Wednesday [16/09/2021]

Question 1

A newborn child is assessed. They are found to be in the 25th centile for their weight along with a systolic murmur heard best over the back. When feeling the femoral pulses the doctor notices that there is a radio-femoral delay. Which of the following may be causing these examination findings?

Answer 1

Turner’s syndrome

Radio-femoral delay is associated with coarctation of the aorta
This question is asking about a neonate presenting with a systolic murmur, low birth weight and radio-femoral delay, these are all characteristic features of coarctation of the aorta. Of the above conditions, only Turner’s syndrome has a strong association with coarctation of the aorta.

Question 2

What is CHARGE syndrome?

Answer 2

CHARGE syndrome is a genetic syndrome associated with some congenital heart defects. However, these are most commonly tetralogy of Fallot or ventricular septal defects.

Question 3

What is Klinefelter’s syndrome?

Answer 3

Klinefelter’s syndrome is a condition caused by 47, XXY (an extra X chromosome) which characteristically presents in slim tall males with infertility and lack of secondary sexual characteristics.

Question 4

Characteristic of patent ductus arteriosus?

Answer 4

A patent ductus arteriosus is a common congenital heart defect that will not cause radio-femoral delay. The characteristically associated murmur is a venous hum, which is a continuous murmur.

Question 5

Features of coarctation of aorta?

Answer 5

infancy: heart failure
adult: hypertension
radio-femoral delay
mid systolic murmur, maximal over back
apical click from the aortic valve
notching of the inferior border of the ribs (due to collateral vessels) is not seen in young children

Question 6

Associations of coarctation of aorta

Answer 6

Turner’s syndrome
bicuspid aortic valve
berry aneurysms
neurofibromatosis

Question 7

A 28-year-old man of African descent presents to his general practitioner with complaints of fever, reduced appetite and shortness of breath on exertion for the last 3 months.

As part of the initial workup, blood samples are taken which are significant for the following:

Calcium 2.9 mmol/L (2.1-2.6)

A chest x-ray is performed which is reported as showing bilateral hilar lymphadenopathy.

What is the most likely diagnosis?

Answer 7

Hypercalcaemia + bilateral hilar lymphadenopathy → ?sarcoidosis
This patient presented with symptoms of fever, reduced appetite and shortness of breath on exertion, which are suggestive of a systemic disease affecting the respiratory system. primarily. The fact that he is a young, male patient of African descent points to the diagnosis of sarcoidosis, which can present with the aforementioned symptoms and with an insidious onset (3 months).

Sarcoidosis, the correct answer, is made even more likely due to the combination of findings of hypercalcaemia and bilateral hilar lymphadenopathy, which are typical of the condition.

Question 8

What would make diagnosis more likely leprosy?

Answer 8

Leprosy is incorrect, as lack of skin and neurological manifestations such as paresthesias makes this condition less likely in this case

Question 9

Features of sarcoidosis

Answer 9

acute: erythema nodosum, bilateral hilar lymphadenopathy, swinging fever, polyarthralgia
insidious: dyspnoea, non-productive cough, malaise, weight loss
skin: lupus pernio
hypercalcaemia: macrophages inside the granulomas cause an increased conversion of vitamin D to its active form (1,25-dihydroxycholecalciferol)

Question 10

Which populations sarcoidsosi more common in?

Answer 10

Sarcoidosis is a multisystem disorder of unknown aetiology characterised by non-caseating granulomas. It is more common in young adults and in people of African descent

Question 11

Syndromes associated with sarcoidosis?

Answer 11

Lofgren’s syndrome is an acute form of the disease characterised by bilateral hilar lymphadenopathy (BHL), erythema nodosum, fever and polyarthralgia. It usually carries an excellent prognosis

In Mikulicz syndrome* there is enlargement of the parotid and lacrimal glands due to sarcoidosis, tuberculosis or lymphoma

Heerfordt’s syndrome (uveoparotid fever) there is parotid enlargement, fever and uveitis secondary to sarcoidosis

Question 12

A 38-year-old man was admitted to the surgical receiving unit by his GP with sudden onset epigastric pain. His is a known alcoholic and is also overweight, but has no other past medical history. He has severe nausea and vomiting, unable to tolerate any food or drink.

His blood results come back as below:

Hb 154 g/L Male: (135-180)
Female: (115-160)
Platelets 290 * 109/L (150 - 400)
WBC 27.8 * 109/L (4.0 - 11.0)

Na+ 140 mmol/L (135 - 145)
K+ 3.8 mmol/L (3.5 - 5.0)
Adj Ca2+ 1.8mmol/L (2.2 - 2.6)
Urea 9.1 mmol/L (2.0 - 7.0)
Creatinine 102 µmol/L (55 - 120)
CRP 13 mg/L (< 5)

ALP 74 U/L (30-130)
ALT 27 U/L (<41)
Bilirubin 12 µmol/L (<21)

Which differential is most likely to account for his abnormal results?

Answer 12

Acute pancreatitis may cause hypocalcemia

Acute pancreatitis is the most likely diagnosis as it is the most likely to result in a hypocalcaemia. It can also can a raised WBC and CRP due to the inflammatory process.

Alcoholic liver disease is unlikely as the LFTs are normal and this would not account for the hypocalcaemia. It would also be unlikely to present in such an acute manner.

Peptic ulcers can also cause epigastric pain however would not account for the hypocalcaemia or such raised inflammatory markers.

Ruptured abdominal aortic aneurysm may present with sudden onset epigastric pain radiating to the back and is important to consider. However it would be accompanied by a low haemoglobin and would not normally cause hypocalcaemia.

Spontaneous bacterial peritonitis occurs as a complication of liver cirrhosis and would cause raised white cells however it would be unlikely with normal LFTs and hypocalcaemia is not a typically presentation of this.

Question 13

You are asked to review a 76-year-old woman with metastatic bowel cancer. She was admitted four days prior with abdominal pain and has not opened her bowels for the last six days.

She is receiving diamorphine via a syringe driver. However, she is still having intermittent severe abdominal pain.

Which of the following medications should be added to her syringe driver?

Answer 13

Syringe drivers: respiratory secretions & bowel colic may be treated by hyoscine hydrobromide, hyoscine butylbromide, or glycopyrronium bromide

This patient is experiencing colicky pain secondary to mechanical obstruction caused by bowel cancer.

Hyoscine butylbromide (also known as scopolamine butylbromide) is the correct answer in this case. It is an antimuscarinic drug that reduces smooth muscle contractions. It is therefore useful in the treatment of colicky pain.

Metoclopramide is incorrect as it is a prokinetic antiemetic. It will therefore worsen the pain by promoting bowel contraction against the obstruction.

Codeine phosphate is incorrect as the patient is already receiving diamorphine via her syringe driver. Further analgesia should be added by increasing the dose of diamorphine, not adding another opioid.

Midazolam is incorrect as it is a sedative which would not address the underlying cause of her symptoms.

Gabapentin is incorrect at it is not indicated for the treatment of pain due to gastrointestinal obstruction.

Question 14

What type of drug is metoclopramide and what will it worsen?

Answer 14

Metoclopramide is incorrect as it is a prokinetic antiemetic. It will therefore worsen the pain by promoting bowel contraction against the obstruction.

Question 15

When are syringe drivers considered?

Answer 15

A syringe driver should be considered in the palliative care setting when a patient is unable to take oral medication due to nausea, dysphagia, intestinal obstruction, weakness or coma. In the UK there are two main types of syringe driver: 
Graseby MS16A (blue): the delivery rate is given in mm per hour 
Graseby MS26 (green): the delivery rate is given in mm per 24 hours

Question 16

Which syringe driver drugs should you give with sodium chloride rather than water?

Answer 16

The majority of drugs are compatible with water for injection although for the following drugs sodium chloride 0.9% is recommended:
granisetron
ketamine
ketorolac
octreotide
ondansetron

Question 17

Commonly used drugs for N and V syringe driver?

Answer 17

nausea and vomiting: cyclizine, levomepromazine, haloperidol, metoclopramide

Question 18

Commonly used drugs for respiratory secretions/bowel colic syringe driver?

Answer 18

hyoscine hydrobromide, hyoscine butylbromide, or glycopyrronium bromide.

Question 19

Commonly used drugs for agitation/restlessness syringe driver?

Answer 19

midazolam, haloperidol, levomepromazine

Question 20

Commonly used drugs for pain syringe driver?

Answer 20

diamorphine is the preferred opioid

Question 21

Mixing and compatibility issues with syringe drivers drugs?

Answer 21

diamorphine is compatible with the majority of other drugs used including cyclizine*, dexamethasone, haloperidol, hyoscine butylbromide, hyoscine hydrobromide, levomepromazine, metoclopramide, midazolam
cyclizine is incompatible with a number of drugs including clonidine, dexamethasone, hyoscine butylbromide (occasional), ketamine, ketorolac, metoclopramide, midazolam, octreotide, sodium chloride 0.9%

*precipitation may be seen at higher doses

Question 22

A 61-year-old woman attends general practice with her daughter, who believes her mum has been looking ‘yellow’ recently. On observation, the patient is visibly jaundiced and her abdomen is distended.

On questioning, the patient describes feeling increasingly bloated over the past month and has found ‘small red dots’ appearing on her upper chest, these disappear when pressed on, and subsequently, refill from the centre. She is uncertain if she has lost weight but she does describe her clothes seeming baggier over the past few months.

She has a background of type 2 diabetes, hypertension, and liver cirrhosis secondary to chronic hepatitis B. She admits to missing several follow up appointments with gastroenterology over the past couple of years.

What is the most likely cause of the patient’s deterioration?

Answer 22

Hepatocellular carcinoma

Deterioration in patient with hepatitis B - ? hepatocellular carcinoma

Hepatocellular carcinoma (HCC) is correct. The patient is presenting with decompensated liver disease. HCC is a known cause of this. Further, hepatitis B is known to be a risk factor for hepatocellular carcinoma, and the patient’s history of probable weight loss, anorexia, and missing follow up appointments fits this picture

Question 23

How often should patients Dx with cirrhosis have surviellance?

Answer 23

Patients diagnosed with cirrhosis should have surveillance at six-monthly intervals for HCC consisting of abdominal ultrasound and measuring AFP levels

Question 24

What is hepatitis B a risk factor for?

Answer 24

Further, hepatitis B is known to be a risk factor for hepatocellular carcinoma, and the patient’s history of probable weight loss, anorexia, and missing follow up appointments fits this picture

Question 25

Mx options for patients Dx with liver cirrhosis

Answer 25

Management options include surgical resection in early stages, radiofrequency ablation, transarterial chemoembolization multikinase inhibitors such as sorafenib, and liver transplantation.

Question 26

Difference between compensated and decompensated liver disease?

Answer 26

Compensated: When you don’t have any symptoms of the disease, you’re considered to have compensated cirrhosis. Decompensated: When your cirrhosis has progressed to the point that the liver is having trouble functioning and you start having symptoms of the disease, you’re considered to have decompensated cirrhosis.

Question 27

Signs of decompensated liver disease

Answer 27

fatigue easy bruising and bleeding itching yellowing of the skin and eyes (jaundice) fluid build-up in the abdomen (ascites) fluid build-up in the ankles and legs abdominal pain nausea fever brownish or orange urine loss of appetite or weight loss confusion, memory loss, or insomnia (hepatic encephalopathy)

Question 28

Signs of hepatitis D infectino

Answer 28

Hepatitis D infection is incorrect, as although it can cause a decompensated liver failure picture, there are no signs pointing to this in the clinical scenario. This would typically present with features of acute hepatitis such as fever, nausea and vomiting, abdominal pain, jaundice, dark urine and pale stools, but rarely progresses to chronic hepatitis.

Question 29

What can hepatitis D in someone with heptaitis B cause?

Answer 29

Fulminant hepatitis is a rare syndrome of massive necrosis of the liver parenchyma. This can be due to infection with certain hepatitis viruses e.g. hepatitis D co-infection in someone with hepatitis B. Hepatitis A can also cause fulminant hepatitis, although this is more rare. Other causes include toxic agents or drug-induced injury e.g. with acetaminophen. This presents with rapid deterioration, including coagulopathy due to liver failure, disseminated intravascular coagulation, and hepatorenal syndrome. This may progress to coma and cerebral oedema over a period of several days to weeks.

Question 30

What does pancreatic adenocarconma classical present as?

Answer 30

Pancreatic adenocarcinoma classically presents with painless jaundice. Although, it is common also to present in non-specific ways such as with anorexia, weight loss, epigastric pain, atypical back pain. There may also be loss of exocrine function e.g steatorrhoea and loss of endocrine function e.g diabetes mellitus. Some features of this fit with the clinical presentation, such as jaundice, weight loss and anorexia, however, the other signs of hepatic decompensation do not fit.

Question 31

What is cholangiocarcinoma?

Answer 31

Bile duct cancer, also called cholangiocarcinoma, is a cancer that's found anywhere in the bile ducts. The bile ducts are small tubes that connect different organs. They are part of the digestive system.

Question 32

Cholangiocarnoma presentation?

Answer 32

Cholangiocarcinoma typically presents with persistent jaundice, biliary colic pain, Sister Mary Joseph nodes (periumbilical lymphadenopathy) and Courvoisier's sign (a palpable mass in the right upper quadrant).

Question 33

What is hepatitis B?

Answer 33

Hepatitis B is a double-stranded DNA hepadnavirus and is spread through exposure to infected blood or body fluids, including vertical transmission from mother to child.

Question 34

Incubation period for hepatitis B

Answer 34

The incubation period is 6-20 weeks.

Question 35

Features of hepatitis B

Answer 35

The features of hepatitis B include fever, jaundice and elevated liver transaminases.

Question 36

Cx of hepatitis B infection

Answer 36

chronic hepatitis (5-10%). 'Ground-glass' hepatocytes may be seen on light microscopy fulminant liver failure (1%) hepatocellular carcinoma glomerulonephritis polyarteritis nodosa cryoglobulinaemia

Question 37

Who should be immunised against hepatitis B?

Answer 37

Immunisation against hepatitis B (please see the Greenbook link for more details) children born in the UK are now vaccinated as part of the routine immunisation schedule. This is given at 2, 3 and 4 months of age at risk groups who should be vaccinated include: healthcare workers, intravenous drug users, sex workers, close family contacts of an individual with hepatitis B, individuals receiving blood transfusions regularly, chronic kidney disease patients who may soon require renal replacement therapy, prisoners, chronic liver disease patients contains HBsAg adsorbed onto aluminium hydroxide adjuvant and is prepared from yeast cells using recombinant DNA technology around 10-15% of adults fail to respond or respond poorly to 3 doses of the vaccine. Risk factors include age over 40 years, obesity, smoking, alcohol excess and immunosuppression testing for anti-HBs is only recommended for those at risk of occupational exposure (i.e. Healthcare workers) and patients with chronic kidney disease. In these patients anti-HBs levels should be checked 1-4 months after primary immunisation the table below shows how to interpret anti-HBs levels:

Question 38

How to interpret anti-HB levels?

Answer 38

Anti-HBs level (mIU/ml) Response \> 100 Indicates adequate response, no further testing required. Should still receive booster at 5 years 10 - 100 Suboptimal response - one additional vaccine dose should be given. If immunocompetent no further testing is required \< 10 Non-responder. Test for current or past infection. Give further vaccine course (i.e. 3 doses again) with testing following. If still fails to respond then HBIG would be required for protection if exposed to the virus

Question 39

Mx of hepatitis B

Answer 39

pegylated interferon-alpha used to be the only treatment available. It reduces viral replication in up to 30% of chronic carriers. A better response is predicted by being female, \< 50 years old, low HBV DNA levels, non-Asian, HIV negative, high degree of inflammation on liver biopsy whilst NICE still advocate the use of pegylated interferon firstl-line other antiviral medications are increasingly used with an aim to suppress viral replication (not in a dissimilar way to treating HIV patients) examples include tenofovir, entecavir and telbivudine (a synthetic thymidine nucleoside analogue)

Question 40

On a ward round you see a 70-year-old female who’s recently had a resection of her bowel for colon cancer, and has been bed bound for several days. She complains of a sore, red calf, and a feeling of breathlessness. A pulmonary embolism is suspected, and a CT-pulmonary angiogram (CTPA) is ordered, however it comes back negative for a pulmonary embolism (PE). What is the next most appropriate action to aid diagnosis?

Answer 40

Perform a proximal leg vein doppler ultrasound: - Investigating suspected PE: if the CTPA is negative then consider a proximal leg vein ultrasound scan if DVT is suspected. Perform a ventilation-perfusion (V/Q) scan is incorrect because it would not be completed before a leg vein ultrasound. In diagnosing a PE, a ventilation perfusion (V/Q) scan can be performed after a negative CTPA in certain clinical circumstances, though this is rare as CTPA is considered the gold standard. However a proximal leg vein doppler should be performed first (it is faster, cheaper, and exposes the patient to less radiation). Proximal leg vein CT-venogram is incorrect, as it is not used for diagnosing DVT, and is mostly reserved for research due to the primary role of ultrasound as a faster, cheaper, radiation-free alternative. Perform an emergency electrocardiogram (ECG) is incorrect, because while it is appropriate to do an ECG, this would not be diagnostic of a PE and would not diagnose the painful, erythematous calf. However, an ECG would invariably be done to rule out certain dyspnoea differentials, if all else came back negative. Repeat CTPA after 24 hours is incorrect because CTPA is very unlikely to change after 24 hours, and would also re-expose the patient to significant amounts of radiation. While an ECG will undoubtedly be performed in such a scenario, PE can not be diagnosed on ECG and must be further investigated, usually with doppler ultrasound and/or a CTPA.

Question 41

How often is it that patients present iwhr textbook triad Sx?

Answer 41

We know from experience that few patients (around 10%) present with the textbook triad of pleuritic chest pain, dyspnoea and haemoptysis. Pulmonary embolism can be difficult to diagnose as it can present with virtually any cardiorespiratory symptom/sign depending on its location and size.

Question 42

Studies suggest actually which features more likely for a PE?

Answer 42

The PIOPED study1 in 2007 looked at the frequency of different symptoms and signs in patients who were diagnosed with pulmonary embolism. The relative frequency of common clinical signs is shown below: Tachypnea (respiratory rate \>20/min) - 96% Crackles - 58% Tachycardia (heart rate \>100/min) - 44% Fever (temperature \>37.8°C) - 43% It is interesting to note that the Well's criteria for diagnosing a PE use tachycardia rather than tachypnoea. All patients with symptoms or signs suggestive of a PE should have a history taken, examination performed and a chest x-ray to exclude other pathology.

Question 43

PE rule out criteria? [PERC]

Answer 43

NICE updated their guidelines on the investigation and management of venous thromboembolism (VTE) in 2020. One of the key changes was the use of the pulmonary embolism rule-out criteria (the PERC rule) a copy of criteria can be found in the image below all the criteria must be absent to have negative PERC result, i.e. rule-out PE this should be done when you think there is a low pre-test probability of PE, but want more reassurance that it isn't the diagnosis this low probability is defined as \< 15%, although it is clearly difficult to quantify such judgements a negative PERC reduces the probability of PE to \< 2% if your suspicion of PE is greater than this then you should move straight to the 2-level PE Wells score, without doing a PERC

Question 44

What to do if PE is likely [mrep than 4 points]?

Answer 44

If a PE is 'likely' (more than 4 points) arrange an immediate computed tomography pulmonary angiogram (CTPA) If there is a delay in getting the CTPA then interim therapeutic anticoagulation should be given until the scan is performed. interim therapeutic anticoagulation used to mean giving low-molecular-weight heparin NICE updated their guidance in 2020. They now recommend using an anticoagulant that can be continued if the result is positive. this means normally a direct oral anticoagulant (DOAC) such as apixaban or rivaroxaban - if the CTPA is positive then a PE is diagnosed - if the CTPA is negative then consider a proximal leg vein ultrasound scan if DVT is suspected

Question 45

What to do if PE is unliekly [4 points or less?]

Answer 45

arranged a D-dimer test if positive arrange an immediate computed tomography pulmonary angiogram (CTPA). If there is a delay in getting the CTPA then give interim therapeutic anticoagulation until the scan is performed if negative then PE is unlikely - stop anticoagulation and consider an alternative diagnosis

Question 46

CTPA or V/Q scan?

Answer 46

The consensus view from the British Thoracic Society and NICE guidelines is as follows: CTPA is now the recommended initial lung-imaging modality for non-massive PE. Advantages compared to V/Q scans include speed, easier to perform out-of-hours, a reduced need for further imaging and the possibility of providing an alternative diagnosis if PE is excluded if the CTPA is negative then patients do not need further investigations or treatment for PE V/Q scanning may be used initially if appropriate facilities exist, the chest x-ray is normal, and there is no significant symptomatic concurrent cardiopulmonary disease. V/Q scanning is also the investigation of choice if there is renal impairment (doesn't require the use of contrast unlike CTPA)

Question 47

Sensitivty of D-dimer for PE?

Answer 47

D-dimers sensitivity = 95-98%, but poor specificity age-adjusted D-dimer levels should be considered for patients \> 50 years

Question 48

ECg changes on PE?

Answer 48

the classic ECG changes seen in PE are a large S wave in lead I, a large Q wave in lead III and an inverted T wave in lead III - 'S1Q3T3'. However, this change is seen in no more than 20% of patients right bundle branch block and right axis deviation are also associated with PE sinus tachycardia may also be seen

Question 49

CXR in PE

Answer 49

a chest x-ray is recommended for all patients to exclude other pathology however, it is typically normal in PE possible findings include a wedge-shaped opacification

Question 50

V/Q scan in PE

Answer 50

sensitivity of around 75% and specificity of 97% other causes of mismatch in V/Q include old pulmonary embolisms, AV malformations, vasculitis, previous radiotherapy COPD gives matched defects

Question 51

CTPA scan in PE?

Answer 51

peripheral emboli affecting subsegmental arteries may be missed

Question 52

A 62-year-old female with a history of Grave's disease presents with nausea, lethargy and abdominal pain. Examination reveals increased pigmentation of the buccal mucosa. Which one of the following is the best investigation to confirm the suspected diagnosis of Addison's?

Answer 52

The short synacthen test is the best test to diagnose Addison's disease In a patient with suspected Addison's disease the definite investigation is an ACTH stimulation test (short Synacthen test). Plasma cortisol is measured before and 30 minutes after giving Synacthen 250ug IM. Adrenal autoantibodies such as anti-21-hydroxylase may also be demonstrated.

Question 53

If ACTH stimulation is not readily available, how can cortisol be tested?

Answer 53

If an ACTH stimulation test is not readily available (e.g. in primary care) then sending a 9 am serum cortisol can be useful: \> 500 nmol/l makes Addison's very unlikely \< 100 nmol/l is definitely abnormal 100-500 nmol/l should prompt a ACTH stimulation test to be performed

Question 54

Associated electrolyte distrubance in Addison's diseas?

Answer 54

Associated electrolyte abnormalities are seen in around one-third of undiagnosed patients: hyperkalaemia hyponatraemia hypoglycaemia metabolic acidosis

Question 55

A 34-yr-old man is brought to the Emergency Department by his wife after becoming increasingly drowsy and confused. He is normally fit and well, only taking methotrexate for his psoriasis. CT head shows multiple ring enhanced lesions. A diagnosis of toxoplasmosis is suspected. What is the most appropriate treatment?

Answer 55

Immunocompromised patients with toxoplasmosis are treated with pyrimethamine plus sulphadiazine. Pyrimethamine is used to treat toxoplasmosis but in immunocompromised patients it is used in combination with sulphadiazine.

Question 56

What is toxoplasmosis?

Answer 56

Toxoplasma gondii is an obligate intracellular protozoan that infects the body via the gastrointestinal tract, lung or broken skin. It's oocysts release trophozoites which migrate widely around the body including to the eye, brain and muscle. The usual animal reservoir is the cat, although other animals such as rats carry the disease.

Question 57

Most infections of toxoplasmosis present how?

Answer 57

Most infections are asymptomatic. Symptomatic patients usually have a self-limiting infection, often having clinical features resembling infectious mononucleosis (fever, malaise, lymphadenopathy). Other less common manifestations include meningoencephalitis and myocarditis.

Question 58

Ix for toxoplasmosis?

Answer 58

Serology is the investigation of choice

Question 59

Tx for toxoplasmosis?

Answer 59

No Tx is usually required unless severe infection or patient is immunosuppressed

Question 60

How often is cerebral toxoplasmosis is patients with HIV?

Answer 60

Cerebral toxoplasmosis accounts for around 50% of cerebral lesions in patients with HIV

Question 61

Sx, Ix and Mx for HIV toxoplasmosis?

Answer 61

constitutional symptoms, headache, confusion, drowsiness CT: usually single or multiple ring-enhancing lesions, mass effect may be seen management: pyrimethamine plus sulphadiazine for at least 6 weeks

Question 62

What is congential toxoplasmosis and how can it present?

Answer 62

Congenital toxoplasmosis is due to transplacental spread from the mother. It causes a variety of effects to the unborn child including neurological damage cerebral calcification hydrocephalus chorioretinitis ophthalmic damage retinopathy cataracts

Question 63

A 23-year-old female attends an appointment with her GP. She complains of a 6-month history of abdominal bloating, pain, and diarrhoea. She has a background of type 1 diabetes mellitus. The GP suspects she may have coeliac disease and arranges some blood tests, the result of which are below: tTG-IgA (tissue transglutaminase antibodies) 8U/ml (7-10) Total IgA 1.5g/L (0.8 - 3.0) When the GP discusses the results with the patient, she informs him that she had excluded gluten from her diet for 2 months before this blood test. Based on this information, what is the next most appropriate investigation?

Answer 63

Repeat tTG-iIgA in 6w once patient has reintroduced gluten into their diet. The correct answer is to repeat the tTG-IgA in 6 weeks time, once the patient has reintroduced gluten into their diet. NICE guidelines suggest the patient reintroduce gluten 6 weeks prior to coeliac screening. This is because it is possible to get normal ranges of tTG-IgA (tissue transglutaminase antibodies) if the patient has not eaten gluten in the past 6 weeks

Question 64

Which bowel disease is T1DM a RF for?

Answer 64

This patient has type 1 diabetes mellitus, which is a risk factor for coeliac disease, therefore it is important to repeat the test in this patient to exclude coeliac disease.

Question 65

What can repeated exposure to gluten cause to patients with coleiac?

Answer 65

Coeliac disease is caused by sensitivity to the protein gluten. Repeated exposure leads to villous atrophy which in turn causes malabsorption.

Question 66

Conditions associated with coeliac disease?

Answer 66

Conditions associated with coeliac disease include dermatitis herpetiformis (a vesicular, pruritic skin eruption) and autoimmune disorders (type 1 diabetes mellitus and autoimmune hepatitis).

Question 67

Dx of coeliac disease?

Answer 67

Diagnosis is made by a combination of serology and endoscopic intestinal biopsy. Villous atrophy and immunology normally reverses on a gluten-free diet.

Question 68

Serology for coeliac disease?

Answer 68

``` tissue transglutaminase (TTG) antibodies (IgA) are first-choice according to NICE endomyseal antibody (IgA) needed to look for selective IgA deficiency, which would give a false negative coeliac result anti-gliadin antibody (IgA or IgG) tests are not recommended by NICE anti-casein antibodies are also found in some patients ```

Question 69

Endoscopic intestinal biopsy for patients with coeliac

Answer 69

the 'gold standard' for diagnosis - this should be performed in all patients with suspected coeliac disease to confirm or exclude the diagnosis traditionally done in the duodenum but jejunal biopsies are also sometimes performed findings supportive of coeliac disease: villous atrophy crypt hyperplasia increase in intraepithelial lymphocytes lamina propria infiltration with lymphocytes Rectal gluten challenge has been described but is not widely used

Question 70

A 68-year-old male patient is admitted to the emergency department by ambulance with sudden onset severe chest pain. On arrival, he is drowsy and unable to provide a history. His wife tells you that he has had severe gastroenteritis for the past week and has been vomiting profusely, he has been unable to tolerate any oral intake for the past 2 days. His observations are as follows: heart rate 122/min, BP 96/88mmHg, oxygen saturations 96% on room air, respiratory rate 16/min, temperature 36.5ºC and, GCS 11. On examination, heart and lung sounds are normal but you can feel a crackling sensation when palpating the chest over the sternum. A 12-lead ECG is performed which shows sinus tachycardia. What is the most likely underlying diagnosis?

Answer 70

Vomiting → severe chest pain, shock - Boerhaave syndrome The correct answer is Boerhaave syndrome. This is an acute oesophageal rupture due to extreme vomiting. The patient has a triad of prolonged and violent vomiting, sudden onset chest pain and signs of shock (hypotension and tachycardia), indicating Boerhaave syndrome. The patient additionally has examination findings of surgical emphysema, with the crackling sensation over the chest on palpation, which is a clinical finding in oesophageal rupture.

Question 71

How would aortic dissection present?

Answer 71

Aortic dissection is incorrect. This is where the innermost layer of the aorta (intima) tears and blood enters the middle layer (media) of the aorta. Aortic dissection would present with sudden onset chest pain or back pain, often described as tearing in nature and may also present with signs of haemodynamic compromise and shock. However, it is unlikely given the patient’s history of severe vomiting and examination findings of surgical emphysema.

Question 72

how would pericarditis present? What would ECG changes be?

Answer 72

Pericarditis is incorrect. Pericarditis is an inflammation of the pericardium. This presents with pleuritic chest pain, often sudden onset and improved by sitting up and worsened by lying down. Examination findings include a pericardial friction rub heard on auscultation of the heart not palpable like the ‘crackling’ feeling of surgical emphysema as found in this patient. ECG changes such as saddle-shaped ST elevation would additionally likely be present.

Question 73

What is type 2 myocardial infarction?

Answer 73

Type 2 myocardial infarction is incorrect. This is a myocardial infarction where there is no unstable coronary artery disease or plaque formation, but the ischaemia is caused by a mismatch between myocardial oxygen demand and supply.

Question 74

Cause of type 2 myocardial infarction

Answer 74

Severe dehydration can be a cause of type 2 myocardial infarctions and whilst the patient is likely severely dehydrated and has sudden onset chest pain, there are no dynamic changes on a 12-lead ECG suggestive of ischaemia nor would it account for the examination findings of surgical emphysema.

Question 75

Go through 3 less common oeosphageal disroders and their presentation

Answer 75

Plummer-Vinson syndrome Triad of: dysphagia (secondary to oesophageal webs) glossitis iron-deficiency anaemia Treatment includes iron supplementation and dilation of the webs Mallory-Weiss syndrome Severe vomiting → painful mucosal lacerations at the gastroesophageal junction resulting in haematemesis. Common in alcoholics. Boerhaave syndrome Severe vomiting → oesophageal rupture

Question 76

A 26-year-old newly qualified nurse presents as she has developed a bilateral erythematous rash on both hands. She has recently emigrated from the Philippines and has no past medical history of note. A diagnosis of contact dermatitis is suspected. What is the most suitable to test to identify the underlying cause?

Answer 76

The skin patch test is useful in this situation as it may also identify for irritants, not just allergens. Useful for contact dermatitis. Around 30-40 allergens are placed on the back. Irritants may also be tested for. The patches are removed 48 hours later with the results being read by a dermatologist after a further 48 hours

Question 77

When is skin prick used for allergies?

Answer 77

Most commonly used test as easy to perform and inexpensive. Drops of diluted allergen are placed on the skin after which the skin is pierced using a needle. A large number of allergens can be tested in one session. Normally includes a histamine (positive) and sterile water (negative) control. A wheal will typically develop if a patient has an allergy. Can be interpreted after 15 minutes Useful for food allergies and also pollen

Question 78

When is radioallergosorbent test used [RAST]?

Answer 78

Determines the amount of IgE that reacts specifically with suspected or known allergens, for example IgE to egg protein. Results are given in grades from 0 (negative) to 6 (strongly positive) Useful for food allergies, inhaled allergens (e.g. Pollen) and wasp/bee venom Blood tests may be used when skin prick tests are not suitable, for example if there is extensive eczema or if the patient is taking antihistamines

Question 79

A 28-year-old man is a new patient at your surgery and attends for an initial visit. He has been in good health and has not had any hospitalisations. With regards to his family history. he reports that his father died of sudden cardiac death at age 38. A post-mortem examination revealed that his cause of death was hypertrophic cardiomyopathy. What is the probability that your patient inherited the same condition?

Answer 79

HOCM has an autosomal dominant inheritance pattern. Hypertrophic cardiomyopathy can be inherited in an autosomal dominant pattern. As one parent was affected, there is a 50 percent chance of passing the mutated HOCM gene to their child

Question 80

What is the most important cause of sudden cardiac death in the young?

Answer 80

Hypertrophic obstructive cardiomyopathy (HOCM) is an autosomal dominant disorder of muscle tissue caused by defects in the genes encoding contractile proteins. The estimated prevalence is 1 in 500. HOCM is important as it is the most common cause of sudden cardiac death in the young.

Question 81

PP of HOCM

Answer 81

Pathophysiology the most common defects involve a mutation in the gene encoding β-myosin heavy chain protein or myosin-binding protein C results in predominantly diastolic dysfunction left ventricle hypertrophy → decreased compliance → decreased cardiac output characterized by myofibrillar hypertrophy with chaotic and disorganized fashion myocytes ('disarray') and fibrosis on biopsy

Question 82

Features of HOCM

Answer 82

Features often asymptomatic exertional dyspnoea angina syncope typically following exercise due to subaortic hypertrophy of the ventricular septum, resulting in functional aortic stenosis sudden death (most commonly due to ventricular arrhythmias), arrhythmias, heart failure jerky pulse, large 'a' waves, double apex beat ejection systolic murmur increases with Valsalva manoeuvre and decreases on squatting hypertrophic cardiomyopathy may impair mitral valve closure, thus causing regurgitation

Question 83

Echo, associations and ECG findings

Answer 83

Associations Friedreich's ataxia Wolff-Parkinson White Echo findings - mnemonic - MR SAM ASH mitral regurgitation (MR) systolic anterior motion (SAM) of the anterior mitral valve leaflet asymmetric hypertrophy (ASH) ECG left ventricular hypertrophy non-specific ST segment and T-wave abnormalities, progressive T wave inversion may be seen deep Q waves atrial fibrillation may occasionally be seen

Question 84

A 22-year-old male presents to the emergency department with palpitations and lightheadedness which started suddenly 2 hours ago. He is not short of breath and currently has no chest pain. His observations are as follows: heart rate 140/min and regular, BP 134/78mmHg, oxygen saturations 99% on room air, respiratory rate 18/min, temperature 37.5ºC, and GCS 15. On examination, he has a regular, strong radial pulse and is tachycardic. You cannot hear any added heart sounds and his calves are soft and non-tender. The doctor in the emergency department arranges a 12-lead ECG. The ECG shows a regular rhythm, heart rate 141/min, narrow QRS complexes and a shortened PR interval. What is the most appropriate initial management for this patient?

Answer 84

The first-line management of SVT is vagal manoeuvres: e.g. Valsalva manoeuvre or carotid sinus massage. This patient is presenting with narrow complex tachycardia. On the patient’s ECG, there are regular, narrow QRS complexes and a shortened PR interval, suggestive of supraventricular tachycardia. The correct answer is to attempt a Valsalva manoeuvre or other vagal manoeuvres such as a carotid sinus massage. Vagal manoeuvres are the first-line management of a narrow complex tachycardia to try and convert the patient back to sinus rhythm.

Question 85

What is the valsalva maneurver?

Answer 85

The Valsalva maneuver is performed by moderately forceful attempted exhalation against a closed airway, usually done by closing one's mouth, pinching one's nose shut while expelling air out as if blowing up a balloon. Variations of the maneuver can be used either in medical examination as a test of cardiac function and autonomic nervous control of the heart, or to clear the ears and sinuses (that is, to equalize pressure between them) when ambient pressure changes, as in scuba diving, hyperbaric oxygen therapy, or air travel.[1] A modified version is done by expiring against a closed glottis. This will elicit the cardiovascular responses described below but will not force air into the Eustachian tubes

Question 86

Characteristics of SVT?

Answer 86

Whilst strictly speaking the term supraventricular tachycardia (SVT) refers to any tachycardia that is not ventricular in origin the term is generally used in the context of paroxysmal SVT. Episodes are characterised by the sudden onset of a narrow complex tachycardia, typically an atrioventricular nodal re-entry tachycardia (AVNRT). Other causes include atrioventricular re-entry tachycardias (AVRT) and junctional tachycardias.

Question 87

Acute Mx of SVT

Answer 87

Acute management vagal manoeuvres: e.g. Valsalva manoeuvre, carotid sinus massage intravenous adenosine 6mg → 12mg → 12mg: contraindicated in asthmatics - verapamil is a preferable option electrical cardioversion

Question 88

Prevention of SVT episodes?

Answer 88

beta-blockers radio-frequency ablation

Question 89

You are reviewing the blood tests of an 81-year-old woman who was admitted yesterday morning with community-acquired pneumonia. She is being treated with intravenous (IV) antibiotics and is taking regular paracetamol and naproxen for osteoarthritis. Some of her blood tests from this morning and from admission are shown below: Admission bloods Bloods from this morning Hb (115-160) 132 g/L 128 g/L Platelets (150-400) 257 \* 109/L 321 \* 109/L WBC (4.0-11.0) 18.4 \* 109/L 14.9 \* 109/L Na+(135-145) 138 mmol/L 141 mmol/L K+ (3.5-5.0) 4.2 mmol/L 3.7 mmol/L Urea (2.0-7.0) 5.6 mmol/L 10.1 mmol/L Creatinine (55-120) 62 µmol/L 191 µmol/L CRP (\<5) 185 mg/L 142 mg/L Which of the following is best describes the most recent investigation findings?

Answer 89

KDIGO AKI stage 3 ↑ creatinine \>3.0 times, or ↓ urine output \<0.3 mL/kg/hr for ≥ 24 hours This patient has developed an AKI while an inpatient in hospital. Her regular naproxen use and infection are likely to have contributed to this. Since admission, her creatinine has increased to more than 3 times from baseline so this would be classed as stage 3 AKI according to the Kidney Disease: Improving Global Outcomes (KDIGO) criteria. Other criteria for stage 3 AKI are an increase in creatinine to ≥ 353.6 µmol/L or a reduction in urine output to \<0.3 mL/kg/hour for ≥24 hours.

Question 90

Define stage 1 AKI?

Answer 90

Stage 1 AKI would be defined as an increase in creatinine by 1.5-1.9 times the baseline according to the KDIGO criteria. Other criteria for stage 1 AKI are an increase in creatinine by ≥ 26.5 µmol/L or a reduction in urine output to \<0.5 mL/kg/hour for ≥ 6 hours.

Question 91

Defien stage 2 and stage 4 AKI

Answer 91

Stage 2 AKI would be defined as an increase in creatinine by 2.0-2.9 times the baseline. The other criteria for stage 2 AKI is a reduction in urine output to \<0.5 mL/kg/hour for ≥12 hours. Stage 4 is not a stage of AKI that exists within the KDIGO criteria.

Question 92

WHat increases the risk of AKI?

Answer 92

This guideline discuss what increases the risk of AKI: Emergency surgery, ie, risk of sepsis or hypovolaemia Intraperitoneal surgery CKD, ie if eGFR \< 60 Diabetes Heart failure Age \>65 years Liver disease Use of nephrotoxic drugs NSAIDs aminoglycosides ACE inhibitors/angiotensin II receptor antagonists diuretics

Question 93

Diagnostic criteria for AKI

Answer 93

It also defines the criteria for diagnosing AKI Rise in creatinine of 26µmol/L or more in 48 hours OR \>= 50% rise in creatinine over 7 days OR Fall in urine output to less than 0.5ml/kg/hour for more than 6 hours in adults (8 hours in children) OR \>= 25% fall in eGFR in children / young adults in 7 days.

Question 94

Summarise staging criteria of an AKI

Answer 94

Stage 1 Increase in creatinine to 1.5-1.9 times baseline, or Increase in creatinine by ≥26.5 µmol/L, or Reduction in urine output to \<0.5 mL/kg/hour for ≥ 6 hours Stage 2 Increase in creatinine to 2.0 to 2.9 times baseline, or Reduction in urine output to \<0.5 mL/kg/hour for ≥12 hours Stage 3 Increase in creatinine to ≥ 3.0 times baseline, or Increase in creatinine to ≥353.6 µmol/L or Reduction in urine output to \<0.3 mL/kg/hour for ≥24 hours, or The initiation of kidney replacement therapy, or, In patients \<18 years, decrease in eGFR to \<35 mL/min/1.73 m2

Question 95

Referral criterai for an AKI

Answer 95

Refer to a nephrologist if any of the following apply: Renal tranplant ITU patient with unknown cause of AKI Vasculitis/ glomerulonephritis/ tubulointerstitial nephritis/ myeloma AKI with no known cause Inadequate response to treatment Complications of AKI Stage 3 AKI (see guideline for details) CKD stage 4 or 5 Qualify for renal replacement hyperkalaemia / metabolic acidosis/ complications of uraemia/ fluid overload (pulmonary oedema)

Question 96

A 28-year-old male is referred to the medical assessment unit by his GP following a 2-day history of visible haematuria and a productive cough. He has had no dysuria, abdominal pain, or urinary frequency. He admits to coughing up blood and experiencing nosebleeds for the past 5 days. A urine dip shows protein ++, red blood cells ++ and is negative for nitrites and leukocytes. Blood tests are carried out and the results are shown below: Hb 136 g/L Male: (135-180) Platelets 380 \* 109/L (150 - 400) WBC 6.0 \*109/L (4.0 - 11.0) Na+ 136 mmol/L (135 - 145) K+ 4.5 mmol/L (3.5 - 5.0) Urea 10.8 mmol/L (2.0 - 7.0) Creatinine 299µmol/L (55 - 120) The doctor assessing the patient suspects acute glomerulonephritis. What is the most likely underlying cause of the patient’s glomerulonephritis?

Answer 96

Anti-GBM disease typically presents with haemoptysis + AKI/proteinuria/haematuria. The correct answer is anti-glomerular basement membrane (GBM) disease (previously known as Goodpasture’s syndrome). The patient is presenting with haemoptysis and haematuria, typical of anti-GBM disease. They additionally have an acute kidney injury, haematuria and proteinuria, suggestive of a rapidly progressive glomerulonephritis secondary to anti-GBM disease. It is caused by serum antibodies that react with glomerular and alveolar basement membranes. Patients with anti-GBM disease may also experience other symptoms like a cough, bleeding from the nose and dyspnoea.

Question 97

Presentation of FSGS

Answer 97

Focal segmental glomerulosclerosis (FSGS) is incorrect. This is a common cause of nephrotic syndrome (proteinuria, hypoalbuminemia and peripheral oedema) in adults which would not account for the patient's symptoms of haematuria or haemoptysis.

Question 98

Presentation of HSP

Answer 98

Henoch-Schönlein purpura is incorrect. This is a small-vessel vasculitis most commonly seen in children, not adult males. This typically presents with a purpuric rash, normally on the buttocks or backs of the legs, joint pain and abdominal pain in conjunction with either nephrotic syndrome or acute glomerulonephritis.

Question 99

Presnetation of IgA-nephropathy

Answer 99

IgA-nephropathy is incorrect. This is a cause of glomerulonephritis caused by IgA immune complexes depositing in the kidney, this would not account for the haemoptysis experienced by the patient. This would typically occur 2-6 days following a respiratory tract or gastrointestinal infection which this patient has no history of.

Question 100

Presentation of membranoprolferative glomurlonephritis

Answer 100

Membranoproliferative glomerulonephritis is incorrect. Whilst this is an immune-mediated glomerulonephritis caused by immune-mediated damage to the glomerulus, this would not account for the patient’s haemoptysis or epistaxis.

Question 101

What is Goodpasture's syndrome? What is it associated with?

Answer 101

Anti-glomerular basement membrane (GBM) disease (previously known as Goodpasture's syndrome) is a rare type of small-vessel vasculitis associated with both pulmonary haemorrhage and rapidly progressive glomerulonephritis. It is caused by anti-glomerular basement membrane (anti-GBM) antibodies against type IV collagen. Goodpasture's syndrome is more common in men (sex ratio 2:1) and has a bimodal age distribution (peaks in 20-30 and 60-70 age bracket). It is associated with HLA DR2.

Question 102

Features of GBM?

Answer 102

pulmonary haemorrhage rapidly progressive glomerulonephritis this typically results in a rapid onset acute kidney injury nephritis → proteinuria + haematuria

Question 103

Ix for GBM

Answer 103

renal biopsy: linear IgG deposits along the basement membrane raised transfer factor secondary to pulmonary haemorrhages

Question 104

Mx for GBM

Answer 104

plasma exchange (plasmapheresis) steroids cyclophosphamide

Question 105

Factors increasing the risk of pulmoanry haemorrhage

Answer 105

smoking lower respiratory tract infection pulmonary oedema inhalation of hydrocarbons young males

Question 106

types of collagen

Answer 106

Question 107

types of hepatitis

Answer 107

Question 108

2-level PE Wells score

Answer 108

Question 109

How to manage PE flowchart?

Answer 109

Question 110

What do these ECGs show?

Answer 110

HOCM