has no prognostic significance. 50% of cases is associated with EBV infection and distribution is bimodal with peaks in young and old. There is classically pain in lymph nodes on alcohol consumption.

Answer 829

F. SUperior vena cava syndrome - SVC syndrome occurs due to SVCO. The most common cause of this is malignancy (this smoker probably has lung cancer which is the most likely cause in those >50). There are also benign causes such as iatrogenically due to pacemaker leads and central venous catheters causing SVC thrombosis. Common symptoms are facial swelling and arm swelling as seen, as well as dyspnoea, cough and facial plethora. There may also be a headache, chest pain, blurry vision and stridor. These symptoms tend to be worse on bending forwards or lying down. The most useful imaging test is a chest CT with IV contrast which establishes the diagnosis and also shows the exact location of the pathology. A CXR in SVCO can show a widened mediastinum or the lung cancer which is the cause.

Answer 830

E. Pancreatic carcinoma - The presence of Virchow’s node (Troisier’s sign), a hard enlarged node in the left supraclavicular fossa, points towards a malignancy in the abdominal cavity. This is most often stomach cancer but does not have to be. The lymph drainage of the abdominal cavity drains into Virchow’s node as the lymph drains most of the body from the thoracic duct and enters the venous circulation at the left subclavian vein.

Answer 831

G. Perform coronary angioplasty - For confirmed ST elevation MI the 1st line treatment which gives the best results is primary PCI with stenting and is indicated if the person presents to A&E within 90 minutes of first presentation. CABG should be strongly considered if the patient fails PCI and should be done within 12 hours of onset of symptoms, ideally within 6. This is London’s best hospital but if this were Orkney and they did not have PCI capacity or the ability to transfer to a PCI facility within 30 minutes or so, then you would thrombolyse if there are no contraindications. This must be done within 12 hours of symptom onset and ideally within 3 hours as the efficacy of fibrinolytics diminishes over time. You should be aware of the absolute contraindications to thrombolysis such as suspected aortic dissection and prior intracranial haemorrhage. This patient has already been given some treatment for suspected MI (aspirin, oxygen, morphine and GTN). Post-treatment this patient will need aspirin therapy and clopidogrel for at least a year – aspirin should be continued indefinitely. Patients should also be started on a beta blocker, ACE inhibitor and a statin, indefinitely.

Answer 832

K. Add low molecular weight heparin - The mainstay of treatment for acute DVT is anticoagulation. This can be either unfractionated heparin, a LMWH or a factor Xa inhitor like fondaparinux. Fondaparinux has a higher half life than LMWH and there is no effective way of reversing it. LMWH have a shorter half life and some of it can be removed with protamine. Heparin though can be reversed quickly with protamine. Hence, if the patient is at a high risk of bleeding, they should be treated with unfractionated heparin and you should avoid fondaparinux. If they start bleeding you can just chuck them protamine. This however requires monitoring of APTT and platelet counts. If the patient has heparin-induced thrombocytopenia, you can try using fondaparinux. LMWH is recommended in those with active cancer and preferred in pregnancy, and consideration needs to be given in those with renal impairment. In this case LMWH or unfractionated heparin can be given, and the only option on the list is to add LMWH (such as enoxaparin, tinzaparin and dalteparin). Warfarin is started on the same day and LMWH should be used for at least 5 days until the INR is in the therapeutic range of 2-3 before the LMWH can be discontinued. (Some people say the INR has to be in that range for 48 hours before you remove LMWH)

Answer 833

D. Perform echocardiography - She has a murmur so you would want to do an echocardiogram. Doppler echo is best for diagnosis and evaluation of aortic stenosis and is highly sensitive and specific. It will show an elevated aortic pressure gradient and also allow you to quantify LV ejection function and measure the area of the valve.

Answer 834

B. Perform exercise ECG - This patient has presented with stable angina. Resting ECG is often normal however during exercise stress ECG (most often the Bruce Protocol) there will be ST segment depression during exercise indicative of ischaemia. Those unable to exercise to an adequate level may need stress myocardial perfusion imaging or stress echocardiography. 1st line treatment involves lifestyle changes and antiplatelet therapy with aspirin. Anti-anginal theray will also be given, first line being beta-blockade. Statin therapy, blood sugar control in diabetics and BP control with antihypertensives may also be necessary. Those with LMS disease, 3 vessel disease or a reduced EF may benefit from CABG. Single vessel disease may benefit from PCI.

Answer 835

L. Gastroscopy - Weight loss is one of the most common presenting symptoms in patients with gastric cancer. Epigastric pain is present in about 80% and may resemble that of a gastric ulcer. Although commonly mentioned in EMQs, lymphadenopathy is an uncommon presentation. More proximal tumours can also present with dysphagia. Strong risk factors include pernicious anaemia, Helicobacter pylori and the consumption of N-nitroso compounds found in cured meats. The peak incidence occurs between 50-70 and men are twice as likely to have gastric cancer. Most are adenocarcinomas. The first test to order for suspected gastric malignancy is an urgent upper GI endoscopy with biopsy of the lesion. The mainstay of treatment is surgical resection unless there is evidence of metastatic disease.

Answer 836

K. History only - Depression is characterised by low mood, loss of interest and reduced energy persistently over a long period of time. It is a common condition and there is an obvious recent stress here as his wife has died. The diagnosis is clinical even though there are screening tools used in a primary care setting like the PHQ-9 questionnaire. The diagnostic criteria follows DSM-IV-TR which classifies depression into major, minor and dysthymia. Patients tend to respond well to CBT, antidepressants or both. Suicidal ideation should be assessed for. There is a high lifetime chance of recurrence.

Answer 837

H. Fasting blood glucose - T2DM can present with yeast, skin and urinary tract infections on top of unintentional weight loss and fatigue. The pins and needles in her legs is a result of diabetic peripheral sensory neuropathy. This is a microvascular complication of DM and is characterised by peripheral nerve dysfunction. There tends to be loss of sensation typically occuring in a symmetrical ‘glove and stocking’ distribution. Patient’s may also describe a pain or paraesthesia. Examination should include peripheral pulses, reflexes and sensation to light touch, vibration (128Hz tuning fork), pinprick and proprioception. Any pain can be treated with medications like gabapentin. Symptomatic patients need a single random blood glucose of >11.1 or single fasting glucose of >7. Asymptomatic patients need two separate elevated readings for a diagnosis. Alternatively if there are borderline results, an OGTT can be conducted to see if plasma glucose is raised >11.1 two hours after an oral glucose load of 75g. A patient is said to have impaired fasting glucose if fasting glucose falls between 6.1-6.9. Impaired glucose tolerance is present if plasma glucose 2 hours after oral glucose load in OGTT falls between 7.8-11.0.

Answer 838

D. Colonoscopy - This lady needs to be investigated for bowel cancer. There are symptoms and confirmation of IDA which could be due here to GI bleeding. There is also diarrhoea which has not improved and is now chronically a problem. Along with advanced age, this raises the suspicion of malignancy and a colonoscopy needs to be done.

Answer 839

I. Chest x-ray -Sarcoidosis is a chronic multisystem disease with an unknown aetiology with the lungs most commonly affected (giving SOB in this case). The purple lesions are erythema nodosum and are tender erythematous nodules. Lupus pernio is another typical skin manifestation of sarcoidosis presenting with indurated plaques with discoloration on the face. CXR will typically show bilateral hilar lymphadenopathy and CXR findings are used in the staging of disease. Additionally, serum calcium and ACE levels may be raised. A transbronchial biopsy is essential for diagnosis in most cases and shows the presence of non-caseating granulomas. Black people have a higher lifetime risk of sarcoidosis, as do those of Scandinavian origin. The mainstay of treatment for severe disease involves systemic corticosteroids.

Answer 840

J. Bone marrow aspirate - This patient has multiple myeloma. This is characteristed by clonal proliferation of plasma cells in BM and commonly presents with bony pain and symptoms of anaemia (which this patient’s Hb demonstrates). The elevated ESR is also suggestive and hypercalcaemia is present in 30%. The diagnostic test is serum or urine electrophoresis looking for a paraprotein spike of IgG or IgA and light chain urinary excretion (Bence Jones proteins). Bone marrow examination and skeletal survey will also need to be conducted. Bone marrow aspirate in this case and biopsy will show plasma cell infiltration in the bone marrow, and can help to differentiate multiple myeloma from MGUS and solitary plasmacytoma. Bone changes include osteopaenia, osteolytic lesions and fractures.

Answer 841

D. Extrinsic allergic alveolitis - This is EAA, a hypersensitivity pneumonitis. There is history here of exposure to organic dust (avian proteins) with birds. There are a variety of syndromes this could be – pidgeon breeder’s lung, bird fancier’s lung and budgerigar fancier’s disease. History and examination findings depend on whether the EAA is acute, subacute or chronic. Chronic presents like idiopathic pulmonary fibrosis and there may be clubbing. The most important treatment element is to avoid antigen exposure.

Answer 842

C. Acute left ventricular failure - You should know the distinction between LVF and RVF. RVF leads to a backlog of blood and congestion of the systemic capillaries. This causes peripheral oedema and ascites and hepatomegaly may develop. Nocturia may be a symptom as fluid returns from the legs when the patient lies down flat. LVF causes congestion in the pulmonary circulation so the symptoms are respiratory. As seen in this patient, there is SOB and the frothy cough suggestive of pulmonary oedema. In a hospital setting, patients need to be sat upright to improve the SOB and IV access needs to be established. Oxygen, morphine, diuretics (frusemide or another loop diuretic) and nitrates will be given. Once stable, medical treatment of heart failure should be started which involves in the first instance, an ACE inhibitor followed by beta blockade.

Answer 843

E. Pneumonia - This is pneumonia, which has not resolved and gotten worse to cause this man’s confusion. In elderly people, pneumonia can present anyway with atypical symptoms such as confusion and general deterioration. There is also a small chance of metastic spread to cause meningitis. This patient needs to have his CURB-65 score calculated and his pneumonia needs treating. He already scores a point for age and a point for confusion, and likely needs admission, ITU and IV antibiotics with oxygen and ventilation.

Answer 844

G. COPD - Secondary pulmonary hypertension as a result of this patient’s advanced COPD or cor pulmonale has caused peripheral oedema. Cor pulmonale is right heart failure secondary in this case to long standing COPD, caused by chronic hypoxia and pulmonary vascular vasoconstriction secondary to this, giving pulmonary hypertension and right sided heart failure. Signs aside from lower extremity oedema include hepatomegaly, a loud P2 and engorged neck veins. Given he is on inhalers and LTOT already, he has already been diagnosed with COPD but we are seeing if you know that this is what the patient has. If this is a new symptom he needs to have this assessed and may need additional intervention such as diuretics.

Answer 845

B. Opiate intoxication - This suggests opiate OD. Signs include CNS depression, miosis and apnoea. With cocaine you would not find small constricted pupils – they would be dilated. Naloxone is indicated both therapeutically and diagnostically. If there is a response, then it is diagnostic. Another diagnosis should be sought if the patient is unresponsive. IV is the preferred route of administration although naloxone can be given IM or SC if IV access cannot be established. Ventilatory support is key with 100% oxygen. You can check out Toxbase for a full database on poisons and treatments.

Answer 846

B. Aflatoxin B1 - Aflatoxin B1 is a naturally occuring carcinogen produced by many species of Aspergillus (a fungus). It thrives on improperly stored grains and nuts. It is considered a significant etiological factor for liver cancer in Southern Africa and the Far East.

Answer 847

D. Asbestos - Mesothelioma is a malignant tumour of the pleura (or less commonly the peritoneum). There are three main types of asbestos: white, blue and brown, with blue being the most potent cause. Asbestos exposure increases the risk of both squamous cell carcinoma and mesothelioma. A 20-year lag time between exposure and development of mesothelioma is typical.

Answer 848

F. Cadmium - Studies have shown that men in certain occupations, with higher levels of exposure to cadmium, have a higher risk of prostate cancer. It is thought that cadmium interacts with certain hormone receptors and mimics the effects of oestrogen and testosterone, thus abnormally stimulating the growth of the prostate. Furthermore cadmium is thought to interfere with the body’s ability to absorb zinc (which is believed to have a protective effect against prostate cancer.)

Answer 849

A. Aniline dyes - Contact with aniline dyes (not azo dyes) is associated with bladder cancer

Answer 850

G. Oestrogen - Oestrogen stimulates endometrial proliferation, with an increased risk of endometrial carcinoma. When progesterone is given with oestrogen, there is a decreased incidence of cancer. Remember: never give a woman with a uterus oestrogen alone!

Answer 851

D. Wilm's tumour - Wilms' tumour (a.k.a. nephroblastoma) is the commonest intra-abdominal tumour in children, rarely occuring in adults. It is a malignant tumour derived from embryonic mesodermal tissues. Patients present with a loin mass, weight loss, anorexia and fever. Although the tumour is rapidly growing an behaves aggressively, it has an 80% survival rate at 5 years.

Answer 852

C. Kaposi's sarcoma - Kaposi's sarcoma is a malignant tumour of the vascular endothelium consisting of spindle cells and small blood vessels. It is very rare in patients without HIV infection or those who are immunosuppressed. It gives rise to multiple lesions in the skin that appear like purple bruises and are not painful to touch. In about 40% of cases (such as this one) there is gastrointestinal involvement.

Answer 853

E. Pancoast tumour - Pancoast tumours are lung tumours located at the apex of the lung. They typically extend to involve the sympathetic ganglion resulting in ipsilateral Horner's syndrome (ptosis, meiosis, anhydrosis). In some cases, the lower roots of the brachial plexus are also involved, resulting in pain and weakness in the muscles of the arm and hand, specifically in the T1 distribution.

Answer 854

A. Burkitt's lymphoma - Burkitt's lymhpoma is a high-grade B-cell lymphoma endemic in some parts of Africa. It frequently involves the jaw and is most often seen in African children with previous EBV infection (there is a strong association between the two) Other associations include chronic malarial infection and translocation of chromosome 8 on the c-myc gene with chromosome 14.

Answer 855

G. Brodie's tumour - Brodie's tumour (a.k.a. phyllodes tumour) are rare tumours of the fibroepithelial stroma of the breast. The history of a rapidly growing mass that distorts the shape of the breast points towards this disease. Most of these tumours are benign and the prognosis after surgery is excellent.

Answer 856

A. CA 15-3 - High Ca 15-3 levels suggest metastatic breast disease. However, it has poor sensitivity and is therefore not used as a screening procedure to detect breast carcinoma. This tumour marker has recently been superseded by Ca 27-29, which is claimed to be more sensitive and specific. However, CA 27-29 lacks predictive value in the earliest stages of breast cancer and thus has no role in screening for or diagnosing the malignancy.

Answer 857

D. CA 19-9 - Ca 19-9 is used to confirm the diagnosis of malignant pancreatic tumours, differentiating them from chronic pancreatitis. However, it should not be used as a stand-alone diagnostic tool, rather as an adjunct to CT, U/S and ERCP. It is also used for monitoring the response to therapy. Levels can also be elevated in hepatbiliary disease, but it is not used in this setting.

Answer 858

E. Prostate-specific antigen - This patient's history is suggestive of metastatic prostate cancer. Prostate-specific antigen (PSA) is used as a screening test, a diagnostic test, a measure of treatment success and a marker of recurrence. It has replaced the use of prostatic acid phosphatate (PAP). However, PSA lacks the required specificity as it is raised in patients without prostatic cancer (i.e. a high false positive rate). Therefore, it should be used alongside other methods of investigation, and not by itself, to make a diagnosis.

Answer 859

F. Carcinoembryonic antigen - Although carcinoembryonic antigen (CEA) lacks the sensitivity and specificity to be a diagnostic test for colorectal cancer, it has found a valuable application in the detection of recurrence of malignant disease following treatment.

Answer 860

G. α-Fetoprotein - This patient is likely to have hepatocellular carcinoma (HCC) on a background of chronic hepatitis C infection. α-Fetoprotein (AFP) is one of the most widely used tumour markers for HCC, but may also be raised in patients with germ cell tumours, cirrhosis and hepatitis.

Answer 861

H. Superior vena caval obstruction - This is a classic description of SVC obstruction. This occurs when the SVC is subjected to pressure from a tumour in the superior mediastinum or from thrombosis. The most common cause is lymph node metastases from bronchial carcinoma. The main clinical features are due to venous congestion, and there may even be stridor due to obstruction of the trachea or main bronchi. Initial treatment is with oral dexamethasone to reduce oedema around the tumour followed by long-term radiotherapy/chemotherarpy.

Answer 862

F. Spinal cord compression - Metastasis to the spine has lead to compression of this patient's spinal cord (a medical emergency). Spinal cord compression usually causes LMN signs at the level of the lesion and UMN signs below that level. If not recognised immediately and treated, the damage will become permanent.

Answer 863

A. Raised intracranial pressure - This lady's symptoms are suggestive of raised ICP secondary to a mass lesion such as a brain tumour. Actions such as coughing, sneezing and laughing make the pain worse as they all lead to temporary increases in ICP. Patients often report the headache as being worst when they wake up in the morning. This is because ICP increases during sleep, probably from vascular dilatation due to CO2 retention.

Answer 864

I. SIADH - There are many causes of SIADH, however in this case it is due to ectopic ADH secrtion from tumour cells, most commonly small cell lung carcinoma. The clinical features are due to the resulting dilutional hyponatraemia. Severe hyponatraemia may lead to convulsions, seizures, coma and even death. Characteristic the patient with SIADH will persistently excrete concentrated urine (with a higher urine osmolairity than serum), have normal renal and adrenal function and will have no oedema or hypovolaemia. When treating hyponatraemia take care not to correct the serum Na+ too quickly as this may lead to central pontine myelinolysis (as you may remember from your IBFD lectures!).

Answer 865

C. Tumour lysis syndrome - Tumour lysis syndrome usually occurs in patients undergoing chemotherapy for lymphoproliferative malignancies, who have just initiated treatment. Lysis of tumour cells leads to the release of large amounts of potassium, phosphate and uric acid into the circulation. The excess phosphate binds to calcium, leading to hypocalcaemia and its clinical features. Patients are also at risk of developing acute renal failure due to the deposition of uric acid and calcium phosphate crystals in the renal tubules.

Answer 866

A. Gastrointestinal lymphoma - There is an increased incidence of T-cell lymphoma and small bowel adenocarcinoma.

Answer 867

C. Gastric carcinoma - Patients with pernicious anemia have a 2- to 3-fold increased incidence of gastric carcinoma.

Answer 868

I. Cervical carcinoma - The incidence of cervical cancer in celibate women is next to none. Number of sexual partners is a well known risk factor for cervical cancer and it is thought to be associated with the transmission of certain human papilloma virus (HPV) types.

Answer 869

B. Bladder carcinoma - Bladder cancer was one of the first cancers shown to be industrially associated and has an important place in the history of occupational disease. Rubber industry workers who had been exposed to the substance beta-naphthylamine (banned in the 1950s) were found to have developed bladdder cancer after a latent period of 15-20 years.

Answer 870

G. Hepatocellular carcinoma - A large proportion of patients with acute hepatitis C infection develop chronic hepatitis. Of these, 3% develop hepatocellular carcinoma following the development of cirrhosis

Answer 871

F. Hodgkin's lymphoma - The presence of Reed-Sternberg cells is the giveaway here.

Answer 872

K. Chronic myeloid leukaemia - The clinical features here should alert you towards a malignant process. Massive splenomegaly is characteristic of chronic myeloid leukaemia (CML). CML occurs mainly in middle aged and elderly patients and is due to excessive proliferation of myeloid cells in the bone marrow. Up to 90% of patients have the Abelson-BCR gene (the molecular equivalent of the Philadephia chromosome you may have heard of). Patient's without the Philadelphia chromosome tend to have a worse prognosis than those with it.

Answer 873

G. Chronic lymphocytic leukaemia - Chronic lympocytic leukaemia (CLL) is characterised by neoplastic proliferation of mature B lymphocytes. The clinical manifestations are due to immunosuppression and bone marrow failure, however it usually has an insidious onset with 25% of patients diagnosed incidentally (as in this case). It occurs most commonly in the elderly and is the most common leukaemia in the Western world.

Answer 874

I. Acute myeloid leukaemia - Acute myeloid leukaema (AML) is generally seen in adulthood and is caused by proliferation of myeloid precursor cells. Investigations usually show a raised WCC, although it may be normal or low. The brusing and anaemia seen in this patient are due to the effects of marrow failure. The blood film shows blast cells and the presence of Auer rods, which are virtually pathognomonic of AML. ALL vs. AML Question; A 3 year old girl presented with several bruises over her body and tiredness. On examination she appeared plae and had petechial haemorrhages. Blood tests revealed low Hb and high WCC and a thrombocytopenia. Peripheral blood film showed the presence of blast cells. ACUTE LYMPHOBLASTIC LEUKAEMIA Why would this be ALL and not AML? Answer: Is the age not the pointer in this question. Blast cells are immature precursors of either lymphoblasts or myeloblasts. They don't normally appear in peripheral blood. When present, they signify ACUTE leukaemia. Special staining is needed to identify the lineage. So basically blast cells can occur in both ALL or AML. This is my understanding anyway....but the age thing is probably the most significant pointer. Answer: Think first of all lymphoid vs myeloid CLL: enlarged rubbery lymph nodes, non tender, also smear cells CML: Philadelphia chromosome, sweats, more likely splenomegaly, increased WCC ALL: bruising, pale, usually children btw 2-4 years old AML: Auer rods, SOB, bone pain.

Answer 875

C. Waldenstrom's macroglobulinaemia - Waldenstrom's macroglobulinaemia is a lymphoproliferative disorder of B cells, which take on a lymphoplasmacytoid appearance. It is characterised by the production of immunoglobulin M (IgM), which gives rise to the clinical features of hypervisosity (nosebleeds, blurred vision, retinal haemorrhage etc.)

Answer 876

D. Acute lymphoblastic leukaemia - Acute lymphoblastic leukaemia (ALL) is the most common leukaemia in children. The immature blast cells infiltrate the marrow and lymphoid tissue causing anaemia, bleeding and a vulnerability to infection. Although the FBC reveals that the patient is anaemic, the WCC is raised due to circulating blast cells. The investigation of choice is bone marrow aspiration (which shows a hypercellular marrow with >20% blasts) and treatment is with combination chemotherapy. ALL vs. AML Question; A 3 year old girl presented with several bruises over her body and tiredness. On examination she appeared plae and had petechial haemorrhages. Blood tests revealed low Hb and high WCC and a thrombocytopenia. Peripheral blood film showed the presence of blast cells. ACUTE LYMPHOBLASTIC LEUKAEMIA Why would this be ALL and not AML? Answer: Is the age not the pointer in this question. Blast cells are immature precursors of either lymphoblasts or myeloblasts. They don't normally appear in peripheral blood. When present, they signify ACUTE leukaemia. Special staining is needed to identify the lineage. So basically blast cells can occur in both ALL or AML. This is my understanding anyway....but the age thing is probably the most significant pointer. Answer: Think first of all lymphoid vs myeloid CLL: enlarged rubbery lymph nodes, non tender, also smear cells CML: Philadelphia chromosome, sweats, more likely splenomegaly, increased WCC ALL: bruising, pale, usually children btw 2-4 years old AML: Auer rods, SOB, bone pain.

Answer 877

Troisier's sign - Troisier's sign is the finding of a palpable solid lymph node located in the left supraclavicular fossa (known as Virchow's node). It is commonly associated with gastric malignancy. Although rarely present, there is always a mark for checking for this node in an abdo exam in your OSCEs!.

Answer 878

B. Erythrocytosis - Erythropoietin is produced in the kidney and renal cell carcinoma may lead to increased production as a paraneoplastic syndrome. This causes a a secondary polycythaemia.

Answer 879

H. Eaton-Lambert syndrome - Eaton-Lambert syndrome is a myasthenic syndrome characterised by impaired release of acetylcholine due to autoantibodies to presynaptic voltage-gated calcium channels. Typically in EMQs, the description is of a patient with absent tendon reflexes and muscle weakness that improved after repeated contraction (much to the amazement of the examining doctors colleagues!). Eaton-Lambert syndrome is associated with small cell lung carcinoma in 60% of cases.

Answer 880

F. Necrolytic migratory erythema - This rash is characteristic of glucagonoma and is also known as 'glucagonoma syndrome'. It is characterised by the spread of erythematous blisters across the lower abdomen, buttocks, perineum and groin.

Answer 881

A. Hypoglycaemia - Insulimonas present with spontaneous episodes of hypoglycaemia, especially when fasting and exercising. Most insulinomas are benign and small tumours, but may cause significant metabolic effects.

Answer 882

I. Tetany - Medullary thyroid carcinoma are uncommon and arise from the parafollicular cells of the thyroid. These tumours produce calctionin and elevated levels are diagnostic. They may occasionally present with hypocalcaemia and tetany as a result of this.

Answer 883

A. Surgery

Answer 884

J. Renal artery stenosis - Renal artery stenosis is basically narrowing of the renal artery. It occurs typically due to atherosclerosis or fibromuscular dysplasia. The history of smoking, hypertension and angina here are risk factors of the former. The presentation tends to be with accelerated or difficult to control hypertension. Acute kidney injury can be seen after starting an ACE inhibitor or an angiotensin II receptor antagonist. The afferent arteriole is stenosed in RAS and angiotensin II is needed to maintain GFR by constricting the efferent arteriole. ACE inhibitors prevent conversion of angiotensin I to angiotensin II, which is needed to maintain renal perfusion pressure in those with RAS. There may not be any clinical consequences of RAS – just because someone’s renal arteries are narrowed does not mean they are suffering worsening kidney function, although this may be the case, especially after blockade of the renin-angiotensin system. A definitive diagnosis is made on imaging, where there is some controversy on what is most appropriate to use. USS is safe and non-invasive but the sensitivity and specificity is low. CT/MR angiography has the risk of contrast nephropathy and nephrogenic systemic fibrosis. Conventional angiography (the best test available) has the risk of bleeding and emboli as well as contrast related risks already mentioned.

Answer 885

F. Conn's syndrome - The normal range for potassium 3.5-5mmol/l. You should really know the normal ranges for common values like sodium, potassium and urea by this stage. In Conn’s, potassium is normal or low. It is important when drawing blood to avoid haemolysing the sample, which will cause a falsely elevated potassium level. It is important for screening to calculate the aldosterone/renin ratio, with >30 being suggestive of Conn’s. In Conn’s, aldosterone is raised and renin is low due to negative feedback. This is in contrast to renal artery stenosis where both aldosterone and renin will be raised. It is important to discontinue diuretics and other interfering medications for at least 6 weeks prior to measuring the ratio. The most reliable diagnostic test is a fludrocortisone suppression test. Treatment can be surgical with excision of the adenoma (if aldosterone production is lateralised to one side) or medical with spironolactone and amiloride. There are also familial forms of primary hyperaldosteronism which show an autosomal dominant mode of inheritance. Spironolactone is an aldosterone receptor antagonist. Amiloride inhibits aldosterone-sensitive sodium channels. They are both examples of potassium sparing diuretics acting on the DCT.

Answer 886

L. Cushing's syndrome - There is weight gain (truncal obesity), hypertension, moon face and striae in Cushing’s due to hypercorticolism. Cushing’s disease is due to an ACTH secreting pituitary adenoma and is responsible for most cases of Cushing’s syndrome. A low dose 1mg overnight dexamethasone suppresion test can be done, or a 24 hour urinary free cortisol collection to diagnose Cushing’s syndrome. Plasma ACTH should guide further investigation. If ACTH is suppressed, the problem is likely to be with the adrenals. If it not suppressed, pituitary or ectopic disease is more likely.

Answer 887

I. Acromegaly - Acromegaly is caused by excess growth hormone and is most often due to a pituitary adenoma. The diagnosis is often made late as the symptoms are insidious in onset. This patient is putting on weight, has prognathism, large hands and feet and glycosuria. There may also be an enlarged nose, soft tissue changes and organomegaly, all as a result of excess GH/IGF-1. Visual impairment may be present is due to the pituitary adenoma putting pressure on the optic chiasm. Tumour mass effect may also cause headaches and there may also be hypopituitarism from stalk compression and CN palsies. IGT and DM are associated and this may result not only in glycosuria but possible polydipsia and polyuria. There may also be altered sexual function, Carpal Tunnel Syndrome and joint dysfunction. Treatment is either sugical with a transsphenoidal approach, or medical (if the tumour cannot be resected/completely resected) with a somatostatin analogue like octreotide and an adjunctive dopamine agonist like cabergoline. If the patient does not respond to SSAs then pegvisomant which is a GH receptor antagonist can be used, although it is very costly. Gigantism occurs as a result of excess GH during childhood.

Answer 888

M. Phaeochromocytoma - Phaeochromocytomas presents with paroxysmal episodes of palpitations, anxiety, excessive sweating, pallor and hypertension. It can be inherited in MEN2, von Hippel-Lindau syndrome and NF1. Diagnosis is based on raised urinary and serum catecholamines, metanephrines and normetanephrines. 24 hour urinary VMA will be elevated. CT is used to localise the tumour. Treatment includes medical with the use of phenoxybenzamine, phentolamine and surgical options. Surgical excision is carried out under alpha and beta blockade to protect against the release of catecholamines into circulation when the tumour is being manipulated. The 10% rule is often quoted: 10% are bilateral, 10% malignant, 10% extraadrenal and 10% hereditary.

Answer 889

F. Hereditary angio-oedema - This patient has urticaria (erythematous, blanching, oedematous, pruritic lesions) and angio-oedema (swelling). A positive family history of angio-oedema raises a suspicion for a diagnosis of hereditary angio-oedema. There are two forms of this condition. One is manifest by absence of C1 esterase inhibitor whereas the other is due to normal levels of dysfunctional C1 esterase inhibitor. This allows the uncontrolled activation of the complement cascade which therefore gives rise to angio-oedema. This is a condition which is inherited in an autosomal dominant manner although it should be noted that some 50% of cases have no previous FH and are thought to be due to new mutations. Laboratory investigations may reveal a decreased level of C1 and decreased levels or function of C1 esterase which would support the diagnosis. In acquired angio-oedema, C1q levels are low unlike in the hereditary form where it is normal – this differentiates the two forms. The mainstay of treatment is with antihistamines. Airway compromise like the stridor this patient is experiencing is an indication for prompt treatment with adrenaline. The stridor here is a sign of severe laryngeal angio-oedema, which is a sign of impending airway obstruction – this needs to be taken seriously and is an emergency.

Answer 890

C. Juvenile idiopathic arthritis - This is juvenile idiopathic arthritis, also known as juvenile rheumatoid arthritis, or Still’s disease. It is the most common chronic arthropathy of children and there are several clinical subtypes. The diagnosis is clinical. Intra-articular steroids offer good control if only a few joints are affected. Methotrexate is also a commonly used disease-modifying agent. More resistant cases are treated with agents which block inflammatory cytokines. Around 10-20% of children with JIA are at risk of developing anterior uveitis and therefore all children with this diagnosis must undergo regular ophthalmological review for inflammation. Remember that symptoms vary according to subtype of disease, which laboratory tests may be useful in classifying. Note also that while this can be called juvenile RA, rheumatoid factor is only positive in a small minority of patients (2-7%).

Answer 891

J. Acute rheumatic fever - Chorea features as part of the acute presentation in 5-10% of patients with rheumatic fever. It can also occur as an isolated event up to 6 months after the initial GABHS infection. It is named Sydenham chorea after the doctor who described St Vitus Dance in the 17th century. Choreiform movements can affect the whole body or just one side of the body, in which case it is referred to as hemi-chorea. The head is often involved with erratic facial movements that resemble grimaces, grins and growns, and the tongue may be affected to resemble a bag of worms when protruded, and protrusion cannot be maintained. In severe cases the patient may have an impaired ability to eat. Chorea disappears with sleep and is made worse by purposeful movements. When the patient is asked to grip the doctor’s hand, the patient will be unable to maintain grip and rhythmic squeezing occurs. There are two signs to look out for in these patients. The first is the spooning sign, which is a flexion at the wrist with finger extension when the hand is held extended. The pronator sign is the second which is when the palms turn outwards when held above the head. Both are consistent with chorea. Remember that the 5 major manifestations of acute rheumatic fever are carditis, polyarthritis, chorea, erythema marginatum and SC nodules – the most common of which are carditis and polyarthritis. The murmur here is a manifestation of carditis. Primary episodes occur mainly in children aged 5-14 and are rare in those over 30. The greatest burden of disease remains in the developing countries and in populations of people living in poverty.

Answer 892

E. Aortic stenosis - Aortic stenosis can present with chest pain, dyspnoea and syncope. It is characterised by a harsh ejection systolic murmur heard loudest at the right upper sternal edge at end expiration, which radiates up towards the carotids. The pulse pressure is narrow and there may be an associated slow-rising and plateau pulse. Doppler echo is vital for diagnosis and shows a pressure gradient across the narrowed valve orifice. This is congenital aortic stenosis due to an abnormally formed aortic valve. He may here be considered for surgical repair or TAVR.

Answer 893

H. Congestive cardiac failure - The signs and symptoms this patient has points to CCF (congestive cardiac failure). SOB with orthopnoea due to the sudden increase in pre-load, indicates LV failure. Neck vein distension is also present, which is a major Framingham criteria for diagnosis. Tachycardia and ankle oedema are both minor criteria for diagnosis. Other major criteria for diagnosis include S3 gallop, cardiomegaly and hepatojugular reflux. For all patients, initial investigations should include ECG, CXR, TTE and bloods including BNP levels. CXR may reveal pulmonary vascular redistribution to the upper zones, Kerley B lines, an increased CTR (cardiomegaly) and pleural effusion. CCF in children occurs as a result of various congenital abnormalities as well as rheumatic fever. Congenital causes include aortic stenosis, PDA and Eisenmenger's syndrome.

Answer 894

A. Amputation - Gangrene occurs as a complication of necrosis and characterised by the decay of body tissue. It can be due to ischaemia, trauma or infection, or a combination of these processes. There is ischaemic gangrene, which arises due to either arterial or venous obstruction. There is also infectious gangrene which include processes like gas gangrene cause by Clostridium perfringens and necrotising fasciitis which has many causes, commonly Streptococcus pyogenes. Diabetes is a risk factor here and is frequently associated with both infectious and ischaemic gangrene. High blood glucose and impaired immunity, peripheral neuropathy and arterial disease contribute to limb-threatening diabetic foot infections. The absent pulses and symptoms this patient is experiencing here (critical limb ischaemia) also suggests diabetic chronic peripheral arterial disease, which in diabetics tends to affect smaller arteries and affects a younger age group when compared to non-diabetics.There is advanced gangrene here, and in cases of severe limb sepsis, amputation is required. This is a two stage process which at first involves guillotine amputation and later, when the infection has cleared, a definitive amputation and wound closure is needed. If the extremity is not viable (such as a large amount of necrosis, profound anaesthesia/paralysis or an inaudible Doppler pulse) then the patient should undergo prompt amputation. Every effort should be made to preserve as many joints as possible in order to improve rehabilitation chances and to decrease the work of walking around with a prosthesis.

Answer 895

L. Conservative management - This is peripheral vascular disease with classic symptoms of claudication (in reality, these classic symptoms only occur in a small minority of patients). ABPI should be performed in symptomatic patients and a result less than or equal to 0.9 is diagnostic for the presence of peripheral vascular disease. You need to however bear in mind that this test may not be accurate if the patient has non-compressible arteries (mainly in diabetic patients). This patient has only presented with claudication which is not severely lifestyle limiting. It depends on how much needing to rest every 800 metres or so bothers him. If he does not feel that this is really a functional disability then no additional treatment is required, but follow-up appointments with a doctor should be made to monitor the development of ischaemic symptoms or coronary and cerebrovascular complaints. If the symptoms are lifestyle limiting then the patient should undergo a supervised exercise programme (only some rather limited quality cohort studies at the moment show an improvement in walking time and symptoms) and medication for symptomatic relief for a period of 3 months. Medication can include cilostazol, pentoxifylline (widely used but no more effective than placebo in RCTs) or naftidrofuryl. Risk factors should also continually be targetted – BP control, statins to lower LDL, beta blockers to target cardiovascular risk and antiplatelet therapy, for instance. If no improvement is made with this regime then patients should be referred to a vascular specialist to have their anatomy defined and assessed for possible revascularisation.

Answer 896

B. Embolectomy - Have a think about the differential diagnosis of sudden onset limb pain. Do you remember the 6 Ps of critical limb ischaemia? This patient’s arrhythmia has caused an embolic event, leading to acute limb ischaemia. There is as a result a sudden decrease in limb perfusion with threatened tissue viability. An emergency vascular assessment needs to be done with duplex ultrasound. Treatment depends on whether the patient already has a history of significant atherosclerosis. If so, there will already be a built up collateral supply so there is potentially a longer time window to act and so anticoagulation and thrombolysis are options. Otherwise an embolectomy will be indicated with a Fogarty catheter if there is not a long enough time window. This is typically done by inserting a Fogarty catheter with an inflatable balloon attached to its tip into the offending atery and passing the tip beyond the clot. The balloon is then inflated and then the catheter is withdrawn to remove the clot.

Answer 897

I. Fasciotomy - This patient has developed compartment syndrome most likely as a result of soft tissue injury or direct injury to the musculature following the recent embolectomy. Additional causes include fractures and compartment haemorrhage. This condition results from raised interstitial pressure in closed osseofascial compartments. The classical clinical diagnosis will be of the following 6 Ps: pain, pressure, pulselessness, paralysis, paraesthesia and pallor (uncommon). The history here of severe extremity pain and tightness following documented trauma is classical. The pain tends to be out of proportion to the injury and is made worse by passive stretching of the muscle groups which are contained by the affected compartment. Passive stretching of the muscles of the compartment which is involved will also elicit pain. Note that true paralysis is a late sign, as is loss of pulses and pallor. Paraesthesia is however an early seen sign. If the diagnosis is uncertain in an at risk patient then compartment pressure measurement is indicated. Measurements of serum CK and urine myoglobin will also be indicated and these may be elevated with muscle cell lysis and necrosis. This is not due to an occlusive dressing (if it were, the first line treatment would be to remove this dressing). Therefore, a fasciotomy is indicated regardless of time of diagnosis with fasciotomy of all compartments with elevated pressure. There is a clear 6 hour window whereby there are lower amputation and death rates compared to delays >6 hours, so this needs to be done as a matter of urgency. The incision needs to be long enough too! Wound care post-fascitomy is important to prevent the risk of secondary infection and to debride any necrotic tissue, or to consider skin grafts. Post-operatively, care will be MDT with physical and occupational therapies and a range of motion exercises to try an d get the patient fully functional.

Answer 898

E. Femoro-popliteal bypass - Broadly speaking, if there is disease with less severe stenosis then endovascular revascularisation is the recommended approach. Surgical revascularisation is recommended if there is more severe stenosis. At this stage, there is no need to get bogged down by the exact recommendations. This significant stenosis will likely require some form of revascularisation. The best option on the list for disease occuring only on one side and localised to the SFA is a femoro-popliteal bypass (colloquially referred to as a fem-pop). Usually the patient's own long saphenous vein is used as a graft. As you may remember from year 2 anatomy, the SFA becomes the popliteal artery at the popliteal fossa. As a brief summary, femoro-femoral cross-over grafts are done for cases of unilateral iliac artery occlusion. Aorto-bifemoral bypass is used for atherosclerosis of the infrarenal aorta and iliacs.

Answer 899

A. Duplex doppler ultrasound - This man is presenting with classic features of a TIA. A TIA is colloquially called a ‘mini stroke’ with symptoms typically lasting under an hour (and resolve within 24 hours). An antiplatelet drug such as aspirin is effective secondary prevention if the patient is not already anticoagulated. The patient will be anticoagulated if they have a likely or known cardioembolic source such as AF. Clopidogrel is an alternative in those who do not tolerate aspirin. The description of transient visual disturbance like a curtain descending over the eye is characteristic of amaurosis fugax. Amaurosis fugax is a transient and painless loss of vision in one eye due to the passage of an embolus into the central retinal artery. The cause could be embolic from the internal carotid artery to cause an occlusion of the ipsilateral retinal artery. Patients presenting with TIAs should be investigated for carotid artery stenosis with a carotid Doppler ultrasound as there is a high risk of having a subsequent full blown stroke. Furthermore if there is a stenosis of >70%, the patient may be a candidate for carotid endarterectomy. Presence of ipsilateral carotid stenosis suggests artery-to-artery embolic event as the cause and this should be the target for surgical or interventional treatment. Follow up tests could be CT angiography or MRA to expand on the abnormal Doppler results. They are not appropriate first line investigations to do here. Head CT is usually normal in TIA. ECG should also be done to investigate for AF which is a common risk factor for embolic cerebral ischaemia.

Answer 900

B. CT scan - Generally, elective surgical repair is indicated in patients with large symptomatic AAA – repair of aneuryms over 5.5cm offers a survival advantage. Also, young healthy patients and women in particular may benefit from early repair for smaller AAAs. Data suggests that EVAR (endovascular AAA repair) is equivalent to open repair in terms of overall survival but the rate of secondary interventions is higher with EVAR. Generally, those with a greater risk of perioperative morbidity and mortality, such as patients with co-morbidities such as COPD, may benefit from the less invasive approach (anatomy permitting). Younger and healthier patients may benefit from the relative durability of traditional open repair. A CT scan is useful for diagnosis aortic aneurysms which lie close to the origins or or proximal to the renal arteries. While abdominal ultrasound can also identify the AAA and aortic dilation, a CT scan is more useful in localising this lesion and its relationship to the renal vasculature

Answer 901

C. Ankle-brachial pressure index - This is peripheral vascular disease (claudication) and the first investigation to do here is an ABPI. This is an ankle brachial pressure index and has a sensitivity of 95% and a specificity of 100%. It is however important to remember that it may not be accurate in patients who have non-compressible arteries – so beware, particularly in diabetics. Those with either severely stenotic or totally occluded arteries may have a normal ABPI if there is abundant collateral circulation. ABPI of les s than or equal to 0.9 is diagnostic for the presence of peripheral vascular disease. ABPI is performed by taking the systolic pressure of the left and right brachial arteries and the left and right PT and DP arteries pressure. The ABPI is the highest of the DP and PT pressure divided by the higher of the left and right arm brachial artery pressure. Finding the artery with the probe is a skill which may take a bit of practice so have a play around with the probe if you are stuck on a vascular attachment. ABPI is a marker of peripheral atherosclerosis as well as a predictor of vascular events. Risk factors for PVD include smoking, diabetes, old age, hyperlipidaemia and history of coronary or cerebrovascular disease. Treatment is outlined in the explanation for question 2 of the previous EMQ set.

Answer 902

D. No investigation necessary - This is a history of a ruptured AAA. There is back pain here and shut down peripheries and pallor due to blood loss. This patient is in haemorrhagic shock. As this AAA has ruptured, this man will need urgent surgical repair, with of course standard resuscitation measures. Investigations would just waste time. The airway will needed to be managed with supplemental oxygen and ET intubation, a central venous catheter will need to be inserted, an arterial catheter and urinary catheter will also be needed for monitoring, and the target systolic BP is 50-70. Infusing too many fluids may increase the risk of death. The most effective form of surgical repair is an EVAR (endovascular AAA repair), anatomy permitting, otherwise traditional open repair is performed. Open repair has a mortality of 48%. Antibiotics will also be needed to cover bacteria to prevent graft infection. This will be prescribed in line with local protocols

Answer 903

A. Duplex doppler ultrasound - Ablative procedures include stripping and ligation, the aim of which is to permanently remove the varicose vein. Radiofrequency ablation (RFA) can also be done, as well as endovenous laser therapy and foamed sclerotherapy. Phlebectomy or sclerotherapy can also be performed. This is generally reserved for symptomatic cases, although this woman has a cosmetic issue with the appearance of her legs which is causing her distress. There are complications of ablation which the patient will need to be made aware of though, such as bleeding, infection, saphenous nerve injury and neovascularisation. A duplex ultrasound is the investigation which is required here. It can assess reversed flow and valve closure time. This should be done with the patient standing and with the leg in external rotation for best sensitivity. Specific segments which are affected by reflux can be delineated as the superficial and deep truncal veins, perforators and tributaries can all be visualised. Reflex in the great saphenous or common femoral can be detected with Valsalva while more distal reflux can be elicitied by compressing the leg above the Doppler probe to see if any blood is being forced back towards the feet.

Answer 904

H. Intermittent claudication - This is peripheral vascular disease with classic symptoms of claudication (in reality, these classic symptoms only occur in a small minority of patients). Remember also that intermittent claudication can also occur in the large muscle groups of the upper leg, which is indicative of narrowing of the deep femoral artery. ABPI should be performed in symptomatic patients and a result less than or equal to 0.9 is diagnostic for the presence of peripheral vascular disease. You need to however bear in mind that this test may not be accurate if the patient has non-compressible arteries (mainly in diabetic patients like this one). If she does not feel that this claudication is really a functional disability then no additional treatment is required, but follow-up appointments with a doctor should be made to monitor the development of ischaemic symptoms or coronary and cerebrovascular complaints. If the symptoms are lifestyle limiting then the patient should undergo a supervised exercise programme (only some rather limited quality cohort studies at the moment show an improvement in walking time and symptoms) and medication for symptomatic relief for a period of 3 months. Medication can include cilostazol, pentoxifylline (widely used but no more effective than placebo in RCTs) or naftidrofuryl. Risk factors should also continually be targetted – BP control, statins to lower LDL, beta blockers to target cardiovascular risk and antiplatelet therapy, for instance. If no improvement is made with this regime then patients should be referred to a vascular specialist to have their anatomy defined and assessed for possible revascularisation.

Answer 905

I. Compartment syndrome - This patient has developed compartment syndrome due to trauma and the fracture. This condition results from raised interstitial pressure in closed osseofascial compartments. The classical clinical diagnosis will be of the following 6 Ps: pain, pressure, pulselessness, paralysis, paraesthesia and pallor (uncommon). The history tends to be of severe extremity pain and tightness following documented trauma. The pain tends to be out of proportion to the injury and is made worse by passive stretching of the muscle groups which are contained by the affected compartment. Passive stretching of the muscles of the compartment which is involved will also elicit pain. Note that true paralysis is a late sign, as is loss of pulses and pallor. Paraesthesia is however an early seen sign. If the diagnosis is uncertain in an at risk patient then compartment pressure measurement is indicated. Measurements of serum CK and urine myoglobin will also be indicated and these may be elevated with muscle cell lysis and necrosis. This is not due to an occlusive dressing (if it were, the first line treatment would be to remove this dressing). Therefore, a fasciotomy is indicated regardless of time of diagnosis with fasciotomy of all compartments with elevated pressure. There is a clear 6 hour window whereby there are lower amputation and death rates compared to delays >6 hours, so this needs to be done as a matter of urgency. The incision needs to be long enough too! Wound care post-fascitomy is important to prevent the risk of secondary infection and to debride any necrotic tissue, or to consider skin grafts. Post-operatively, care will be MDT with physical and occupational therapies and a range of motion exercises to try an d get the patient fully functional.

Answer 906

C. Dead limb - This gangrene may well have mixed infectious and ischaemic aetiology – both of which have associations with DM. You will also hear infectious gangrene being referred to as ‘wet gangrene’ and ischaemic gangrene being referred to as ‘dry gangrene’. Atherosclerosis is associated with DM and forms an important cause of dry gangrene. This occurs due to chronic impairment of blood flow and typically presents with tissue which is dry, black and cold – in most cases, self-amputation eventually occurs. Wet gangrene includes causes such as necrotising fasciitis and gas gangrene. Evidence of non-viability and irreversibility of the affected limb include major tissue loss, sensory loss with rest pain, inaudible arterial Doppler signals and muscle weakness. Fixed mottling does not blanch on pressure and is frequently associated with a limb which is beyond salvage.

Answer 907

A. Critical limb ischaemia - Is this patient’s ulcer an arterial ulcer with diabetic neuropathy, or a diabetic ulcer? Difficult to tell. However, this patient does have critical limb ischaemia. Critical limb ischaemia is chronic severe limb ischaemia. This patient has rest pain which is relieved by hanging the leg dependent – this is classical. These patients have chronic ischaemic symptoms of the leg such as ischaemic rest pain, gangrene and non-healing wounds. Ischaemic cause of these symptoms needs to be established urgently, or if the patient already has a documented history of PVD, then they can be referred to a vascular surgeon for revascularisation. Their arterial anatomy needs to be defined and assessed. Risk factors also need to continue to be aggressively targetted. If they are not a candidate for revascularisation then the patient should be assessed for amputation where necessary and be on appropriate risk factor reduction medication.

Answer 908

F. Deep vein thrombosis - This patient has a DVT. There is a history of smoking, DM and immobility. Other risk factors include recent major surgery, active malignancy, pregnancy and malignancy. A Wells score is determined in all patients with a suspected DVT with the condition being likely if the score is 2 or greater. The most definitive test is venography but it is invasive. Compression USS of the proximal deep venous system is preferred but Doppler venous flow testing can be used if other tests are unavailable and will demonstrate low flow in affected veins. This requires a trained technician. Anticoagulation is the mainstay of treatment with unfractionated heparin, a LMWH or an anti FXa agent such as fondaparinux.

Answer 909

J. Leriche syndrome - Leriche’s syndrome, named after some French surgeon, is aortoiliac atherosclerotic occlusive disease and is one of those eponymous syndromes which can be found in your Oxford Handbook. This involves the abdominal aorta and/or both of the iliac arteries. It presents with the classic triad of buttock claudication, impotence and reduced or absent femoral pulses. This combination is referred to as Leriche’s syndrome.

Answer 910

E. Aspirin and clopidogrel - Dual antiplatelet therapy is recommended for at least 12 months in all patients whether or not they have been stented. Aspirin should be continued forever and clopidogrel for at least a year. Note that prasugrel, a platelet inhibitor, has been found in relatively recent studies to be superior to clopidogrel in outcome measures when given for at least a year – although there is an increased risk of bleeding in low weight patients and in those over 74 years of age (lower doses are recommended in these patients)

Answer 911

C. ACE inhibitor - ACE inhibitors should be started early (when the patient is haemodynamically stable, optimally on the first day in hospital) for a favourable effect on ventricular remodelling, particularly for patients with a large anterior wall MI. There is good evidence to suggest that ACE inhibitors are more effective at reducing overall mortality and sudden cardiac death after 2-42 months compared to placebo, especially when started within 14 days of an acute MI.

Answer 912

L. Clopidogrel and atorvastatin - Remember that in acute stroke, there is no current evidence that non-aspirin antiplatelet drugs such as dipyridamole, clopidogrel and glycoprotein IIb/IIIa inhibitors are effective so they are not indicated. However, clopidogrel is useful in the secondary prevention of stroke and is indicated in those who are sensitive to aspirin. A statin is also given here for its lipid lowering effects to lower the risk of stroke and other cardiovascular events. In terms of antiplatelet drugs for secondary prevention, a systematic review of the studies have found that the most cost-effective treatment is clopidogrel, followed by MRD (modified release dipyridamole) followed lastly by aspirin. Incidentally, in terms of cost-effectiveness, clopidogrel should be the first choice agent anyway.

Answer 913

G. Warfarin - Anticoagulation with warfarin is indicated for atrial fibrillation in the secondary prevention of stroke, with target INR of 2.5 (range 2-3). This is for when the stroke (or indeed TIA) is following a cardioembolic source like in this case. If the stroke is not cardioembolic then these drugs may not confer any long term benefit and will increase the patient’s risk of serious bleeding. Those who are not appropriate candidates for warfarin should have clopidogrel added to aspirin to reduce the risk of stroke. Note that I have not included ‘Aspirin and warfarin’ as an option because this can be given in some cases, such as after multiple recurrent strokes caused by AF and co-existing coronary artery disease, but is controversial.

Answer 914

A. Aspirin and atorvastatin - I will accept ‘Clopidogrel and atorvastatin’ too for this question, as clopidogrel is the most cost effective antiplatelet drug for the secondary prevention of ischaemic stroke or TIA. However, aspirin is what is generally given. The statin, as mentioned before, is given for its lipid lowering effects. It is mainly given for patients with LDL >2.6 and is recommended for those with atherosclerotic ischaemic stroke or TIA, to lower the risk. This patient has PVD which is due to atherosclerosis and suggests that statins will help. Aspirin and clopidogrel would not be given together in this patient as this would unnecessarily increase the risk of a major bleed.

Answer 915

D. Metastatic disease - Back pain and a raised ALP is an ominous finding which is usually indicative of bone metastases. Alkaline phosphatase is an enzyme which is also a marker for bone turnover. There is often a history of maligancy before this back pain and commonly implicated malignancies include breast, lung, prostate, thyroid and kidney cancer. Neurological deficits may occur if the tumour destruction is extensive and causes compression of nerves. The patient may also have generalised systemic symptoms on examination including fever, chills, weight loss and focal tenderness. XR may demonstrate lysis of the vertebral body and MRI may show up a lytic or blastic lesion.

Answer 916

A. Perforated duodenal ulcer - Long standing RA leads you towards high NSAID use. NSAIDs, along with H pylori infection are a major risk factors in peptic ulcer disease. The patient’s advanced age adds to this risk. This accounts for black tarry stools over many years which result from blood loss from the ulcer. The acute symptoms now of severe abdominal pain and hypotension is likely to be due to acute blood loss from a perforated ulcer. This patient needs emergency surgery. NSAID induced ulcers are more commonly gastric than duodenal. Stopping NSAID use will reduce ulcer recurrence. If NSAIDs cannot be discontinued, then prescribing a PPI alongside will reduce recurrence.

Answer 917

B. Mesenteric infarct - The irregularly irregular pulse is a hallmark sign of atrial fibrillation which has led to cardioembolism and subsequent occlusion of the mesenteric vasculature. Untreated AF can lead to a thrombus forming inside the heart which can then embolise like this case to the mesenteric vasculature. This person is also old, which is an additional risk due to comorbidities like atherosclerosis. This patient has the symptoms and signs of ischaemic bowel disease (which encompasses acute mesenteric ischaemia, chronic mesenteric ischaemia and colonic ischaemia). This is likely acute mesenteric ischaemia – something that in a person who presents like this, you should maintain a high index of suspicion for as the presentation can be quite non-specific but the condition can be deadly. You would likely in this case opt for surgical intervention without delay although you can consider some form of imaging first to localise the bleed.

Answer 918

C. Pancreatitis - The AAA here has nothing to do with the question, and at 5cm, it is not massively prone at the moment to rupture. However, if the patient is otherwise young, fit and healthy, elective repair can soon be considered (the normal abdominal aorta is 1.5cm diameter, and remember that rupture is more common in females than males). This is acute pancreatitis. Jaundice here is suggestive of gallstone aetiology with obstruction to the common bile duct, though pancreatic oedema can itself cause jaundice. Nausea and vomiting is not uncommon and can occur with agitation and confusion. Complicated haemorrhagic pancreatitis may exhibit Cullen’s sign, Grey-Turner’s sign and Fox’s sign. Make sure you know what these are and you are familiar with the causes of acute pancreatitis (GET SMASHED). Those caused by hypocalcaemia may also display Chvostek’s sign and Trousseau’s sign. Key to diagnosis is serum amylase or lipase levels which are massively elevated. Prognostic criteria are outlined in Ranson’s criteria applied on admission and after 48 hours, or the modified Glasgow score which you can find in your Oxford Handbook.

Answer 919

J. Ruptured abdominal aortic aneurysm - This is a history of a ruptured AAA. There is abdominal (which the patient states as chest) pain radiating around to the back here and pallor due to blood loss suggesting this diagnosis. As this AAA has ruptured, this man will need urgent surgical repair, with of course standard resuscitation measures. Investigations would just waste time although it seems he has had a troponin, and likely ECG too. Misdiagnosing this condition is pretty poor of the doctors with the history here of pain which radiates to the back. The airway will needed to be managed with supplemental oxygen and ET intubation, a central venous catheter will need to be inserted, an arterial catheter and urinary catheter will also be needed for monitoring, and the target systolic BP is 50-70. Infusing too many fluids may increase the risk of death. The most effective form of surgical repair is an EVAR (endovascular AAA repair), anatomy permitting, otherwise traditional open repair is performed. Open repair has a mortality of 48%. Antibiotics will also be needed to cover bacteria to prevent graft infection. This will be prescribed in line with local protocols.

Answer 920

M. Dissecting aortic aneurysm - I don’t know why the A&E Sister has fast bleeped a medical student here but BP differential between the 2 arms is a hallmark feature of this condition. Pulse differences may also be present in the lower limbs. Aortic dissection, however, typically presents with tearing chest pain (but this isn’t something a collapsed patient is in a position to report). There may also be interscapular pain with dissection of the descending aorta. Dissecting aneurysms are either type A, which involves the ascending aorta, or type B. Type A dissections require urgent surgery whereas type B can be managed medically if it is not complicated by end organ ischaemia. There may also be the diastolic murmur of AR in proximal dissections. A CT scan is indicated as soon as a diagnosis of aortic dissection is suspected and should be from the chest to the pelvis to see the full extent of the dissecting aneurysm. What you will see is the intimal flap. MRI is more sensitive and specific but is more difficult to obtain acutely. Of course, the investigations are irrelevant when you have an emergency case and a collapsed patient in front of you who needs immediate resuscitation measures starting with your ABC. Strong risk factors include hypertension, atherosclerotic aneurysmal disease, Marfan’s or Ehlers-Danlos, bicuspid aortic valve, coarctation, smoking, FH and annulo-aortic ectasia.

Answer 921

G. Myocardial infarction - Given the list of options here, this is likely to be an atypical MI which is more common in diabetics and the elderly, likely to be due to autonomic neuropathy. They are known as ‘silent’ MI and should be excluded in all causes of collapse. An ECG is indicated here after the clear CT scan (although may have already been done on admission by alert staff). STEMI, new LBBB or confirmed posterior MI is an indication for PCI/thrombolysis. It is worth noting that RV infarction is present in 40% of inferior infarcts so in this case, right sided ECG leads should also be obtained.

Answer 922

J. Endovascular aneurysm repair - Incidental finding of a large AAA requires elective surgical repair (exceeding 5.5cm in men, 5cm in women – repair of aneuryms greater or equal to 5.5cm offers a survival advantage). Additionally, rapid increase in size is also an indication for elective repair. Young and healthy patients, particularly women, may benefit from early repair of smaller AAAs. (>5cm). Data suggests EVAR is equivalent to open repair in terms of overall survival but there is a higher rate of secondary interventions with EVAR. Therefore younger and healthier patients may benefit more from open repair. Patient 1 is however is elderly and has co-morbidities. An EVAR is the best way forward here. However note also that EVAR could entail a complication of endovascular repair leak, which would require corrective treatment. Endoleak is persistent blood flow outside the graft and within the aneurysm sac. There is 24% risk after EVAR. However, this is not a complication of open repair, which is probably preferred in most cases in those who are fit and healthy enough to have it such as Patient 4. Management of this complication would depend on the type of endoleak.

Answer 923

H. Endarterectomy - Amaurosis fugax is a transient and painless loss of vision in one eye due to the passage of an embolus into the central retinal artery. This temporary arrest of blood flow leads to vision loss. The cause could be embolic from the internal carotid artery to cause an occlusion of the ipsilateral retinal artery. Patients presenting in this way should be investigated for carotid artery stenosis with a carotid Doppler ultrasound and if there is a stenosis of >70%, the patient may be a candidate for carotid endarterectomy. Presence of ipsilateral carotid stenosis suggests artery-to-artery embolic event as the cause here and this should be the target for surgical or interventional treatment.

Answer 924

E. Ultraasound - For asymptomatic small AAA, surveillance is indicated. Infra and juxtarenal AAAs between 4.0-5.4cm in diameter (bear in mind that young and healthy patients with >5cm may benefit from repair!) should be monitored by USS or CT every 6-12 months. There is good quality evidence that the risk of rupture is 20% for aneurysms larger than 5.0-6.0cm in diameter. Note also that AAAs

Answer 925

K. open repair of aneurysm - Incidental finding of a large AAA requires elective surgical repair (exceeding 5.5cm in men, 5cm in women – repair of aneuryms greater or equal to 5.5cm offers a survival advantage). Additionally, rapid increase in size is also an indication for elective repair. Young and healthy patients, particularly women, may benefit from early repair of smaller AAAs. (>5cm). Data suggests EVAR is equivalent to open repair in terms of overall survival but there is a higher rate of secondary interventions with EVAR. Therefore younger and healthier patients may benefit more from open repair. Patient 1 is however is elderly and has co-morbidities. An EVAR is the best way forward here. However note also that EVAR could entail a complication of endovascular repair leak, which would require corrective treatment. Endoleak is persistent blood flow outside the graft and within the aneurysm sac. There is 24% risk after EVAR. However, this is not a complication of open repair, which is probably preferred in most cases in those who are fit and healthy enough to have it such as Patient 4. Management of this complication would depend on the type of endoleak.

Answer 926

A. Angioplasty - Stenosis and thrombosis of AV fistulae may lead to loss of vascular access sites which presents a problem for chronic haemodialysis patients. Percutaneous transluminal angioplasty is a commonly employed technique used to correct the stenosis and restore viability. All patients who are having haemodialysis should be educated about vein preservation with limiting venepuncture and IV access in the access arm.

Answer 927

B. Femoral-distal bypass - Previous angioplasties have failed so there is clearly not point in having another endovascular intervention such as PTA (percutaneous transluminal angioplasty) with balloon dilation, stents, laser, atherectomy or thermal angioplasty. The occlusion here is at the level of the popliteal artery so a femoral-distal bypass is indicated. Surgical revascularisation is recommended for lesions involving the popliteal artery. Make sure you know your vascular anatomy! (At least the big vessels).

Answer 928

B. Urgent surgical repair - This is a history of a ruptured AAA. The history of heavy smoking is a risk factor here for atherosclerotic disease. There is abdominal pain radiating around to the back here and pallor due to blood loss suggest this diagnosis. As this AAA has ruptured, this man will need urgent surgical repair, with of course standard resuscitation measures. Investigations would just waste time. The airway will needed to be managed with supplemental oxygen and ET intubation, a central venous catheter will need to be inserted, an arterial catheter and urinary catheter will also be needed for monitoring, and the target systolic BP is 50-70. Infusing too many fluids may increase the risk of death. The most effective form of surgical repair is an EVAR (endovascular AAA repair), anatomy permitting, otherwise traditional open repair is performed. Open repair has a mortality of 48%. Antibiotics will also be needed to cover bacteria to prevent graft infection. This will be prescribed in line with local protocols. AAA is defined as dilation of the abdominal aorta to a diameter >3cm – this represents a 50% increase. There has been an aetiological shift – of the first 10 AAA repairs performed by Eastcott in London in the 1950s, 4 of them were tuberculous or syphilitic. Now most are atherosclerotic. Of men >65, 5% have an AAA and there is a M:F of 8:1 (although in terms of risk of rupture, there is a 3:1 female preponderance). This condition is still a common cause of sudden death (3rd in this country), and most are completely asymptomatic up until the point they rupture. A misdiagnosis as either ureteric colic, or acute pancreatitis, is disasterous so this is really one not to be missed. Of all patients who have a ruptured AAA, 75% die before they even reach hospital. The main three processes involved in aetiology are: inflammation, loss of smooth muscle and excess proteolysis due to MMPs. Incidentally, DM appears to be protective in AAA development.

Answer 929

I. Ultrasound in 6 months - These questions require a knowledge of guidelines for AAA management. What you need to know: * Ruptured AAA needs resuscitation measures and urgent surgical repair as mentioned above. * Symptomatic but not yet ruptured AAA needs semi-urgent surgical repair. EVAR can be offered if aorto-iliac anatomy is permitting. Urgent traditional open repair of symptomatic unruptured AAAs carries increased morbidity and mortality with a rate between that of ruptured AAA repair and elective repair. Beta blockers should be used pre-op with peri-op antibiotic therapy also initiated. * Incidental finding of a large AAA requires elective surgical repair (exceeding 5.5cm in men, 5cm in women – repair of aneuryms greater or equal to 5.5cm offers a survival advantage). Additionally, rapid increase in size is also an indication for elective repair. Young and healthy patients, particularly women, may benefit from early repair of smaller AAAs. (>5cm). Data suggests EVAR is equivalent to open repair in terms of overall survival but there is a higher rate of secondary interventions with EVAR. Therefore younger and healthier patients may benefit more from open repair. * For asymptomatic small AAA, surveillance is indicated. Infra and juxtarenal AAAs between 4.0-5.4cm in diameter (bear in mind that young and healthy patients with >5cm may benefit from repair!) should be monitored by USS or CT every 6-12 months. There is good quality evidence that the risk of rupture is 20% for aneurysms larger than 5.0-6.0cm in diameter. * AAAs

Answer 930

H. Ultrasound in 1 or more years - These questions require a knowledge of guidelines for AAA management. What you need to know: * Ruptured AAA needs resuscitation measures and urgent surgical repair as mentioned above. * Symptomatic but not yet ruptured AAA needs semi-urgent surgical repair. EVAR can be offered if aorto-iliac anatomy is permitting. Urgent traditional open repair of symptomatic unruptured AAAs carries increased morbidity and mortality with a rate between that of ruptured AAA repair and elective repair. Beta blockers should be used pre-op with peri-op antibiotic therapy also initiated. * Incidental finding of a large AAA requires elective surgical repair (exceeding 5.5cm in men, 5cm in women – repair of aneuryms greater or equal to 5.5cm offers a survival advantage). Additionally, rapid increase in size is also an indication for elective repair. Young and healthy patients, particularly women, may benefit from early repair of smaller AAAs. (>5cm). Data suggests EVAR is equivalent to open repair in terms of overall survival but there is a higher rate of secondary interventions with EVAR. Therefore younger and healthier patients may benefit more from open repair. * For asymptomatic small AAA, surveillance is indicated. Infra and juxtarenal AAAs between 4.0-5.4cm in diameter (bear in mind that young and healthy patients with >5cm may benefit from repair!) should be monitored by USS or CT every 6-12 months. There is good quality evidence that the risk of rupture is 20% for aneurysms larger than 5.0-6.0cm in diameter. * AAAs

Answer 931

C. Elective open repair - These questions require a knowledge of guidelines for AAA management. What you need to know: * Ruptured AAA needs resuscitation measures and urgent surgical repair as mentioned above. * Symptomatic but not yet ruptured AAA needs semi-urgent surgical repair. EVAR can be offered if aorto-iliac anatomy is permitting. Urgent traditional open repair of symptomatic unruptured AAAs carries increased morbidity and mortality with a rate between that of ruptured AAA repair and elective repair. Beta blockers should be used pre-op with peri-op antibiotic therapy also initiated. * Incidental finding of a large AAA requires elective surgical repair (exceeding 5.5cm in men, 5cm in women – repair of aneuryms greater or equal to 5.5cm offers a survival advantage). Additionally, rapid increase in size is also an indication for elective repair. Young and healthy patients, particularly women, may benefit from early repair of smaller AAAs. (>5cm). Data suggests EVAR is equivalent to open repair in terms of overall survival but there is a higher rate of secondary interventions with EVAR. Therefore younger and healthier patients may benefit more from open repair. * For asymptomatic small AAA, surveillance is indicated. Infra and juxtarenal AAAs between 4.0-5.4cm in diameter (bear in mind that young and healthy patients with >5cm may benefit from repair!) should be monitored by USS or CT every 6-12 months. There is good quality evidence that the risk of rupture is 20% for aneurysms larger than 5.0-6.0cm in diameter. * AAAs

Answer 932

G. Addison's disease - This patient has symptoms of adrenal insufficiency. Her Systolic Bp falls by more than 20mmHg on standing which means she has postural hypotension. Hyperpigmentation of the gingiva and buccal mucosa suggests high ACTH output which in this patient would be due to primary adrenal insufficiency...Addison's disease. The condition is probably of AI origin in this girl and a positive finding may be 21 hydroxylase auto-antibodies.

Answer 933

J. Cushing's disease - This man has weight gain and impaired wound healing, already you should be thinking cushings or diabetes...The blood test used here is being performed to differentiate between cushings disease and cushings syndrome. 10ug ovine or human CRH is administered to a patient who has fasted at least 4 hrs. pre test and serial post test ACTH and cortisol measurements are taken. In pituitary driven cushing's disease the cortisol can be manipulated, that is to say exogenous CRH will cause a recordable increase in serum cortisol by the 2hr mark (and ACTH). However if the source of the excess cortisol is an ectopic ACTH producing tumour for example, this is not subject to any feedback mechanisms and so no change in serum cortisol will be seen on giving CRH.

Answer 934

L. Addisonian crisis - This man is found collapsed, the cause of his collapse is not given but one differential is always cardiovascular. He is shown to be hyponataraemic and hyperkalaemic. This picture fits adrenal failure well, the raised potassium is a result of mineralocorticoid deficiency leading to decreased K+ excretion. The CXR is hinting at upper lobe fibrosis as seen in patients who have had Tuberculosis, and further the abdo film shows calcification of the adrenals bilaterally which is the radiographic appearance of TB infiltration of the adrenal galnds. This man has Addison's is caused by TB. Furthermore he is found collapsed with low blood glucose. He has signs of shock...Tachycardia and oliguria so he is in fact in Addisonian crisis which should be treated promptly with gluocorticoid replacement and dextrose, this man has evidence of mineralocorticoid deficiency also so fludrocortisone should be coadministered.

Answer 935

D. Pituitary apoplexy - Has bitemporal hemianopia which has been worsening..this should spark thoughts of a growing pituitary adenoma. But then she presents with an acute change. With a tumour a sudden change is usually vascular.. in this case the patient is suffering pituitary apoplexy whereby her enlarging pituitary tumour has undergone haemorrhage. This has basically wiped out pituitary function in a moment which explains her cardiovascular collapse and immensely painful headache. In fact apoplexy can present just like a sub-arachnoid haemorrhage in patients with known adenomas... Splitting headache - INCREDIBLY BAD - Xanthochromia (from extravasation of blood through the diaphrgam sellae into the sub aracahnoid space) - Vomiting - Loss of consciousness. ``` The opthalmoplegia (diplopia) is a mass effect of the tumour being displaced by the bleed to impinge on the cavernous sinus and hence the IIIrd Cranial Nerve. Management is fluid resuscitation, and then hormone replacement of all lacking hromines! NB: In EMQS xanthochromia is usually pathognomonic of SAH. ```

Answer 936

I. Sheehan's syndrome - Pre-eclampsia (proteinuria + HTN in pregnancy) is a major risk for post partum haemorrhage...In this ladies case she had to receive blood products and Syntocin (a synthetic oxytocin drug used to combat uterine atony and help stop haemorrhaging). Blood loss doesn't normally cause changes in pituitary function however in pregnancy the pituitary gland is particularly vulnerable. Hyperplasia and Hypertrophy of the lactotrophe cells in pregnancy (due to increased LH/FSH) leads to an increase in size of the adenphypophysis, however there is no such increase in the vascular supply to the anterior pituitary. When blood is lost in post -partum haemorrhage (PPH) the ant. pituitary thus infarcts and necroses. This is Sheehans syndrome...it can present insidiously or in a major way. It may be picked up if the mother fails to breast feed, but amennorhoea following birth and loss of pubic and axillary hair is also a common presentation. Only occasionally is full blown Sheehan's encountered where the Mother becomes hypothyroid and can develop Diabetes mellitus due to wildly deranged Ant. Pituitary function. DX: low oestradiol and pituitary hormone levels. NB: Diabetes Insipidus is a rare complication since the posterior pituitary has a rich arterial blood supply that is rarely compromised in PPH.

Answer 937

C. Nelson's syndrome - Worsening loss of peripheral vision infers pituitary adenoma. Hyperpigmented scars suggests a state of increased ACTH production however this patient doesn't seem to be in adrenal failure, she is overweight. She has had abdominal surgery and was on metyrapone (a drug which inhibits cortisol production) and Aminoglutethamide (an adrenolytic) Basically this woman is overweight and has striae because she was cushingoid.. the cause was adrenal hyperplasia. She was on medication to control this but the decision was made to operate and a bilateral adrenalectomy was performed. The consequences of this is that she has very minimal cortisol production and her pituitary is striving to fuel this by pumping out lots of ACTH (hence the dark scars). The visual field problems are due to an undiagnosed pituitary adenoma that was non functional but since her surgery the pituitary tumour has grown substantially since there is no negative feedback coming from circulating cortisol. This is basically Nelson's syndrome. NB : these days bilateral adrenalectomy is very rare and the pituitary is sometimes irradiated to prevent Nelson's from occurring. This patient needs glucocorticoid replacement and transsphenoidal surgery.

Answer 938

K. Ectopic ACTH producing tumour - This lady is cushingoid. That's given. But is the pathology Cushing's disease or syndrome - the dexamethasone suppression test tells us this. Administration of low dose dexamethasone (0.5 mg) would suppress cortisol production in a normal person. If suppression was not achieved then hypercortisolism is present. The high dose dexamethasone test uses 2mg which is capable of producing partial/complete suppression of cortisol if the problem is a pituitary tumour (since the pituitary retains some feedback control). However an ectopic source of ACTH would not be responsive to exogenous feedback and so its not suppressed. This lady therefore has Cushing's Syndrome caused by an ectopic source of ACTH. The darkening of the palmar crease confirms excess ACTH and the hypokalaemia supports this showing there is excessive aldosterone stimulation. Common causes for ectopic ACTH = Small cell carcinoma of the lung and Carcinoid tumours. The presence of Acanthosis nigricans (velvet under axilla) might push you towards investigating for carcinoid tumours. NB: Acanthosis is not only caused by Diabetes and in real life is frequently followed up to screen for Ca. EMQS nigricans + Diabetes pretty much.

Answer 939

B. Panic attack

Answer 940

H. Phaeochromocytoma

Answer 941

C. Inconclusive sample

Answer 942

F. Insulinoma

Answer 943

E. Graves disease

Answer 944

1. Primary adrenal insufficiency - Tetracosactide is synthetic ACTH. When administered during a short synacthen test it can be used to determine whether a patient has adrenal or pituitary hypocortisolism. A Normal test would be : basal cortisol >170mM and 30 minutes cortisol should be at least double the basal measurement and be greater than 550mM This man's basal cortisol is normal however his 30 minutes cortisol has less than doubled and is less than 550mM which indicates that the adrenals are not functioning properly. In secondary adrenal insufficiency the basal cortisol would be very low but would exhibit large increases on administration of tetracosactide.

Answer 945

3. Inconclusive - The estimated serum osmolality is 271 mosm which is low. However one can see that is mainly due to the low sodium. The Triglycerides given are seriously elevated so this is a case of pseudohyponatraemia. This occurs in samples with hyperproteinaemia or hyperlipidaemia as the excess fat/ protein occupies a disproportionately large amount of plasma volume thus diluting the sodium. This sample is inconclusive! The excess fat would not change the directly measured plasma osmolality but does change the estimated value. Thus if we were to compare actual measured osmolality with our estimate we would expect an osmolar gap. A gap greater than 10-15 mOsm indicates either an error, a state of excess fat/protein or the presence of solutes not being measured such as blood ethanol.

Answer 946

A. Inadequate treatment

Answer 947

H. Epileptic seizure

Answer 948

G. Microadenoma

Answer 949

D. Macroadenoma

Answer 950

B. Metclopramide

Answer 951

H. SIADH - Opioids and operative pain are known causes for SIADH. The man is hyponatraemic with concentrated urine. His urine is inappropriately concentrated given his dilutional hyponatraemia. This is caused by excessive production of ADH. Excessive fluid replacement post surgery can exarcebate SIADH. The diagnosis requires absence of hypovolaemia and oedema (and use of diuretics). It also depends on urine osmolality being >500 mOsm and high in sodium. Causes Include Drugs, malignancies (ie.SCC of the lung) CNS disorders including guillain barre and head trauma. Chest infections in hospital can lead to SIADH developing. Metabolic diseases can also be responsible.

Answer 952

C. Liddle's syndrome - This man has Liddle's syndrome a form of pseudohyperaldosteronism which is caused by overactive eNaC ( epithelial sodium channel) found in the collecting ducts. Liddles is an autosomal dominant condition hence the strong family history. The biochemical picture of hypernatraemia and hypokalaemia mimicks that of hyperaldosteronism, normally aldosterone modulates the eNacs activity however in Liddle's the problem is the channel does not dissocciate properly so blockade of aldosterone with spironolactone is ineffective. Direct eNaC antagonists are used...Amiloride or Triamterene in conjucntion with a low sodium diet.

Answer 953

G. Conn's adenoma - This lady has true primary hyperaldosteronism caused by a conns adenoma. Hyperaldosteronism should always be considered in the young hypertensive. Hypokalaemia and hypernatraemia are caused by the aldosterone excess. Headaches are due to the hypertension and hypokalaemia there are also a number of musculoskeletal symptoms that patients complain of. The tingling can be peri-oral and the twitching was alluding to trousseau's sign of hypocalcaemia. Trousseau's sign : ALWAYS an EMQ in the exam, basically in hypocalcaemia there is carpo-pedal spasm when blood pressure cuff is inflated to above systolic pressure. Chvostek's sign : tapping the zygoma produces facial spasm. the hypocalcaemia is caused because hyperaldosteronism leads to metabolic alkalosis and the raised blood pH makes calcium less available to tissues thus causing a functional hypocalcaemia. Liddles is not an option because there is nil family history of note. Mx = spironolactone and surgery.

Answer 954

B. Addison's disease - Tanned with hyponatraemia...it must be addisons. The hyperkalaemia shows there is mineralocorticoid deficiency..replacement therapy is key as addisonian crisis may ensue.

Answer 955

D. Paraneoplastic syndrome - Smoker with radiotherapy..the hyponatraemia is caused by SIADH, the aetiology is paraneoplastic syndrome. He has a bronchial or small cell carcinoma which is producing excess ADH.

Answer 956

C. Impaired fasting glucose - This patient hasn't eaten for over 12 hours so his BM is a fasting glucose measurement. Normal value for which would be less than 6 mmol/L

Answer 957

E. Non- significant - This patient has had a random blood glucose taken. A reading of > 11.1 mmol/L would almost definitely mean she was diabetic. A reading greater than 7.8 might be suggestive of some dysfunction and possible prediabetes although it is not conclusive. However her reading is 7.7 mmol..nothing can be derived from this.. it is a non-significant reading.

Answer 958

A. Impaired glucose tolerance - The random glucose for this patient is suggestive of a problem and the oral glucose tolerance test gives a reading

Answer 959

B. Normal - This womans fasting BM is completely innocuous. After eating a meal her blood glucose remains below a level at which there may be suspicion of IGT. This patient is therefore definitely normal * A useful marker for body glucose regulation is looking at post prandial rises in BMs. If after eating the BMs increase by more than 2.2mM from fasting baseline it may be useful to test further.

Answer 960

D. Diabetic - WHO criteria for diagnosing Diabetes courtesy of cheese and onion :- symptoms (vaginal thrush is one..impaired immune system) + raised venous glucose detected on one occasion. OR 2 separate raised BMs (fasting >7mM or random >11.1mM etc..) OGTT: the patient fasts for 8-14 hours and baseline glucose is measured (usually around 7am) 1.75g per kg body weight glucose solution is given (up to 75g) drunk quickly and BMs are recorded again at 2hrs.

Answer 961

2. 40% - So although HbA1c can not be used diagnostically in diabetes it represents the amount of endogenous glycation occuring in vivo . Glycation produces Advanced glycation end products which are responsible for damaging vasa nervorum etc causing microvascular complications of diabetes. Lens protein Glycation is also responsible for the increased occurrence of catract in Diabetics.

Answer 962

B. Pancreatitis - This patient is an alcoholic and his management points to abdominal pathology which in his case is acute pancreatitis: any pathology affecting the pancreas can disrupt it's endocrine function..surgery, trauma chemical inflammation etc. here this has affected insulin production

Answer 963

C. Drug induced - this patient has rheumatoid arthritis a long standing inflammatory disease for which she no doubt has been placed on oral prednisolone...a side effect of corticosteroids is hyperglycaemia, mimicking the action of endogenous cortisol. The answer is drug induced as opposed to cushing's syndrome because no other signs of Cushing's are mentioned at all.

Answer 964

E. Pituitary adenoma - this patient is young and in a cardiology clinic..none of the options point to congenital heart disease however. The patient has macroglossia and is being treated with octreotide (a somatostatin analogue). This drug is useful in suppressing over active pituitaries, in this case once producing excess growth hormone. The most common cause would be an adenoma and his symptoms would include cardiomegaly and hypertension along with hyperglycaemia.

Answer 965

D. Lung cancer - This patient has small cell lung cancer which is causing him to suffer from Lambert-Eaton myasthenic syndrome. This is an auto-immune condition where calcium channel antibodies inhibit the release of acetylcholine from the calcium channels' pre-synaptic nerve terminal, thus causing muscle weakness. This condition resembles myasthenia gravis except the weakness improves with repeated activity, where it worsens in MG. Also, the respiratory and facial muscles are usually not involved or if they are it is not so severe as in MG.

Answer 966

H. PCOS - Young and obese may point to TIIDM however she has been prescribed the OCP for years.This is to manage the androgen excess resulting from her PCOS. Evidence for this is her thinning hair and oily skin coupled with her abdominal ultrasound used to diagnose her condition. Insulin resitance, obesity, dyslipidaemia and oligoovulation are all features of this condition.

Answer 967

1. Captopril - No mention is made of any arryhthmia so Amiodarone/ Digoxin aren't necessarily required. The man has impaired fasting glucose and so is at risk of progressing to diabetes. Thiazide diuretics are recognised as causing iatrogenic Diabetes Mellitus and so are unsuitable here. This man has LVF without Atrial fib and so an ACE-i' is first line therapy.They relieve the pre and afterload on the heart reducing progression to Left ventricular dilatation. It has also been shown that patients with Impaired Fasting Glucose and heart failure treated with ACE-i drugs are at much less risk of progression to diabetes over the short term at least (3% vs~50%).

Answer 968

G. Gliclazide

Answer 969

F. Dumping syndrome

Answer 970

D. Insulinoma

Answer 971

E. Insulin

Answer 972

B. Alcohol

Answer 973

F. Waterhouse-Friderichsen syndrome - Adrenal haemorrhage following massive gram negative meningitis

Answer 974

2. External carotid

Answer 975

C. Amiodarone - Any patient with a history of miscarriage should be investigated for thyroid dysfunction especially hpothyroidism. In this ladies case she has been taking amiodarone as an anti-arrhythmic long term, Amiodarone is one of several drugs that can cause hyper/hypothyroidism.

Answer 976

D. External neck irradiation - The man's history points to oral cancer, for which he would have received a wide local excision most likely. This would have been followed up with radiotherapy to the local nodes and tissues of the neck. In this gentleman the radiation has caused his thyroid to become underactive hence the lethargy and tiredness. The tingling in his hands at night is a classic presentation of Carpal tunnel syndrome (median nerve compression at the flexor retinaculum) which has an increased incidence in patients with hypothyroidism.

Answer 977

B. Hasimoto's thyroiditis - This patient has signs of hypothyroidism; depressed mood and impaired taste. Also she has hypothyroid myopathy, evidenced both symptomatically and biochemically by the elevated creatine kinase. Further to this it is worth noting that hypothyroidism has other effects that can be seen on bloodwork. Amongst these are anaemia and hypercholesterolaemia. It is the thyroid that moves on swallowing and on examination hers is rubbery and firm, this is typical of hashimoto's thyroiditis which is one of the leading causes of hypothyroidism in the uk. De quervains is not an option owing to lack of preceding viral infection and no tenderness over the neck. Hashimoto's thyroiditis An Autoimmune thyroiditis with associations with HLA -DR5 (and some conditions such as Turner's syndrome, T1DM and pernicious anemia) that shows T - lymphocyte infiltration of the gland and reactive antibodies against the TSH receptor and less commonly Thyroglobulin. It is most prevalent in middle aged women and is a prominent cause of goitre, due to the infiltration of the gland and elevated TSH levels (since T4 production is decreased). Note that at presentation most patients are euthyroid, although around half end up hypo due to destruction of the thyroid. It is possible to be toxic with hasimotos. Hashitoxicosis however is rare and can be transient. Treatment is generally thyroxine T4 given at doses sufficient to suppress endogenous T4 production and minimise TSH, this is done to lessen the risk of a goitre forming and subsequently growing.

Answer 978

A. Myxoedema coma - This patient is at the severe end of the hypothyroid spectrum. She has depressed level of consciousness (this can descend into frank coma) and is hypoventilating, other features that can present in this serious complication are bradycardia, and hyponatraemia. Further to this a massive proportion are hypothermic (core temp

Answer 979

H. Thyroxine abuse - This patient is not worried by her weight loss as it is self induced, she has no features that confirm grave's disease though she is thyrotoxic...the mother's condition is not stated but you can deduce that the girl has access to thyroxine which she is abusing for it's metabolic properties.

Answer 980

D. Reidel's thyroiditis - Riedel's is typically described as Iron hard or 'woody'. It is of an autoimmune origin and 25-50% of those affected are hypothyroid. The presenation is of a painless lump in the neck that can also cause local compressive symptoms. ie. Tracheal/oesophageal compression..stridor etc. Since the lump is hard and craggy it can be easily mistaken for carcinoma, however there is no lymphadenopathy in this patient or general cancer symptomology. Riedel's can be associated with sclerosing cholangitis and peculiarly responds to Tamoxifen, there can also be use for steroids.

Answer 981

C. Graves disease - anxious and with opthalmoplegia, immediately you are thinking thyrotoxic and more specifically Grave's as opthalmopathy is almost always associated with this particular pathology. The anti TSH receptor antibodies are a finding in Graves and they are likely responsible for the opthalmopathy and pre-tibial myxoedema sometimes seen. Thyroid stimulating immunoglobulin (TSI) is also implicated. A Radio-idoine uptake scan of the thyroid would show a 'hot' gland with diffuse symmetrical enlargement.

Answer 982

G. Early De Quervains thyroiditis - preceeding viral infection already points towards De quervains. The fine needle aspirate shows giant cells which are collections of macrophages which form in response to infection or foreign bodies. In this case the gentleman is anxious suggesting he is thyrotoxic, this is reinforced by the low uptake on radio iodine scanning. In the early stages of De Quervain's thyroiditis viral infiltration of the gland leads to destruction and release of preformed T4/T3, this leads to thyrototoxicosis. There is low uptake as the gland is responding to feedback, the raised serum T4 is a consequence of gland destruction not increased synthesis; as a result of this there is a second phase where the endogenous T4/T3 stores are depleted and the patient becomes hypothyroid. early management is symptomatic and generally supportive...beta blocakde.

Answer 983

A. MEN 2B - this fixed lump is accompanied by lymphadenopathy, warning bells go off that this may be malignant. There are local invasion signs also as confirmed by pemberton' Pemberton's sign - I put this into a thyroid exam, It can equally be justified in a respiratory examination. Basically you ask the patient to lift their arms straight above their head as high as possible and then listen for inspiratory stridor, look for facial flushing or distension of veins acorss the neck. You can ask the patient to tell you if they fell light headed when performing this test. It basically looks to see if there is evidence of SVC (superior venae cava) syndrome. If there is a large goitre or apical lung tumour for example impinging on the SVC, lifting the arms will lead to compression and cause congestion of blood in the head. This can be seen as a plethoric face, a cyanosed face, the patient may experience nausea and headache etc. Positive Pemberton's sign signifies thoracic inlet obstruction causes of which include: - Retrosternal goitre - Lung carcinomas - Aortic Aneurysms Thoracic inlet scan shows that a goitre is compressing structures such as the Superior vena cava. This seems to be maligant..Medullary thyroid carcinoma. however the rest of the patient must be taken into account..she has a marfanoid appearance and suggestion of other features of the syndrome. the lens work would be to correct dislocation, and the aortic valve work no doubt was to correct aortic root dilatation and valve incompetency seen in Marfan's. The nodules are neurofibromatoses typical of MEN 2b.s sign.

Answer 984

5. Graves disease

Answer 985

A. Potassium iodide - This patient is suffering with grave's disease and requires removal of her goitre as she is likely to have high circulating levels of TSI (thyroid stimulating immunoglobulin) which can cross the placenta and stimulate toxicosis in the foetus..leading to failure to thrive etc. The fetal thyroid status can be measured via it's heart rate. Carbimazole is standard management but if this doesn;t control maternal grave's surgery can be performed. As she has an audible bruit her goitre is likely extremely vascular and as she is pregnant the risks of excessive blood loss during thyroidectomy are multiple and serious. KI - potassium iodide can be given to saturate the thyroid and reduce vascularity pre surgery. Radioiodine is of course out of the question-risk of serious teratogenesis.

Answer 986

E. Surgical decompression - There is significant retroorbital oedema and fibrosis around the optic nerve, decompressive surgery should help relieve this and prevent further atrophy.

Answer 987

G. Stop treatment - The sore throat should be treated with a high index of suspicion as it might warn of neutropenic agranulocytosis, a serious side effect of Carbimazole. Bloods should be taken to determine if this has occurred and if so the drug should be stopped.

Answer 988

B. Radioiodine - sounds like a malignant thyroid has been removed (weight loss, dysphagia) and the thyroglobulin is mentioned as it is useful as a marker of neoplastic activity in patients with these malignancies (much like PSA in prostate Ca), carcinoembryonic antigen : CEA may also be used in the same way. Recurrence or suggestion of should be treated promptly with followup irradiation.

Answer 989

C. Propanolol - de quervains, sore, red thyroid caused by infection. It is early stage hence he is thyrotoxic. Management is mainly supportive..analgesia and beta blockade for the palpitations and anxiety.

Answer 990

B. Hashimoto's thyroiditis - Firm and diffuse goitre can be present physiologically (ie. preganancy), caused by Autoimmune disease : Grave's/Hasimoto's, drugs (sulphonylureas) or infective causes. The only information given is that she is hypothyroid. The most liley cause is thus Hashimoto's.

Answer 991

G. Multinodular goitre - Lumpy swelling and euthyroid (or borderline hyper- suppressed TSH with normal T4/T3) you would think multinodular goitre. She has no cancer B symptoms or infective history. These goitres are major causes of thoracic inlet obstruction and can also involve the laryngeal nerve causing hoarseness..a symptom that would also make you worry about infiltrative carcinoma. After palaption, ultrasound of the thyroid accompanied by Fine needle aspirate can be useful to check there are no malignant changes within the multinodular goitre. (worrying USS findings may be calcification of cysts - the so called 'halo' sign)

Answer 992

H. Medullary cell carcinoma - This patient has a maliganacy, the bone pain suggests it has metastasised (medullar cell carincoma frequently does so). Raised calcitonin is noted as medullary carcinoma originates from the parafollicular c cells of the thyroid, responsible for producing calcitonin as part of the calcium, homeostasis mechanism...raised calcitonin is fairly diagnostic. Thyroid malignancies: GOOD - differentiated (around 90% of all malignancies) : Papillary (80%) and Follicular (10%) carcinomas : these originate from the thyroxine producing stroma and commonly present as a solitary lump in a euthyroid patient. Infiltrative symptoms such as hoarseness and dysphagia may be seen..Surgical management +/- radioiodine followup (these tumours take up iodine quite well) is usually effective. Papillary tumours are slow growing and have excellent survical rates. BAD - from undifferentiated to anaplastic - Medullary cell Ca - aggressive and metastatic these tumours do not take up iodine well and are less curable by surgery. often linked with MEN or can be a familial neoplasm. The elderly are particularly affected by Lymphomas and Anaplastic carcinomas - both are rare and rapidly growing however biopsy to differentiate these is important here as lymphomas have a decent prognosis whilst anaplastic carcinomas are pretty much incurable.

Answer 993

2. Inferior oblique

Answer 994

A. Venous ulcer - Venous ulcers occur on a background of deep venous insufficiency. There is oedema and a brown skin discolouration due to leaching of pigments and haemosiderin deposition. In addition there may be lipodermatosclerosis and an inflammatory response, which is seen as an eczema-like thickening and hardening of the skin. The skin can also be drawn tightly around the ankle. Ulceration usually follows trauma and is usually on the medial gaiter region. The base has granulation tissue and is sloughy in nature and there is a sloping edge to the ulcer. The shape is often irregular. Look up some photos to help you remember. Once significant arterial disease is excluded (ulcers can have mixed components), the mainstay of treatment is with compression bandaging, appropriate dressings and treatment of any infection with antibiotics. Maggots can also be used and varicose veins should be treated where possible to reduce recurrence. If the ulcer is not healing, a biopsy should be considered (Marjolin’s ulcer).

Answer 995

J. Pyoderma gangrenosum - This is pyoderma gangrenosum, which presents with multiple lesions, most commonly affecting the lower extremity and is linked to UC although it can be seen less commonly in Crohn’s, and is also seen in conditions such as RA and the myeloid dyscrasias. These lesions start as tender papules or vesicles which develop into painful ulcers with a dusky purple edge and surrounding induration and erythema. The base may contain granulation tissue and lesions heal with atrophic scars.

Answer 996

N. Arterial ulcers - Arterial ulcers are deep and painful with a well defined edge, usually found on the shin or foot. They often have a pale base and a punched out appearance. The absent pulses and longstanding PVD with DM here is also indicative. There may be local changes such as cold peripheries, loss of hair, dusky cyanosis and toenail dystrophy. The surrounding skin is often white and shiny. It is typically most painful in bed and the pain is sometimes relieved by having the legs dependent. On examination, peripheral pulses may be absent or reduced like this case. An angiogram with contrast will define the lesion and determine whether it can be improved by surgical intervention. Pain often increases when the legs are at rest and elevated. They can occur between the webs of toes so it is important to always check these in your peripheral vascular examination.

Answer 997

E. Sickle cell disease - Adults with sickle cell anaemia may present with leg ulcers although it has to be said that it is very unusual for a person with sickle cell disease to reach adulthood without being aware of their diagnosis. The adult patient could present with unexplained haemolysis, possible intermittent episodes of pain due to vaso-occlusive crises, avascular necrosis and retinal haemorrhage too. The doppler derived ankle-brachial pressure index is normal here which points away from peripheral arterial disease, which in this relatively young person is unlikely in any case. The typical constellation of findings of venous ulcers are not seen (oedema, lipodermatosclerosis). Additionally the pain would appear to be out of proportion to a standard arterial or venous ulcer and instead sounds more like a the pain of a vaso-occulusive crisis, particularly with the recurrent nature. In real medicine, this is unlikely as it is rare for sickle cell anaemia to present this late, but the fact this lady is from sub-Saharan Africa is a key fact. From an epidemiological perspective, the prevalence is 10-30% in sub-Saharan Africa. Between 25 and 30% of newborns in western Africa are carriers of sickle cell trait. This patient here will need to have their peripheral blood film reviewed following by haemoglobin electrophoresis and HPLC. Sickle solubility is a rapid test which can also be done but will not differentiate sickle trait from sickle disease.

Answer 998

B. Diabetic ulcer - This is a case of diabetic neuropathy. This is a microvascular complication of DM and is characterised by peripheral nerve dysfunction. Complications range from the painless neuropathic ulcer described, at areas of the foot where there is weight loading (particularly the metatarsal heads), to the Charcot foot with severe architectural destruction of the foot. Foot ulceration is a common precusor to amputation. Foot care is crucial in DM. Examination should include peripheral pulses, reflexes and sensation to light touch with a 10g monofilament, vibration (128Hz tuning fork), pinprick and proprioception. Any neuropathic pain may be treated with medications like pregabalin and gabapentin (but unlikely to much effect).