MRCP Facts Flashcards
1
Q
Which antibody is implicated in VITT?
A
Antibody against platelet factor 4 (PF4)
These activate platelets via their Fcy11a receptors to cause thrombosis in a similar mechanism to HITT
2
Q
What translocation occurs in acute promyelocytic leukaemia?
A
t(15:17)
Retinoic acid receptor- alpha (RARA) gene on chromosome 17 is involved in a reciprocal translocation with the promyelocytic leukaemia gene (PML) on chromosome 15.
Treatment is with all-trans-retinoic acid
3
Q
What causes tear drop poikilocytes?
A
myelofibrosis
4
Q
What is the thrombolysis window for stroke?
A
4.5 hours from symptom onset to rule out of haemorrhage
5
Q
1mg of prednisolone is equivalent to how many mg of hydrocortisone?
A
4mg
6
Q
What is the first line antihypertensive drug in diabetic patients?
A
ACEi regardless of age
7
Q
What is the key differential for a loss of the corneal reflex?
A
Vestibular schwannoma - benign tumour of the myeslin-forming cells of the vestibulocochlear nerve (CN8). It can compress CN 5 which results in a loss of the corneal reflex
Bilateral vestibular schwannomas are seen in neurofibromatosis type 2.
8
Q
At what PaO2 should steroids be started in PJP?
A
Less than 9.3kPa
9
Q
What 3 hereditary syndromes are associated with phaeochromocytomas?
A
Von Hipple-Lindau syndrome
MEN-2
Succinate Dehydrogenase
10
Q
What is the valve area and mean gradient across the valve in severe aortic stenosis?
A
Valve area: <1cm^2
Mean Gradient: >40 mmHg
11
Q
What is the hallmark feature of postural orthostatic tachycardia syndrome (POTS)?
A
An increase in HR >30 in standing without a drop in BP
12
Q
What is the most likely diagnosis if someone has symptoms of asthma that do not resolve with inhalers, proximal bronchiectasis on HRCT, Increased eosinophils and IgE?
A
Allergic bronchopulmonary aspergillosis (ABPA)
Diagnosis confirmed with positive skin prick test to aspergillus fumigatus (Positive RAST test)
In some patients there will also be positive IgG precipitins to aspergillus.
Oral steroids are the mainstay of initial treatment. Itraconazole is sometimes used 2ns line
13
Q
What are the components of the GRACE score?
A
Age Systolic BP Heart rate Killip class (features of CCF) Creatinine ST segment deviation Elevated Troponin Cardiac arrest on admission
14
Q
What HLA is expressed in the majority of people with coeliac disease?
A
HLA-DQ2 in 95%
HLA-DQ8 in the remainder
15
Q
What are the 3 features of Wernicke’s encephalopathy?
A
Confusion
Ataxia
Opthalmoplegia
16
Q
Which 2 conditions commonly cause lymphocyte predominant pleural effusions?
A
Malignancy and Tuberculosis
17
Q
What are the 3 commonest causes of viral meningitis?
A
Enterovirus
Echovirus
Coxsackie A and B
Viral aetiology is most likely if someone has:
- Increased CSF opening pressure
- Slightly increases CSF protein
- CSF lymphocytosis
18
Q
Which artery is most commonly implicated in lateral medullary syndrome?
A
Vertebral artery followed by the posterior inferior cerebellar artery
Features include:
- Ipsilateral horners, ataxia and sensory loss (face)
- Contralateral spinothalamic sensory loss from the neck down
19
Q
How can you diagnose upper airway obstruction from pulmonary function testing? e.g. a goitre
A
Empey index
FEV1 divided by PEFR
Normal value <10. >10 indicates obstruction
20
Q
What is the mechanism by which metformin exacerbates lactic acidosis?
A
Decreased activity of pyruvate dehydrogenase
21
Q
In what conditions are oral bisphosphonates contraindicated?
A
Oesophageal disorders: achalasia, scleroderma, strictures and varices
(IV alternatives are advised)
22
Q
After suspected anaphylaxis, when should a mass cell tryptase level be sent?
A
ASAP. A second sample should be sent within 1-2 hours (but no later than 4 hours) from the onset of symptoms
23
Q
What is the mechanism of hypercalaemia in sarcoidosis?
A
Increased synthesis of 1, 25- dihydroxyvitamin-D3 by macrophages
24
Q
What is thyrotoxic periodic paralysis?
Who does it occur in?
What triggers it?
How is it treated?
A
Presents similarly to hypokalaemic periodic paralysis but with the presence of thyrotoxicosis
Under conditions of thyrotoxicosis, a genetic mutation in potassium transport is activated causes hypokalaemia and subsequent muscle weakness.
It occurs in predominantly Chinese, Japanese, Vietnamese, Filipino, and Korean Males
—————————-
Triggers include exertion, alcohol and certain foods (high carbohydrate load, high salt)
——————————
Because the total body potassium level is not low, cautious replacement of potassium is important.
25
Which is the single most important test to measure in patients with pituitary incidentalomas?
Serum Prolactin
---------------------------------
Increased Prolactin can indicate either:
- Direct tumour secretion
- --Normal levels <600
- --Microprolactinoma 800-2000
- --Macroprolactinoma >2000
-Disconnection of the hypothalamus and normal pituitary gland by a large non-functioning pituitary mass. Prolactin levels are usually <2000 in these cases
26
What is the diagnostic criteria for SIADH?
The following triad must be present:
- Hyponatraemia <130
- Dilute serum - Plasma osmo <270
- Concentrated Urine - Urine osmo >100
Hypovolaemia must be excluded by showing that the Na in the urine is >20-30 and there is absence of hypotension
You must also exclude other endocrine causes of hyponatraemia (e.g. Adrenal failure and Hypothyroidism)
27
How do patients with malignancy develop hypercalcaemia?
Solid Tumours
- Increased PTHrP e.g. adeno- and squamous cell cancers
- Bone lysis e.g. breast cancer
Haematological malignancy
- Increased calcitriol e.g. lymphoma, granulomatous disease
- Bone lysis e.g. multiple myeloma
28
What is the mMRC dyspnoea score?
I only get breathless with strenuous exercise 0
I get short of breath when hurrying on level ground or walking up a slight hill 1
On level ground, I walk slower than people of my age because of breathlessness, or I have to stop for breath when walking at my own pace on the level 2
I stop for breath after walking about 100 yards or after a few minutes on level ground 3
I am too breathless to leave the house or I am breathless when dressing/undressing 4
29
What criteria can you use to differentiate between mild, moderate and severe ulcerative colitis in adults?
Modified Truelove and Witt's criteria
The components are the daily frequency of bloody stool, temperature, heart rate, haemoglobin level and inflammatory marker levels.
30
How do you manage a patient who has had an ultrasound which is consistent with a diagnosis of non-alcoholic fatty liver disease (NAFLD)?
Calculate the NAFLD Fibrosis Score. This helps to identify patients at risk of advanced fibrosis
- Score 0.672: high risk of fibrosis.
- -Usually considered for a liver biopsy
The FIB-4 score can alternatively be used.
31
Without imaging, how do you know if it is the right or left carotid artery which has caused the neurological features of a TIA?
Facial/ limb weakness
-Opposite side to the weakness
Monocular blindness
-Same side of blindness
Aphasia/ Dysphasia
-Usually the left ICA
(could be the right if the speech centre is on the right).
32
What 3 electrolyte abnormalities can lead to long QT syndrome?
Hypokalaemia
Hypomagnasaemia
Hypocalcaemia
33
What are the features of a severe C, Difficile infection?
Temperature >38.5 degrees C
WCC >15
Acute Kidney Injury
Hypotension and lactic acidosis
Evidence of severe colitis (e.g. abdo pain)
Radiological features of ileus or toxic megacolon
34
What is the best diagnostic test for dengue fever in someone with only a couple days of symptoms?
Nonstructural protein 1 (NS1)
-------------------------
Allows rapid detection within the first few days of the fever as antibodies do not appear for up to 5 days or more.
35
Which congenital condition is associated with long QT syndrome and deafness?
Jervell-Lange Nielsen Syndrome due to mutations in the KCNE1 and KCNQ1 genes.
These genes code for potassium channels in the inner ear and cardiac muscle
36
How can HSV-2 meningitis recur?
To cause Mollaret's Meningitis.
37
What 4 autoimmune diseases are associated with primary biliary cirrhosis?
CREST syndrome
Sjogren's syndrome
Rheumatoid arthritis
Hashimoto's thyroiditis
38
What are iron study results in haemochromatosis?
Increased Ferritin
Increased transferrin saturation
Decreased TIBC
39
What are some indications for liver transplantation in primary biliary cirrhosis?
Bilirubin >100 mmol/L
Recurrent bacterial cholangitis
Refractory ascites and itching
40
What are the indications for treating sarcoidosis with steroids?
```
Brain: neurosarcoid
Eyes: uveitis
Face: lupus pernio
Heart: blocks, cardiomyopathy
Lungs: worsening lung function
Blood: hypercalcaemia
```
41
What are the differences between Heerfordt-Waldenstrom and Lofgren's syndrome when seen as extrapulmonary manifestations of sarcoidosis?
Heerfordt-Waldenstrom
- Parotid Enlargement
- Uveitis
- Cranial nerve palsies
- Fever
Lofgren's
- Bilateral hilar lymphadenopathy
- Polyarthritis
- Erythema Nodosum
42
What artery causes complete heart block after inferior MI?
Posterior interventricular artery (branch of the right coronary artery)
Supplies the AV node
---------------------
Fluids can be used to optimise RV filling pressures and ionotropes can be considered in refractory hypotension
43
What ECG changes occur in hypothermia?
Bradycardia and first degree heart block
Long QT interval
Osborn Wave: small positive J wave at the end of the QRS complex
Atrial and ventricular arrhythmias.
44
What are the 4 paraneoplastic syndromes associated with small cell lung cancer?
SIADH - Ectopic ADH
Cushing's - Ectopic ACTH
Lambert Eaton - Antibodies to voltage gated calcium channels
Peripheral Neuropathy - Neuronal nuclear antibody (Hu antibody)
45
What 2 complications of haemochromatosis can resolve with effective treatment?
Cardiomyopathy and testicular atrophy
Irreversible complications include arthropathy, diabetes, liver cirrhosis and hypogonadotrophic hypogonadism
46
What are the 3 histological types of mesotheliomas?
Mixed
Sarcomatous (worse prognosis)
Epithelial
47
What chelation agent is used in Wilson's disease and Haeochromatosis?
```
Wilson's = Penicillamine
Haemochromatosis = Desferrioxamine
```
48
From which cell type does bronchial carcinoid originate?
Kulchitsky cells - these arise from the APUD system, just like small cell lung cancer
Bronchoscopy identifies most of these as highly vascular "cherry like" tumours that cause recurrent haemoptysis and bronchial obstruction
It is rare to develop carcinoid syndrome with these tumours.
49
What features suggest VT rather than SVT with aberrant conduction?
```
AV dissociation
QRS concordance in chest leads
Left axis deviation
QRS >160ms
Capture beats (intermittent conduction)
No response to adenosine/ carotid sinus massage
```
50
What causes Farmer's lung?
spores of saccharopolyspora rectivirgula in hay/ straw
51
What causes Bird fancier's lung?
Avian serum proteins in faeces
52
What causes Bagassosis?
Thermoactinomyces sacchari in sugar cane workers
| type of hypersensitivity pneumonitis
53
What causes Malt Worker's Lung?
Aspergillus clavatus
54
What causes Mushroom worker's lung?
Thermophilic actinomycetes
55
What causes Psittacosis?
Chlamydia psittaci from birds
56
At what pulmonary artery pressure can you make a diagnosis of pulmonary artery hypertension?
PA pressure >25 mmHg at rest
| PA pressure >30 mmHg after exercise
57
Which condition in females causes recurrent pneumothoraces and chylorous effusions?
Lymphangioleiomyomatosis (LAM)
------------------------
This is where there is progressive growth of smooth muscle cells throughout the lungs, pulmonary blood vessels, lymphatics and pleurae due to mutations in the tuberous sclerosis genes
If these clinical features are seen in males, then the diagnosis to consider is tuberous sclerosis - an autosomal dominant disorder of genes located on chromosome 9 and 16.
58
Why do patients with Hayde's syndrome have GI bleeding?
Heyde's syndrome is the combination of microcytic anaemia and calcific aortic stenosis.
Patients commonly have angiodysplasia which can be diagnosed with a mesenteric angiogram.
The mechanism of bleeding is through the destruction of von Willebrand factor as the blood flows over the stenosed aortic valve.
The combination of vWF deficiency and angiodysplasia results in GI bleeding.
59
What are the clinical features of pulmonary hypertension?
```
Exertional dyspnoea
Syncope
Loud P2
Left parasternal heave (due to RVH)
Large a waves in JVP (due to tricuspid regurgitation)
```
60
Which antibodies are usually positive in the three types of autoimmune hepatitis?
Type 1
- Anti-nuclear antibody (ANA)
- Anti-smooth muscle antibody (SMA)
Type 2
-Anti-liver kidney microsomal antibodies (LKM-1)
Type 3
-Anti-soluble liver antigen (SLA)
61
What is the mechanism of renal injury with aciclovir in the presence of dehydration?
Crystalluria
-----------------------------
Aciclovir is renally eliminated
In the presence of dehydration, aciclovir can precipitate as crystals in the kidney tubules causing AKI
62
What is the mechanism of action of alteplase?
Human tissue plasminogen activator. It accelerates the conversion of plasminogen to plasmin.
It binds to fibrin-rich clots and cleaves plasminogen to form plasmin which then degrades the fibrin matrix of the thrombus
63
Which organism is implicated if a young patient has a diarrhoeal illness, right iliac fossa pain and mesenteric adenitis?
Yersinia enterocolitica - a gram-negative bacillus-shaped bacterium
acquire this after consumption of contaminated food and through blood transfusions. Diagnosis is predominantly based on positive stool cultures. It mimics appendicitis and a flare of Crohn's disease.
64
How would you manage the rash in discoid lupus erythematosus if it has not responded to potent topical steroids and photoprotection?
----------------------------
Hydroxychloroquine
Discoid lupus erythematosus can cause scarring so early treatment is important. Acitretin is of similar efficacy to hydroxochloroquine but has far more adverse effects including teratogenicity.
65
Why do people with primary hyperparathyroidism have hypophosphataemia?
Increased phosphate excretion
Excess PTH decreases reabsorption of phosphate at the proximal tubule of the kidney. Therefore, more phosphate is excreted into the urine.
66
How is Dubin Johnson Syndrome inherited and which protein is implicated?
Autosomal recessive defect in cMOAT
This causes defective hepatic excretion of conjugated bilirubin. As a result, the urine is dark. Liver biopsy shows dark granules (melanin) within hepatocytes.
67
What are the extracolonic manifestations of FAP Coli?
Retinal pigment hypertrophy
Fibromas and epidermoid cysts
Supernumery teeth and dentigerous cysts
Osteomas of the skull and mandible
68
How do you calculate osmolality?
2 (Na and K) + glucose + Urea (all in mmol/L)
69
Which conditions are associated with an aortic dissection?
```
Hypertension
Trauma
Bicuspid Aortic Valve
Marfan and Ehlers-Danlos Syndrome
Turner and Noonan's syndrome
Pregnancy
Tertiary Syphilis
```
70
When is it possible to have a haemoglobin and haematocrit within normal levels in a patient with polycythaemia rubra vera (PRV)?
If there is co-existing severe iron deficiency
Normally in PRV there will be:
Increased haemoglobin, increased haematocrit, increased WCC, increased platelet count.
71
What did the RALES study show about using spironolactone in patients with heart failure?
A substantially decreased mortality by 30% from the addition of spironolactone to patients with heart failure (LV ejection fraction <35%) on otherwise "optimal" conventional treatment.
72
How does a Glomus jugulare tumour present?
Gradual onset of right conductive hearing loss, right palatal palsy, dysphonia, bovine cough and wasting of the right side of tongue
Typically insidious onset over years with unilateral lower cranial nerve involvement.
73
What is the definitive diagnostic test for pemphigus vulgaris?
Direct immunoflourescence of skin
74
Where in the brain does the D2-receptor antagonist, haloperidol, predominantly exert its antiemetic action?
Area Postrema in the medulla.
75
What are some of the complications of Mycoplasma Pneumonia?
Blood
- Haemolytic anaemia (cold agglutinins IgM)
- DIC
Skin
-Erythema multiform and nodosum
CNS
-Meningoencephalitis, Guillain Barre syndrome, transverse myelitis and cerebellar ataxia
Heart
-Pericarditis and Myocarditis
GIT
-Hepatitis and Pancreatitis
Renal
-Glomerulonephritis
76
A defect in which gene causes Cowden syndrome?
Defect in PTEN tumour suppressor gene
This syndrome is a hamartomatous polyposis syndrome of the gastrointestinal tract with characteristic mucocutaneous lesions e.g. oral mucosal papillomas, palmoplanter keratosis and trichilemmomas
There is increased risk of malignancy especially of the breast and thyroid glands.
77
Which type of streptococcus viridans commonly is associated with infective endocarditis following dental work?
Streptococcus mitis
78
How is Gardener's syndrome inherited and what are some of its features?
Autosomal dominant
----------------------------
- Multiple adenomatous intestinal polyps of the small and large bowel. 90% of patients will develop bowel cancer by the age of 45 years. Colectomy is recommended once these polyps appear
- Osteomas
- Soft tissue tumours i.e. fibromas, lipomas
- Increased risk of papillary carcinoma of the thyroid
79
How is Peutz-Jegher's syndrome inherited and what are its features?
Autosomal dominant mutation of the STK 11 gene on the long arm of chromosome 19
-----------------------
- Hamartomatous polyps in the GI tract which increase the risk of cancer
- Pigmented freckles (melanocytic macules) on lips, face, palms, and soles
- Increased incidence of GI bleeding and intussusceptions.
80
Why do patients with renal impairment develop opioid toxicity?
Morphine is metabolised to active glucuronide metabolites with are excreted renally
Renal impairment leads to accumulation of morphine-6-glucuronide which is a potent opioid agonist with a higher affinity than morphine.
81
Which commonly used antimicrobials can cause QT prolongation?
Quinolones e.g. levofloxacin, moxifloxacin
Macrolides e.g. erythromycin, clarithromycin
Antimalarials e.g. quinine
Antiprotozoal e.g. pentamidine
Azole antifungals e.g. fluconazole, ketoconazole
82
What organism commonly causes athlete's foot?
Trichophyton rubrum
83
What is the first line treatment for erythrodermic psoriasis?
Topical white soft paraffin.
----------------------
This partially decreases inflammation, improves the skin barrier (which is generally defective in erythroderma) and helps decrease water loss to prevent dehydration
Erythrodermic psoriasis may be associated with systemic upset.
84
What are the components of the Waterlow Score in assessing the risk of developing a pressure sore?
```
Age
Sex
Body Weight
Nutritional Status
Continence
Skin type
Mobility
```
85
What is lipodermatosclerosis and which layers of the skin does it affect?
Lipodermatosclerosis is a chronic sclerosing panniculitis of the lower legs and is related to venous hypertension.
Fibrous tissue replaces the hypodermis and if extensive, it also replaces the papillary dermis. Patients experience pain, hardening of the skin, redness, swelling and a tapering of the legs above the ankles.
86
If someone presents with a STEMI which is presumed to be related to stent thrombosis, which antiplatelets do you give before angiography if they are already taking aspirin and clopidogrel?
Aspirin and Prasugrel
Stent thrombosis may be related to some degree to clopidogrel resistance/ failure
87
How do you differentiate between MEN 1 and MEN 2?
Genetics
- MEN 1 = MEN1 gene Chromosome 11q13
- MEN 2 = RET oncogene Chromosome 10
Tumours
- MEN 1 = Pancreatic tumours and pituitary tumours
- MEN 2 = Phaeochromocytomas and Medullary thyroid
88
What is the normal function of BRCA-1?
BRCA-1 is a tumour suppressor gene which codes for a DNA repair protein.
89
What are the features of Gertmann syndrome?
Parietal lobe is implicated
- Dysgraphia or agraphia (inability to write)
- Acalculia (inability to do maths)
- Finger agnosia (inability to identify one's own or another's fingers)
- Limb disorientation (Inability to make the distinction between the right and left side of the body)
90
Which antibody can be found to be positive in ulcerative colitis?
pANCA
-------------------------------
In UC there is an increased incidence of PSC, PBC and chronic active hepatitis.
91
What is the first line diagnostic investigation of coronary artery disease?
CT calcium scoring
- If Ca score is between 1-400 then do a CT coronary angiogram
- If Ca score is >400 then perform an invasive coronary angiogram.
92
Which 4 GI diseases are associated with H. Pylori infection?
MALT lymphoma
Peptic ulcers disease
Gastric carcinoma
Atrophic gastritis
93
In neuromuscular weakness, at what level of forced vital capacity (FVC) should you be concerned about impending respiratory failure?
FVC <15-20 mls/kg of ideal body weight
| (usually this is around <1L
94
What are the complications of Meckel's diverticulum?
```
Diverticulitis
Intussusception
Perforation
Obstruction
---------------------------
```
Meckel's diverticulum is the vestigial remnant of the omphalomesenteric duct.
It is normally located in the terminal ileum within around 60 cm of the ileoceacal valve and it averages 6cm in length.
About half of them contain ectopic gastric mucosa commonly leading to clinical presentations of peptic ulceration and haemorrhage
Tc-99m pertechnetate accumulates in gastric mucosa and is the study of choice for identifying this ectopic gastric mucosa.
95
How do you treat acute pancreatitis which has been caused by excess triglycerides?
Insulin
Insulin decreases triglyceride levels by activating lipoprotein lipase which metabolises chylomicrons.
If the glucose is normal or insulin doesn't bring down the triglycerides adequately then plasmapheresis is an appropriate alternative
96
Which antibody can be found to be positive in Crohn's disease?
Anti-saccharomyces cerevisiae antibody (ASCA)
--------------------
ASCA favours Crohn's while UC is strongly associated with pANCA.
97
How does the shape of the crystals differ between gout and pseudogout?
Pseudogout = Rhomboid-shaped with positive birefringence
Gout = needle-shaped with negative birefringence
98
What is the duration for post exposure prophylaxis in needle stick injury from an HIV positive patient?
Three drug antiretroviral treatment within 1-2 hours for a total of 28 days (may be started up to 72 hours following exposure).
This decreases risk of transmission by 80%. Serological testing should be performed at 12 weeks following completion.
99
What is the treatment of schistosomiasis?
Praziquantel as a single dose and a short course of steroids to control the inflammatory reaction.
The dose of praziquantel should be repeated after 6 weeks when flukes are fully mature because immature flukes are sometimes resistant to treatment.
100
What is the difference between burst suppression and background suppression in the context of treating status epilepticus?
Burst suppression: Treatment has induced a sufficiently deep coma
Background suppression: Excess sedation (deeper level of coma than in burst suppression)
101
What are the possible auscultatory findings in mitral valve prolapse?
Mid systolic click (occurs later if squatting)
Late systolic murmur (longer if standing)
102
What is the most appropriate antihypertensive to use in bipolar patients who take lithium?
CCBs e.g. amlodipine (even in under 55)
-Does not cause increased serum lithium concentration
----------------------
ACEi, ARBs and thiazide diuretics can all cause lithium
103
How can you clinically differentiate an L5 radiculopathy from a common peroneal nerve palsy in someone with foot drop?
Weakness of ankle inversion is present in an L5 radiculopathy but is not seen in a common peroneal nerve palsy.
This is because the tibialis posterior muscle is supplied by the tibial nerve (L4/5).
104
Which pulmonary function test is most helpful in monitoring the progression of usual interstitial pneumonitis (UIP)?
Transfer factor (TLCO or KCO)
This measures how effectively oxygen diffuses from the alveoli into the blood. It is used to determine the severity of the fibrosis and can be used to monitor progression of disease.
105
What is the mechanism through which clopidogrel inhibits platelet aggregation?
Clopidogrel irreversibly inhibits platelet aggregation via the P2Y12 ADP receptor. The predominant receptor involved in ADP-stimulated activation of glycoprotein IIb/IIIa receptor.
This results in decreased platelet degranulation, thromboxane production and platelet aggregation.
106
Why should you not give flecainide to pts with AF and LVSD?
Negative ionotropic effect
---------------------
An echo must be carried out prior to prescription to rule out structural heart disease or IHD
107
What is the most likely cause for chronic pale and bulky diarrhoea in someone with systemic sclerosis?
Small intestinal bacterial overgrowth
In systemic sclerosis, the small bowel wall is infiltrated with fibrous tissue. This results in dysmotility, dilatation and alterations in bacterial flora. This manifets with abdominal discomfort, flatulence, steatorrhoea and malabsorption.
108
Which imaging modality best differentiates knee pain related to psoriatic arthritis from mechanical pain?
Gadolinium contrast MRI scan of the knee
109
Which chemotherapy-sensitive tumours can cause superior vena cava obstruction (SVCO)?
Small cell lung cancer
Lymphomas
Germ cell tumours
Patients have dilated neck veins and pulseless JVP distension. It is an oncological emergency. The most common cause of SVCO is non-small cell lung cancer (not chemotherapy-sensitive).
110
Scrub typhus is spread by which mite?
Trombiculid mite
111
What are some examples of serotonergic drugs?
```
SSRIs SNRIs TCAs
Litium
Tramadol
Amphetamine
MAO inhibitors
```
112
What are the components of the disaccharides lactose, maltose and sucrose?
```
Maltose = glucose and glucose
Lactose = glucose and galactose
Sucrose = glucose and fructose
```
113
What classically happens to the creatinine kinase in anticholinergic poisoning?
It is usually within normal levels
-------------------
Although anticholinergic poisoning can be associated with hyperpyrexia and tachycardia, the CK is not significantly elevated. This is in contrast to the increased CK that occurs in serotonin syndrome and in neuroleptic malignant syndrome.
114
Which sleep disorder is most associated with an increased risk of Parkinson's disease?
Rapid Eye Movement (REM) Sleep Behaviour Disorder
-------------------------------
This is a parasomnia in which the subjects act out dreams with physical movements that are considered to be the equivalent of what they are doing in their dreams.
REM sleep behaviour disorder is associated with the later development of movement disorders such as Parkinson's disease
115
What is the most likely diagnosis if a patient with longstanding mitral valve prolapse presents with a sudden-onset of left sided chest pain and clinical features of acute severe left heart failure in the presence of a non-specific ECG?
Acute mitral regurgitation
------------------------------
The presence of longstanding mitral valve prolapse causes myxomatous degeneration of the valve apparatus which predisposes to chordal rupture.
Acute, short lived chest pain is a feature of chordal rupture.
116
How many seconds should a pause in heart rate be to consider a permanent pacemaker?
>3 seconds regardless of symptoms
OR
<3 seconds with symptoms
117
What are the features of internuclear ophthamoplegia and which part of the brain is affected to cause it?
Internuclear ophthalmoplegia (INO) is cuased by a lesion of the medial longitudinal fasciculus.
This is a nerve bundle which connects the CN6 nucleus in the pons and the CN3 nucleus in the midbrain
There is loss of ADDuction of the ipsilateral eye (due to paralysis of the ipsilateral medial rectus. The other eye which is in ABDuction has nystagmus.
-------------------------
For example in left INO, if the patient is asked to look to the right, the left eye does not adduct and there is nystagmus in the right eye. If asked to look the left there are no abnormalities seen.
118
What should you consider as the cause if a patient develops GI bleeding after endovascular AAA surgery?
Aortoenteric fistula
119
What is the pathophysiology of bullous pemphigoid?
Antibodies against the hemidesmosomal proteins BP180 and BP230.
120
Which antibodies are positive in the majority of patients with subacute cutaneous lupus erythematosus (SACLE)?
ANA positive
Anti-Ro antibodies
Anti-La antibodies
10-15% of those with SACLE progress to SLE with moderate disease activity.
121
To which 3 molecules does carbon monoxide bind to cause its toxic effects?
It causes type A toxicity by binding to:
- Haemoglobin: left shift of the oxygen dissociation curve
- Myoglobin: reduces its oxygen carrying capacity
- Mitochondrial cytochrome oxidase: inhibits cellular respiration
122
What type of gaze palsy is seen in progressive supranuclear palsy?
Vertical gaze palsy (inability to look downwards)
-------------------
AKA Steele-Richardson-Olszewski Syndrome
123
What cranial nerves are usually affected in lateral sinus thrombosis?
CN 6 and CN 7
124
What are the features of Behcet's disease?
Classic triad: Oral ulcers, Genital ulcers, Anterior uveitis
Also: DVTs, arthritis, aseptic meningitis, colitis and erythema nodosum
-------------------
Patients have a positive pathergy test: the puncture site of a needle prick becomes inflamed and a small pustule developed
125
What is the difference between Charcot's triad and Reynold's pentad in acute cholangitis?
Charcot's triad = Fever, Jaundice, RUQ pain
Reynold's pentad = Charcot's triad, Altered mental status and shock.
126
Which conditions are inherited in an X-linked dominant fashion?
Alport's syndrome
Vitamin D resistant rickets
Rett syndrome
127
What gaze palsy do you get in Parinaud's syndrome and which part of the brain is affected?
Vertical gaze palsy (inability to look upwards)
The dorsal midbrain and superior colliculus are implicated
------------------------
Other eye features include
-Collier's Sign: retraction of the upper eyelid
-Convergence-retraction nystagmus
Large pupils with poor reaction to light
Causes include MS, pineal tumours, strokes, AV malformations and hydrocephalus.
128
What are the components of the Blatchford Score?
```
Urea
Haemoglobin
Systolic blood pressure
Pulse rate
History (malaena and syncopy)
Evidence of chronic/acute heart or liver failure
```
129
What are the RIFLE criteria for acute kidney injury?
Risk:
Cr x 1.5,
>25% reduction GFR,
<0.5ml/kg/hr for 6 hrs
Injury
Cr x 2,
>50% reduction GFR
<0.5 ml/kg/hr for 12 hours
Failure
Cr x 3
<0.3 ml/kg/hr for 24 hours OR anuria for 12 hours
Loss
Loss of renal function >4 weeks
End stage
Loss of renal function >3 months
130
What are the renal considerations when prescribing nitrofurantoin or trimethoprim?
Nitrofurantoin
- Should not be prescribed if the GFR <60 as it may be ineffective and the side effect profile is increased (e.g. development of neuropathy
Trimethoprim
- Should be used with caution in people with CKD. Trimethoprim competes with creatinine secretion and can cause in increase in creatinine.
- Although this is of limited physiological significance, trimethoprim can also precipitate hyperkalaemia and be less effective in treating bacteriuria with advanced renal dysfunction
131
What is the mechanism of secretory diarrhoea caused by vibrio cholera?
1. The vibrio cholera exotoxin activates a GPCR on crypt cells to increase intracellular cAMP
2. This results in the opening of Cl- channels and active transport of chloride into the gut lumen
3. H2O and Na+ ions follow
4. NaCl reabsorption is decreased through inhibition of Na/Cl co-transport in the vili.
132
What drug do you use to treat adrenaline-induced digital ischaemia?
Phentolamine - an adrenergic antagonist
---------------------
Adrenaline causes severe vasoconstriction because of its alpha-adrenergic effect.
133
What is the mechanism of action of terlipressin in managing variceal bleeding?
Constriction of the splanchnic vessels
It is an agonist of the vasopressin V1 receptors
It has been shown to be beneficial in initial haemostasis and in the prevention of rebleeding
134
What causes Foster Kennedy Syndrome and what are its features?
A frontal lobe tumour (e.g. olfactory groove meningioma) directly compresses the ipsilateral optic nerve (resulting in optic atrophy and central scotoma) and the ipsilateral olfactory nerve (resulting in anosmia)
Secondary raised ICP causes papilloedema in the contralateral eye (the eye with the intact optic nerve)
135
In drug overdose, when is single dose activated charcoal (SDAC) and multiple-dose activated charcoal (MSAC) given?
SDAC:
-Ingestion of a potentially toxic dose of a drug within the last hour that is absorbed by activated charcoal
MDAC:
- `ingestion of a life threatening amount of specific drugs:
- -Quinine
- -Phenobarbital
- -Carbamazepine
- -Dapsone
- -Theophylline
136
What are the 4 stages of pathogenesis in primary biliary cirrhosis?
1. Portal inflammation and bile duct abnormalities
2. Periportal fibrosis
3. Septal fibrosis
4. Cirrhosis
----------------
Anti-mitochondrial antibodies (AMA - the M2 subtype)
are the hallmark of PBC. Other antibodies that may be raised include smooth muscle antibodies (SMA) and IgM) (((ALL M)))
Treatment aims to slow progression of the disease with ursodeoxycholic acid and with immunosuppressants, but liver transplantation is the only life saving procedure. Fat-soluble vitamin supplementation is helpful as there is often malabsorption of fat-soluble vitamins (ADEK)
137
What is the Maddrey's discriminant function in alcoholic hepatitis?
This is a validated scoring system to predict prognosis and to decide appropriateness of corticosteroid therapy:
4.6 x (PT - control time) + bilirubin in mg/dl
A discriminant function > 32 indicates severe disease and corticosteroids should be considered
The MELD score is alternative that can be used for the same purpose
138
How long does it take to see results when finasteride is used to treat BPH?
6 months.
139
In cases of severe haemoptysis requiring nebulised adrenaline what is the most likely cause and what is the most likely source of bleeding?
Most frequent diseases causing haemoptysis are:
- Bronchiectasis
- Tuberculosis
- Fungal infections
- Cancer
Source of bleeding
- Bronchial artery (90%)
- Pulmonary artery (5%)
140
When should a referral for a transjugular intrahepatic portosystemic shunt be made?
1. Diuretic-resistant ascites requiring paracentesis
| 2. Uncontrolled bleeding from gastric or oesophageal varices
141
Clinically what antiepileptic is good for seizures not responding to benzos?
Traditionally phenytoin 20mg/kg however most experts now moving over to levetiracetam 40-60mg/kg to max 4.5g.
142
How do you treat Grave's disease in someone who is keen on becoming pregnant?
Propylthiouracil
It is more protein bound than carbimazole and therefore less transmitted to the foetus across the placenta.
143
An overdose of carbamazepine mimics overdose of which class of drug?
Tricyclic antidepressants
-------------------------
The toxic mechanism is sodium channel blockade
144
What are the typical features of gamma-hydroxybutyrate (GHB) overdose?
GHB is a clear and colourless liquid taken as a recreational drug that in overdose typically causes profound coma, airway obstruction and respiratory arrest.
Other features are agitation, nausea, vomiting, bradycardia and meiosis.
It probably acts at the GABA receptors and its effects are typically short-lived with complete recovery within hours.
Patients are noticed to wake up abruptly and self extubate as it is rapidly metabolised to inactive substances
145
How do you diagnose hyperosmolar hyperglycaemic state (HHS)?
Dehydration
Osmolality >320 mOsm/L
Glucose >30 mmol/L
Absence of significant ketosis (<3 mmol/L)
146
Why don't you get hyperkalaemia in secondary hypoadrenalism but you do in primary adrenal failure?
Primary adrenal failure:
-Aldosterone production is affected. Therefore patients are hyperkalaemic and hyponatraemic
Secondary adrenal failure:
-Aldosterone production is unaffected. Therefore patients are not hyperkalaemic.
Note some patients with secondary hypoadrenalism can develop hyponatraemia due to increased ADH which results in an increased free water retention.
147
What conditions cause increased KCO and Normal/ decreased TLCO?
Pneumonectomy/ lobectomy
Kyphoscoliosis
Neuromuscular weakness
Ankylosis of costovertebral joints (e.g. ankylosing spondylitis)
148
According to the GOLD guidance when, can roflumilast be used in COPD?
Roflumilast is a phosphodiesterase-4 inhibitor. It is used to decrease exacerbations for patients with severe and very severe airflow limitation and frequent exacerbations that are not adequately controlled on long-acting bronchodilators.
FEV1 30-49%, Stage 3, Airflow limitation = severe
FEV1 <30%, Stage 4, Airflow limitation = very severe
149
Which 2 abnormalities should be demonstrated when diagnosing Vitamin C deficiency?
Decreased vitamin C levels
| Increased bleeding time
150
What biochemical picture may a patient with Vitamin D deficiency have?
Decreased Calcium
Increased ALP
Increased PTH (secondary hyperparathyroidism)
151
Why shouldn't you be reassured by a "normal" creatinine concentration in pregnancy?
Serum creatinine levels decrease by 50% by the second trimester as a result of increased renal blood flow.
Therefore someone can have renal dysfunction with a normal creatinine concentration in pregnancy (e.g. SLE presenting in pregnancy)
152
What are the causes of culture negative infective endocarditis?
```
Prior antibiotic therapy
Brucella
Bartonella
Coxiella burnetti
HACEK group: Haemophilus, Actinobacillus, Cardiobacterium, Eikenella Kingella.
```
153
What stain is applied to a peripheral blood film to diagnose malaria?
Wright or Giemsa stain
154
What are examples of myositis associated antibodies?
Myositis-associated antibodies are seen in up to 50% of patients with myositis. These are typically seen in other connective tissue diseases:
- Anti-PM/Scl antibodies: seen in polymyositis/systemic sclerosis overlap
- Anti-Ku antibodies: seen in overlap of myositis/ connective tissue diseases
155
What is AA amyloid and which organs does it commonly effect?
Persistent inflammation leads to increased levels of amyloid A.
These conditions include rheumatological conditions (e.g. RA, ankylosing spondylitis, psoriasis, Still's disease and Behcet's disease.
Amyloid A deposits itself typically in the liver, spleen, and kidneys leading to varying degrees of organ dysfunction and failure.
156
What are the 3 most common causes for a chronic cough (>8 weeks) with a normal CXR?
1. Gastroeosophageal reflux disease
2. Asthma syndromes (cough variant asthma and eosinophilic bronchitis
3. Rhinitis
157
What are the 3 types of myositis specific antibodies?
Transfer RNA (tRNA) synthetases
- Many exist
- the main one is anti-Jo-1 antibody which is seen in 25-30% of all patients with polymyositis
- Patients who are anti-Jo-1 positive tend to have interstitial lung disease, arthritis and fevers.
Nuclear Mi-2 Protein
-Seen in approximately 20% of patients with myositis and are more specific for dermatomyositis
Signal recognition peptide (SRP)
- Seen in approximately 5% of patients with polymyositis
- Associated with a poor response to treatment and a poor prognosis.
158
What happens to the levels of pleural fluid adenosine deaminase (ADA) in tuberculous pleurisy?
Increased ADA
159
What is AL amyloidosis?
LIGHT CHAIN = AL
Commonest form of systemic amyloidosis. It is associated with underlying plasma cell dyscrasia from which immunoglobulin light chains accumulate to give accumulation of amyloid in tissues. A biopsy stained with Congo red would show the typical apple green birefringence.
In addition to the tissues involved in AA amyloidosis, the heart, nerves, skin, tongue and connective tissues are involved in AL amyloidosis.
160
What is the difference between incomplete and complete trifascicular block?
Incomplete trifascicular block:
-Bifascicular block + 1st or 2nd degree heart block
Complete Trifascicular block
-Bifascicular block + 3rd degree heart block
161
What are the diagnostic criteria for the diagnosis of Churg-Strauss Syndrome?
The American College of Rheumatology 1990 criteria requires at least 4 of the following criteria:
1. Asthma
2. Eosinophilia > 10%
3. Paranasal sinusitis
4. non-fixed pulmonary infiltrates on CXR
5. Mononeuropathy or polyneuropathy
6. A biopsy showing extravascular eosinophils
162
What are the 2 main indicators for using sodium bicarbonate in managing a poisoned patient?
1. Cardiotoxicity and neurotoxicity in sodium channel blockage (e.g. overdose with TCAs and Type 1 antiarrhythmics)
2. Enhanced elimination in salicylate poisoning.
163
What are the indications for digoxin-specific FAB fragments in cardiac glycoside poisoning?
Serum potassium > 6 mmol/L
Life threatening arrhythmias
Cardiovascular compromise including in a cardiac arrest
164
How does sodium bicarbonate work in managing the salicylate overdose?
1. Provides a large sodium load to overcome sodium channel blockade
2. Buffers acidosis (increased pH) and prevents ionised salicylate from entering the CNS
3. Promotes excretion through urinary alkalization - ionised salicylate accumulates in the renal tubules and cannot be reabsorbed ("ion trapping")
165
How do you diagnose loiasis (loa loa)?
Microfilariae on blood film.
Blood should be taken between 10am and 3pm to coincide with the periodicity of the parasite.
-----------------------------
Loa loa infection is a subcutaneous filarial infection which presents in West and Central Africa. It is transmitted by the bite of chrysops - a small tabanid fly which bites by daytime. These filarial worms can live up to 18 years. Females give birth to live microfilariae which are most easily detected at around midday to match the feeding habits of the vector.
The adult worm migrates through connective tissues and can sometimes be seen migrating across the conjunctiva. Immune-mediated swellings appear periodically lasting hours to days around an area where the adult is migrating. These are known as calabar swellings (e.g. on the hands, wrists and forearms). They are sometimes itchy.
166
What are the 3 toxic mechanisms of salicylates?
1. Cyclo-oxygenase inhibition
2. Central respiratory stimulation and respiratory alkalosis
3. Oxidative phosphorylation uncoupling
167
What conditions cause atypical lymphocytes on blood film?
```
EBV infection
CMV infection
HIV
Rubella
Toxoplasmosis
```
168
Why do patients with acute promyelocytic leukaemia (APL) develop coagulopathy?
Leukaemic cells express:
- Activators of coagulation e.g. tissue factor, which leads to DIC
- Activators of fibrinolysis e.g. annexin II, which leads to hyperfibrinolysis
At presentation, the predominant feature is usually hyperfibrinolysis.
Bleeding tendency is also caused by thrombocytopenia which occurs because of bone marrow invasion and failure to produce platelets.
Blood product support and early initiation of all-trans retinoic acid are important in managing APL.
169
Why is plasmodium falciparum the most severe form of malaria?
It parsitizes red blood cells of all ages:
- This leads to increased parasitaemia, a profound systemic inflammatory response and increased levels of haemolysis
- In contrast:
- -Plasmodium vivax and ovale only parasitize very young red cells
- -Plasmodium malariae only parasitizes old red cells
It exhibits sequestration:
- It avoids immune clearance by inducing changes in parasitized red cells
- These cells adhere to peripheral capillaries (e.g. in the brain, gut and placenta, and avoid splenic destruction
170
How do you treat loiasis and what is the consideration in onchocerciasis co-infection?
Diethylcarbamazine (DEC)
Albendazole can also be used
If there has been recent eye migration, treatment should be delayed
----------------------------
In the case of onchocerciasis co-infection, treatment is with ivermectin.
If DEC is used with onchocerciasis co-infection, this can lead to an unintended severe Mazzotti reaction - an intense pruritic rash, sometimes associated with a serious systemic reaction through the death of subcutaneous microfilariae of onchocerca volvulus.
Note that treatment with ivermectin in a patient with a high filiarial load of loa loa can cause fatal encephalopathy
171
How do you treat the two types of leprosy?
Paucibacillary disease
-Treatment 6 months: Dapsone daily and Rifampiciin monthly
Multibacillary disease
-Treatment 12-24 months: Dapsone and Clofazimine daily; Rifampicin and Clofazimine monthly
----------------
Steroids can be used as adjunct in treatment to help decrease sudden inflammatory reactions that can occur in skin and/ or nerves that can lead to acute damage.
172
How do you clinically differentiate an ulcer nerve palsy from a C8 radiculopathy?
Ulnar Nerve Palsy:
- Sensory loss and weakness of the abductor digiti minimi and flexor digitorum profundus to the 4th and 5th digits
- The ulnar nerve is commonly entrapped at the elbow (medial epicondyle) and can be exacerbated by repetitive or prolonged use of the elbow.
C8 radiculopathy:
- The sensory deficit is a similar distribution but typical splitting of sensory loss on the ring finger is not seen
- Does not cause weakness of the abductor digiti minimi.
173
When should colonoscopic surveillance be offered to patients with IBD?
They must meet 2 main criteria:
1. Symptoms started 10 years ago
2. Patient has ulcerative colitis (but not proctitis alone) OR Crohn's disease affecting more than one section of the colon
- ------------------------
The frequency of colonoscopies is based on several other factors.
174
What is the mechanism of action of tolvaptan?
Selective V2 receptor antagonists
It is licensed for the treatment of SIADH
175
What are the possible complications of latent EBV infection?
Latent EBV is in B-lymphocytes
Low level or intermittent reactivation:
-Allows secretion of live virus into saliva and provides the opportunity to infect others
Chronic low-level reactivation in immunocompromised patients:
- Causes mutations within the genome of the B lymphocytes giving rise to various neoplasms including: B-cell lymphoma, Hodgekin's and Burkitt's Lymphomas, Post-transplant lymphoproliferative disease, Nasopharyngeal carcinoma
- This is seen typically in association with repeated malaria infections, HIV infections, transplant patients and in other forms of immunosuppression
176
1 mg of dexamethasone is equivalent to how many milligrams of prednisolone?
7 mg prednisolone
177
What are the diagnostic criteria for Common Variable Immunodeficiency (CVID)?
Decreased IgG below reference range
Marked decrease in IgA and/or IgM
Abent/ impaired responses to polysaccharide vaccine
Onset after 2 years of age
Exclusion of defined causes of hypogammaglobulinaemia
-----------------------------
This is the most common clinically significant antibody deficiency disease
Patients present with recurrent sinopulmonary infections and often with structural lung and sinus disease. It may predispose to malignancy (e.g. lymphoma)
Note that Selective IgA deficiency (IgAD) is the most common antibody deficiency but is usually asymptomatic
178
What is the mechanism of action of nicotinic acid?
Increased HDL levels
179
What are the 3 reasons sodium bicarbonate is controversial as a treatment for metabolic acidosis?
It can worsen intracellular acidosis
It has have a negative inotropic effect
It can decrease peripheral oxygen delivery
180
What are the effects of parathyroid hormone?
1. PTH enhances renal calcium absorption and promotes the 1-hydroxylation of 25-hydroxyvitamin D3. 1,25-hydroxyvitamin D3 increases absorption of calcium from the gastrointestinal tract.
2. PTH and 1,25-dihydroxyvitamin D3 also have effects on bones to increase resorption of calcium from them to increase serum calcium
3. PTH inhibits renal phosphate reabsorption, which leads to low serum phosphate levels and phosphaturia
181
What are the components of the HASBLED score?
```
Hypertension
Abnormal renal +/- liver function
Stroke
Bleeding
Labile INRs
Elderly (>65 years)
Drugs that increase bleeding or alcohol
```
Score 3 or more = high risk of bleeding
182
What antibodies are present in the majority of patients with primary Sjogren's syndrome?
Antibodies against SSA/Ro - found in about 75% of patient with primary Sjogren's and in 15% of patients with secondary Sjogren's
183
What are the 4 species of Shigella?
Shigella dysenteriae: most severe disease
Shigella flexneri
Shigella boydi
Shigella sonnei: mildest disease
Shigella sonnei is commonly seen in the UK
184
What are the clinical features of scrub typhus?
Scrub typhus is a rickettsial spotted fever occurring in Thailand caused by Orientia tsutsugamushi. It is transmitted by the bite of a trombiculid mite. The incubation period is 5-10 days.
The illness starts abruptly with fever, headache, myalgia, nausea, vomiting and cough. A rash appears on day 5-7 on the trunk and limbs.
It can be complicated by meningoencephalitis, myocarditis, shock and ARDS
185
What condition causes ochronotic pigmentation, ochronotic arthritis and darkening of urine on standing?
Alkaptonuria
-----------------------
This is caused by the deficiency of homogentisic acid oxidase which converts homogentisic acid to acetoacetic acid.
Homogentisic acid which accumulates in the urine oxidases on standing, turning the urine black.
The tissues may become darkened, described as ochronosis, and there may be associated degenerative changes.
This condition in contrast to phenylketonuria, has no effect on intelligence.
186
How do you differentiate between femoral nerve, obturator nerve and common peroneal nerve neuropathy?
Femoral (L2-L4)
- Weakness of quadriceps
- Loss of sensation over anterior aspect of thigh
- Decreased or absent knee reflex
Obturator (L2-L4)
- Weakness of hip adductors
- Loss of sensation over lateral thigh
Common Peroneal (L4 root)
- Foot drop
- Loss of sensation over lateral aspect of calf and dorsum of foot.
187
how do you differentiate between CN III, CN IV, and CN VI palsies?
CN III (oculomotor)
- Ptosis
- Down and out gaze
CN IV (trochlear)
- Vertical diplopia
- Loss of downward gaze
CN VI (abducens)
- Medial deviation of the eye
- Loss of lateral gaze
188
Why is someone with renal failure at an increased risk of postoperative bleeding?
Uraemia impairs platelet function and leads to increased bleeding time.
189
What are the 4 types of erythropoietin-secreting tumours?
Cerebellar haemangiomas
Uterine Fibromas
Renal Cell Carcinoma
Liver Hepatomas
190
Which autoantibodies are associated with congenital complete heart block in a mother who has SLE during pregnancy?
Anti-Ro (SSA) antiodies
191
Which type of renal calculi is commonly seen following small bowel resection?
Oxalate stones
Enteric oxaluria occurs in malabsorptive conditions where there is in excessive colonic absorption of oxalate.
192
What are the features of Felty's syndrome?
SANTA
```
Splenomegaly
Anaemia
Neutropenia
Thrombocytopenia
ANA positive in 90% of patients
```
193
What is Evan's Syndrome?
Idiopathic Thrombocytopenic Purpura
AND
Autoimmune Haemolytic Anaemia
194
What EEG changes can you see in HSV-1 encephalitis?
Lateralised periodic discharges at 2Hz
195
What are some of the side effects of treating CKD related anaemia with erythropoietin?
Anaphylaxis
Hypertensive crises
Headaches, encephalopathy and seizures
Hyperkalaemia
Thrombosis (e.g. in fistulas for dialysis)
Pure red cell aplasia
Fe deficiency secondary to increased erythropoiesis
196
What are the features of Type 1 and Type 2 autoimmune polyendocrinopathy syndrome (APS)?
Type 1 APS
- Autosomal recessive mutation of AIRE1
- At least 2 of the following must be present
- -Addison's Disease
- -Chronic candisiasis
- -Hypoparathyroidism
Type 2 APS
- Polygenic inheritance linked to HLA DR3/DR4
- Addison's disease AND autoimmune thyroid disease +/- Type 1 DM
197
What do you see on a blood film in microangiopathic haemolytic anaemia?
Helmet cells
Schistocyte
Reticulocytes
198
How does heparin work and if thrombosis occurs while on it what should be considered?
Increases antithrombin levels
This inhibits thrombin and factor 10a which leads to decreased thrombosis (the anticoagulant effect of heparin)
--------------------------
If there is ongoing thrombosis in the presence of heparin, antithrombin III deficiency should be considered.
199
What are the features in Holmes-Adie's Pupil?
The Holmes-Adie pupil is dilated and irregular. It constricts slowly and incompletely to light.
Once the pupil has constricted, it remains small for an abnormally long time (tonic pupil).
This results in a decreased direct and consensual light reflex.
-------------------------
It also has absent deep tendon reflexes.
200
How do you differentiate between Type 1 and Type 2 heparin induced thrombocytopaenia (HIT)?
Type 1 HIT
- Onset: 1-4 Days
- Nadir platelet count >100
- Immune complexes: No
- Thrombosis: No
- Treatment: Observation
Type 2 HIT
- Onset: 5-14
- Nadir platelet count: 50-100
- Immune complexes: Yes (IgG-Heparin-Platelet factor 4
- Thrombosis: Arterial and venous
- Treatment: Stop heparin, Change to an alternative anticoagulant to prevent thromboses.
201
Which classes of anticoagulants can be used to treat the thrombotic phenomena seen in type 2 heparin induced thrombocytopenia (HIT)?
Direct thrombin inhibitors e.g. argatroban
Factor 10a inhibitors e.g. danaparoid, fondaparinux
202
What are the causes of an acute red eye?
Acute angle closure glaucoma
- Painful and sight threatening
- Semi-dilated, non-reactive, oval-shaped pupil with a hazy cornea
- Associated with hypermetropia
Anterior uveitis/ iritis
- Painful
- Small, fixed pupil with ciliary flush
- Associated with systemic diseases (e.g. IBD, ank spond, reactive arthritis
Episcleritis
- Painful
- Pain is worse on movement, tender nodularity to sclera
- Associated with underlying autoimmune disease (e.g. RA, SLE, IBD).
Sub-conjunctival haemorrhage
- Not painful
- Self-limiting, benign
203
What do you look for on flow cytometry in paroxysmal nocturnal haemoglobinuria (PNH)?
Decreased CD59 and CD55
204
How does clostridium tetani cause trismus, risus-sardonicus and opisthotonus?
Decreased release of GABA through the exotoxin tetanospasmin
205
What are the acquired and congenital causes of optic atrophy?
Acquired
- MS
- Chronic papilloedema
- Glaucoma
- Methanol metabolism (formate)
- Smoking
- B1, B2, B6, B12 deficiency
Congenital
- Friedreich's Ataxia
- Wolfram's Syndrome
- Leber's Optic Atrophy
206
What appearance is seen on a bone marrow biopsy with hairy cell leukaemia?
Fried egg appearance - malignant B-cells have villous projections that stain positive with TRAP
In spite of bone marrow hypercellularity, there is often a "dry tap" on marrow aspiration
-------------------------------
Features include hepatosplenomegaly, pancytopenia and a good response to purine-analogue chemotherapy.
207
Which conditions cause irregular dark-red streaks on the retina?
```
These are angioid retinal streaks seen in:
-Paget's Disease
-Sickle-cell disease
-Acromegaly
-Ehlers-Danlos Syndrome
Pseudoxanthoma elasticum
```
208
What conditions are associated with retinitis pigmentosa?
```
Abetalipoproteinaemia
Kearns-Sayre Syndrome
Refsum's Disease
Usher's Syndrome
Alport's Syndrome
Lawrence-Moon-Biedl syndrome
```
209
What is the triad of retinitis pigmentosa?
Night blindness
Tunnel vision
Pigmented bony spicules on fundoscopy
210
What is the role of methylprednisolone in treating optic neuritis in multiple sclerosis?
Decreased pain
Increased rate of recovery
----------------------
There is no evidence that steroids change the eventual neurological outcome e.g. in terms of visual acuity or have any impact on the likelihood of future relapses.
Before starting steroids, it is important to exclude the presence of an intercurrent infection which is a common trigger for relapses in MS
Steroids and antimicrobials can be given together depending on the severity of the infection
211
What are the 5 causes of a raised transfer factor in spirometry?
ALPHA
Asthma
Left to right cardiac shunts
Polycythemia
Haemorrhage (e.g. Wegener's Goodpastures)
Athletics (e.g. exercise, especially in males)
212
What is seen on blood film in brucellosis?
A leucoerythoblastic picture
213
Which conditions are associated with thymomas?
```
Myasthenia gravis
SLE
Dermatomyositis
SIADH
Red Cell Aplasia
------------------------------
Thymomas are the most common tumour of the anterior mediastinum
```
214
What are the diagnostic criteria for MGUS?
Monoclonal protein spike <3g/dl
Plasma cells occupy < 10% of the bone marrow
There are no CRAB features:
-hyperCalcaemia, Renal failure, Anaemia, Bone lesions
215
What is the first line drug to treat focal epilepsy secondary to a stroke?
Lamotrigine
The SANAD study
216
How would you clinically differentiate between chancroid and genital herpes if someone presents with a painful genital ulcer and inguinal lymphadenopathy?
Chancroid presents with a single ulcer and a shorter history of symptoms (e.g. a week)
Herpes usually presents with more than one ulcer and a longer history of symptoms
217
What is the daily risk of a major thromboembolic event in a patient with a mechanical mitral valve without any form of anti-coagulation?
0.02%
218
What class of drug do you use to treat a scleroderma renal crisis?
ACE-i
A scleroderma renal crisis is characterised by marked over activation of the renin angiotensin aldosterone system
219
Which antihypertensives should you avoid if someone has ultrasound evidence of renal asymmetry?
ACE-i and ARBs
Renal asymmetry implies renal artery stenosis
The gold standard test for diagnosis is MRA.
220
What mutation needs to be present for ibrutinib to be used in the treatment of chronic lymphocytic leukaemia (CLL)?
TP53 mutation
-------------------------
Ibrutinib is an inhibitor of Bruton's tyrosine kinase
221
Why are patients with advanced prostate cancer pre-loaded with bicalutamide before starting goserelin?
To decrease goserelin-induced tumour flare
Normally hypothalamus produces GnRH which binds to receptors on gonadotrophin producing cells in the pituitary to release LH and FSH. LH stimulates Leydig cells in the testes to produce testosterone.
Goserelin is a synthetic GnRH agonist. When goserelin binds to the GnRH receptors in the pituitary, its agonistic activity causes immediate release of LH and FSH resulting in increased production of testosterone and tumour flare. This worsens symptoms.
Bicalutamide is an androgen receptor antagonist. Pre loading with bicalutamide blocks the tumours androgen receptors so that the surge in testosterone does not cause tumour flare. It is usually given 3-7 days before starting goserelin and continued for about 3 more weeks.
After a few weeks of treatment with goserelin, GnRH receptors in pituitary cells are downregulated and LH/FSH production are subsequently ceased.
222
What is the most common organism to cause meningitis complicating an ear infection?
Streptococcus pneumoniae
223
In which 2 conditions would you expect the pleural fluid amylase to be elevated?
Oesophageal rupture
| Pancreatitis
224
What is the most likely diagnosis if a patient develops livedo reticularis, skin ulceration and eosinophilia 1 month after starting warfarin?
Cholesterol Embolisation
Warfarin is a recognition precipitant of cholesterol atheroemboli
225
What CXR lesions are seen in Klebsiella pneunomia and what type of sputum is produced?
Cavitation in the upper lobes and thick blood-stained (currant jelly) sputum
Klebsiella pneumonia typically occurs in middle-aged alcoholic men
226
In peripheral vertigo how do you determine the side of the lesion?
Unsteady gait and veer to left
Horizontal nystagmus with the fast component to right
Abnormal head impulse test on the left
227
Which drug do you use to normalise the low calcium and raised PTH levels in someone with CKD?
Alfacalcidol (1-a hydroxycolecalciferol)
--------------------------
Patients with CKD have a deficiency of 1-a-hydroxylase. Therefore they are not able to adequately activate Vitamin D. This leads to impaired gut absorption of calcium and hypocalcaemia.
This causes an increased parathyroid hormone level (secondary hyperparathyroidism) in an attempt to restore the serum calcium concentration to normal.
Correction of the vitamin D deficiency is the key to restoring normal calcium homeostasis and must be done with alfacalcidol.
228
What is the cut off time for assessment and surgery in symptomatic carotid artery stenosis?
Assessment for carotid endarterectomy within 1 week
Surgery within 2 weeks
229
How would you administer flumazenil to someone with low GCS and respiratory failure after an overdose of diazepam?
200 microgram over 15 seconds
An additional 100 microgram can be given every minute up to a total of 1 mg (or 2 mg on the ICU)
230
What are some screening and scoring tools available in hospital to assess depression in the elderly?
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Geriatric depression scale
Hospital anxiety and depression scale
Brief assessment schedule depression cards
Cornell scale
Beck inventory
```
231
What criteria should be met upon discharge in someone admitted with an exacerbation of asthma?
- PEFR > 75% best/ predicted
- Diurnal peak flow variability < 25%
- Should be on discharge medication for 12-24 hours
- Inhaler technique checked and record
- Should have a peak flow meter and a written asthma action plan in the event of deterioration
- GP follow up arranged within 2 working days
- Respiratory clinic follow-up within 4 weeks
232
What does inhaling chemical paraffin cause?
Hydrocarbon pneumonitis
------------------------
Presents with cough, bronchospasm, tachypnoea, and pulmonary oedema.
Often requires intubation and ventilation as symptoms can progress and worsen for up to 72 hours before resolving.
233
What is Katayama Syndrome?
Manifestation of acute schistosomiasis
------------------
It most often occurs with Schistosoma japonicum and mansoni
Typical features include fever, an urticarial rash, hepatosplenomegaly and bronchospasm
234
What is bullous myringitis?
This is where bullae filled with blood form on the surface of the tympanic membrane and burst with effusing blood.
Seen in Mycoplasma Pneumonia
235
What do you see on histology of a sub epidermal blister in someone with bullous pemphigoid?
IgG and C3 at the dermoepidermal junction
236
What is first line for cancer related VTE?
LMWH
---------------------
Recurrence is lower compared to warfarin
NOACs can be used but have not been shown to be equivalent or better efficacy and safety
237
How do the levels of plasma renin differ between Conn's Syndrome and Fibromuscular Dysplasia of the renal artery?
Both conditions present with features of hyperaldosteronism:
- Increased aldosterone, BP and pH (metabolic alkalosis)
- Decreased potassium
However the mechanism of hyperaldosteronism is different and can be determined by the plasma renin:
CONN'S SYNDROME
- The adrenal gland produces high amounts of aldosterone
- This suppresses plasma renin levels through negative feedback
FIBROMUSCULAR DYSPLASIA OF THE RENAL ARTERY
- Fibromuscular dysplasia leads to renal artery stenosis
- This causes renal hypoperfusion which stimulates increased renin
- Increased renin triggers increases aldosterone release
238
What are the 2 important drug interactions of adenosine?
Dipyridamole increases the effect
Aminophylline decreases the effect
239
Which 2 antibiotics commonly cause cholestasis?
Flucloxicillin and Co-amoxiclav
240
Which protein is implicated in pemphigus vulgaris?
Desmoglein-3
241
What causes U-waves on ECG?
Hypokalaemia
242
What type of antibody is rheumatoid factor?
IgM antibody against IgG
243
What is the genetics of McCune-Albright Syndrome?
Caused by a mutation in the GNAS1 Gene
This mutation occurs after fertilisation of the embryo (somatic mutation) and is therefore not inherited.
244
What is the most common cause of death in Friedreich's Ataxia?
Hypertrophic obstructive cardiomyopathy
245
What is the most common cause of cyanotic congenital heart disease?
Transposition of the great arteries is the most common at birth.
Tetralogy of Fallot is the most common overall
--------------------------
In TOF an echo will show a right ot left shunt through a VSD, right ventricular outflow tract obstruction with associated right ventricular hypertrophy and an overriding aorta.
246
Which syndrome causes a sudden increase in appetite and sexual desire in someone who was recently treated for herpes simplex encephalitis?
Kluver-Bucy syndrome
--------------------
This occurs with lesions affecting both temporal lobes (especially the hippocampus and amygdala)
247
Which nerve is implicated if someone has a positive Trendelenburg sign?
Superior gluteal nerve.
248
What syndrome causes persistent infantile unconjugated hyperbilirubinaemia that improves with phenobarbital?
```
Crigler-Najjar Syndrome (Type 2)
--------------------
This is an autosomal recessive disorder in the UDP-glucuronosyl-transferase (UGT) gene which codes the enzyme needed for glucuronidation of bilirubin
-Type 1: absence of UGT activity
-Type 2: severe decrease in UGT activity
```
249
What triad of clinical features do you see in Shy-Dragger syndrome?
Parkinsonism - features respond poorly to dopamine agonists and to L-dopa
Cerebellar signs
Autonomic disturbance - e.g. loss of sphincter control, atonic bladder and urinary retention, impotence, anhidrosis and postural hypotension
250
What is the pathophysiology of acute intermittent porphyria (AIP)?
AIP is caused by a defect in PBG deaminase in RBCs. This leads to toxic accumulation of porphogilinogen (PBG) and delta aminolaevulinic acid (DALA)
-----------------------
Once key feature is that the urine turns deep red on standing (increased urinary excretion of PBG)
Stress, infection, pregnancy, menstruation, the OCP and starvation (low carbohydrate intake may precipitate acute attacks)
Generally speaking, cP450 enzyme inducers are associated with increase risk of acute attacks e.g. phenytoin and chronic alcohol intake.
251
What is the anatomy of a pharyngeal pouch?
What are the clinical features?
Posteromedial diverticulum through Killian's dehiscence.
This dehiscence is a triangular area in the wall of the pharynx between the thyropharyngeus and cricopharyngeus muscles.
----------------------
It is common in older males
Clinical features include:
- Dysphagia to solids and liquids
- Regurgitation of rotten undigested food which results in halitosis
- Nocturnal chronic cough
- Aspiration pneumonia
- Neck swelling which gurgles on palpation
252
What are the features of syringomyelia?
Wasting and weakness of (in particular) intrinsic hand muscles
Spinothalamic sensory loss (pain and temp)
Absent tendon reflexes
253
What virus causes molluscum contagiosum?
DNA poxvirus
Molluscum contagiosum is highly infectious. It is typically seen in younger children and results in small, pearly, umbilicated (central dimple) lesions.
254
How does the motor presentation differ between humeral neck fracture and humeral midshaft fracture?
Neck fracture: Difficulty in abducting the arm due to axillary nerve involvement
Midshaft fracture: Wrist drop due to radial nerve involvement
255
Which diagnostic markers are most sensitive to test for neuroblastoma?
Urinary vanillylmandelic acid (VMA) and/or homovanillic acid (HVA)
These are metabolites of catecholamines
256
Which ligament is inflamed in Osgood-Schlatter disease?
The patellar ligament at the tibial tuberosity
257
What are the 3 stages of lymphogranuloma venereum?
Stage 1: small painless pustule which later forms an ulcer
Stage 2: painful inguinal lymphadenopathy
Stage 3: Proctocolitis - the inflammation can be so severe that it causes a fistula and mimics Crohn's disease
-------------------------------
Caused by Chlamydia trachomatis
258
How does ribavarin cause a raised bilirubin when it is used to treat Hepatitis C?
It causes haemolytic anaemia
259
What does HBeAG represent if it is positive?
It is a marker of infectivity and replication in active hepatitis B virus (HBV) infection. It results from the breakdown of core antigen from infected liver cells.
A minority of people with active HBV infection will have a negative HBeAG. These people are infected with a mutant HBV which is unable to make HBeAg e.g. stop-codon mutation at 1896
260
What is the genetics of Juvenile Myoclonic Epilepsy?
Linked to the short arm of chromosome 6.
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The most common primary generalised epilepsy syndrome in early adulthood.
261
What is the genetics of Charcot-Marie Tooth Disease?
Autosomal dominant defect in PMP-22 gene which codes for myelin.
-------------------
The most common inherited neuropathy.
AKA Hereditary motor-sensory neuropathy type 1
Motor symptoms predominate
e.g. distal muscle wasting, pes cavus, clawed toes and foot drop.
262
Which type of lung cancer is most associated with asbestos exposure?
Adenocarcinoma
263
What is the most common site of paraganglionomas?
Organ of Zuckerkandl - chromaffin body located at the bifurcation of the aorta or the origin of the inferior mesenteric artery
264
What is the most common benign tumour of the small bowel?
Gastrointestinal stromal tumour (GIST) - originate from the interstitial cells of Cajal or precursors of these cells.
265
Which tumour is known to cause diabetes and necrolytic migrating erythema?
Glucagonoma
266
Which condition causes developmental delay, infantile spasms, facial hair, eczema and blue eyes in a 6-month-old baby?
Pnenylketonuria - an autosomal recessive defect in phenylalanine hydroxylase which converts phenylalanine to tyrosine (chromosome 12)
Increased phenylalanine accumulation leads to neurological problems such as learning difficulties and seizures
There is a "musty" odour to the urine and sweat because of pheylacetate and phenylketone
267
What surgical procedure can you perform if ventricular arrhythmias continue to occur in long-QT syndrome despite beta-blockers and frequent ICD shocks?
Left stellate cardiac ganglionectomy
This is an invasive procedure and can result in the development of Horner's Syndrome
268
Mutations in which genes are responsible for hereditary non-polyposis colorectal cancer?
hMSH2 and hMLH1 mismatch repair genes
269
Why would someone with acute diverticulitis have raised red cells and white cells on urinalysis?
Because of secondary periureteric inflammation.
270
What are the classifications and causes of lactic acidosis?
Type A: Tissue hypoperfusion (e.g. shock, anaemia, ischaemia, excess muscle/ muscle fatigue)
Type B: normal tissue perfusion
-B1: Underlying disease - renal/ liver failure, Malignancy, HIV/AIDS, Ketoacidosis, Phaeochromocytoma
- B2: Medication and Toxins
- -Metformin, B-agonists, propofol
- -CO, cyanide, alcohol, ethylene glycol
- B3: Inborn errors of metabolism
- -G6PD
- -Pyruvate DH deficiency
271
Which 2 drug classes are most associated with acute tubulointerstitial nephritis?
Penicillin and NSAIDs - these cause interstitial inflammation which can later progress to interstitial fibrosis
----------------------------------
The common triad of presentation is a rash, fever and eosinophilia
Eosinophiluria is pathognomonic and a renal biopsy is needed for confirmation.
272
How does the phenotype differ between maternal and paternal inheritance when a microdeletion is inherited on chromosome 15?
```
Maternal = angelMan syndrome
Paternal = Prader-willi
```
273
Which NSAID is most commonly associated with convulsions?
Mefenamic acid.
274
What is the most characteristic X-ray finding in rheumatoid arthritis?
Periarticular osteopenia
275
What septal wall thickness is associated with a poor prognosis in hypertrophic obstructive cardiomyopathy?
Septal wall thickness > 3cm
276
What is the difference in pathophysiology between a pseudobulbar and bulbar palsy?
Pseudobulbar
- Bilateral damage of the corticobulbar tracts to the nerve nuclei of CNs 5, 7, 9, 10, 11, 12
- This leads to UMN features of the respective muscles (e.g. spastic paralysis of the tongue and exaggerated gag and jaw jerk reflexes)
Bulbar
-Bilateral damage to the CNs 9, 10, 11, 12 or to their nuclei in the medulla
-This leads to LMN features of the respective muscles (e.g. flaccid paralysis, atrophy and fasciculations of the tongue and an absent gag reflex)
------------------------------------
Since the cranial nerve nuclei in the brain stem are paired, unilateral lesions are often asymptomatic
277
What changes in SpO2 would you expect to see during cardiac catheterisation in a structurally normal cardiovascular system?
The right atrium, right ventricle and pulmonary artery normally have oxygen saturation levels of around 70%
-Deoxygenated blood returns to the right side of the heart via the SVC and inferior vena cava IVC
The left atrium, left ventricle and aorta all have oxygen saturation levels of 98-100%
-The lungs oxygenate the blood
--------------------------------
Any changes in these levels may suggest the presence of a shunt.
278
What is the most common causative organism of neonatal pyogenic meningitis?
E. Coli
---------------------
Usually caused by serotype that contains the capsular antigen K-1
279
What is the most common cause of biliary disease in patients with HIV?
Sclerosing Cholangitis
This is secondary to infections such as Cryptosporidium, CMV and microsporidia
MRCP/ ERCP is used for diagnosis.
280
What organisms cause Elephantiasis?
Wuchereria Bancrofti
-A filarial nematode which is spread by a mosquito vector
------------------------------
Treatment is with diethylcarbamazine
Other less common organisms that can cause a similar presentation include Brugia malayi and Brigia timori
281
How does riluzole work in treating amyotrophic lateral sclerosis?
It prevents stimulation of glutamate receptors
Prolongs life by about 3 months
282
What is the most common cause of cutaneous larva migrans?
Ancylostoma braziliense
-This is common in Central and Southern America
----------------------
Treatment is with Thiabendazole
283
What is the mechanism of action of sorafenib?
Protein kinase inhibitor
It has activity against many protein kinases e.g. VEGFR, PDGR and RAF kinases
Used to treat hypernephromas, hepatocellular carcinoma, FLT3-ITD positive AML and radioactive iodine resistant advanced thyroid cancer
284
What intravenous drug would you give if a 30 year old with asthma develops SVT?
IV verapamil or flecainide
Adenosine is contraindicated in asthma
