BPT Trial Exam Questions 2

Question 1

A 50 year old man is reviewed by a new general practitioner for hypertension which was diagnosed several years ago. His current antihypertensive medications are perindopril 5 mg daily, amlodipine 10 mg daily, and metoprolol 25 mg twice daily. He takes PRN celecoxib for knee pains. On examination, he is overweight with a BMI of 30. His blood pressure was 180/110, similar his home readings. There were no other exam findings of note. Blood tests demonstrated: Na 147, K 3.5, Cl 110, Bicarbonate 32. The general practitioner wonders whether this man may have primary hyperaldosteronism. Which of the following is INCORRECT regarding the patients medications and false positives/negatives of aldosterone/renin testing for screening?

A. Perindopril, false negative
B. Amlodipine, false positive
C. Metoprolol, false positive
D. Celecoxib, false positive

Answer 1

Answer: B - Amlodipine, false positive

When testing aldosterone : renin (ARR), drugs that are ok to continue:

• Verapamil
• Hydralazine
• Prazosin

Most reliable test for hyperaldosteronism is the ARR in the mid-morning seated position >20

Hold diuretics 6 weeks
Other drugs hold 2-4 weeks

Increase renin (false negative ARR) = ADD

• ACEi/ARBs
• Diuretics (spironolactone)
• Dihydropine calcium channel blockers (i.e. Amlodipine)

Reduce renin (false positive ARR) = ABCD

• Alpha-methyldopa
• Beta-blockers
• Clonidine
• Diclofenac (NSAIDs)
• Diuretics

Question 2

A 76 year old lady presents to ED with severe muscle cramps and peri-oral paraesthesia. She has a corrected calcium level of 1.6 mmol/L (Ref 2.10 - 2.55). She has a background of hypertension, chronic kidney disease (GFR 40), osteoporosis and congestive cardiac failure. She was recently commenced a new antihypertensive medication but was unable to recall which. She also reported getting a subcutaneous injection 2 weeks ago for osteoporosis but is also unable to recall what drug this is. Which of the following is most likely to be the cause of her hypocalcaemia?

A. Thiazide diuretic
B. Spironolactone
C. Denosumab
D. Zoledronic acid

Answer 2

Answer: C - Denosumab

Feedback
Denosumab is the most likely cause of hypocalcaemia in this case. The nadir of hypocalcaemia is usually 2 weeks post dose. Patients with CKD are more likely to experience hypocalcaemia.

Thiazides are usually associated with hypercalcaemia. Spironolactone is usually associated with hyperkalaemia.

Paying attention to detail in the question is important. Zoledronic acid is given intravenously and NOT subcutaneously, therefore, it is not the answer.

Question 3

A 79 year old male presents with dyspnoea and cough on the background of idiopathic pulmonary fibrosis. You see he is on nintedanib. What is the main rationale for using nintedanib in idiopathic pulmonary fibrosis?

A. Reduced need for long term oxygen therapy
B. Reduction in FVC decline
C. Reduction in time to exacerbation
D. Improvement in quality of life

Answer 3

Answer: B - reduction in FVC decline

Treatments for ILD:
o Steroids have no benefit and increase mortality
 - PANTHER STUDY terminated early due to worse
outcomes with steroids
 This is in contrast to NSIP/CTD related ILD,
where prednisolone 1st line
o Anti-fibrotic therapy; Nintedanib & Pirfinedone – the only agents which alter disease progression
 - Suitable only for mild-moderate IPF
 - Must be diagnosed by MDM as definite or
possible IPF and have FVC >50%, FER >70%
DLCO >30%
 Nintedanib is a tyroskine kinase inhibitor (multiple TKs)
• Slows rate of FVC decline but unclear survival
benefit and no benefit to QoL (SEs)
• Diarrhoea (>60%), Nausea, (25%), LFT
derangement
 Pirfenidone is an anti-fibrotic which inhibits TGF-beta and fibroblasts
• Slows FVC decline; not a cure with variable
benefits on 6-minute walking distance and O2
saturations
• Survival benefit unclear
• SEs rash (30%), nausea and diarrhoea (35%)

Question 4

A 16 year old male is reviewed in Endocrinology clinic after an incidental finding of hypertension is made by his general practitioner.
It is noted that he has a family history of hypertension, and the GP performs some testing:
Na 140, K 2.6Cl 110, HCO3 37,
Renin: low, Aldosterone: low,
Serum metanephrines: normal,
Urinary cortisol/cortisone ratio: normal,
Low dose dexamethasone test: normal.

Which of the following statements is INCORRECT regarding his condition?

A. His condition is inherited in autosomal dominant condition
B. Both amiloride as well as spironolactone can be used to improve his hypertension and metabolic findings
C. Genetic testing should be performed for mutations in collecting tubule sodium channel
D. The potassium sparing triamterene can be used

Answer 4

Answer: B - both Amiloride as well as spironolactone can be used to improve his hypertension and metabolic findings

Feedback
Patient has Liddle’s syndrome, which is a problem due to activating mutation in ENAC in the cortical collecting tube. Unlike apparent mineralocorticoid excess where the problem is cortisol binding to MR receptor, spironolactone won’t work - need to block the ENAC channel with potassium sparing diuretic
———————————–

Low renin + High Aldosterone = 1 hyperaldosteronism
- Bilateral adrenal hyperplasia, Aldosterone producing adenoma (Conn’s), familial hyperaldosteronism

High renin + High Aldosterone = 2 hyperaldosteronism

• Renal artery stenosis, diuretics (?surreptitious), renin-secreting tumour
• Barter/Gitelman syndrome (cause hypotension)

Low renin + Low aldosterone = apparent mineralocorticoid excess
- exogenous mineralocorticoids, Cushing’s syndrome, liquorice ingestion, Liddle’s syndrome, CAH

Liddle’s syndrome
• Autosomal dominant condition with activating mutation in the ENaC (epithelial Na channel) = amiloride-sensitive sodium channel
o Manifests as mineralocorticoid excess with:
o Hypertension, hypokalaemia and metabolic alkalosis – usually in children
• Biochemically causes apparent mineralocorticoid excess i.e. low renin and aldosterone
• Treatment = potassium sparing diuretics which directly block the tubular Na channels
o Amiloride or Triamterene
o Spironolactone is not effective (antagonizes mineralocorticoid receptor rather than direct effect on channel – low aldosterone level anyway)

Question 5

A 70 year old gentleman presents to the emergency department with cachexia, shortness of breath, and weight loss. A CT scan demonstrates a large right sided pleural effusion that is subsequently drained by the emergency department. Pleural studies demonstrate exudative chemistries. No malignant cells identified. A repeat CT is done demonstrating a large mass in the right lower lobe measuring approximately 8 cm. There is also some associated with right sided hilar lymphadenopathy with normal lymph nodes elsewhere. CT brain/abdomen/pelvis demonstrates no distant metastases. Which of the following most correctly describes his most LIKELY TNM staging?

A. Stage II
B. Stage IIIA
C. Stage IIIB
D. Stage IV

Answer 5

Answer: D - stage IV

Feedback
The size of the tumour is pretty big with hilar lymphadenopathy, probably at least a stage III. However, the pleural effusion is most likely to be malignant despite the negative cytology (sensitivity is not that high) which means that he stage 4. Malignant pleural effusions are M1 in the TNM staging

Question 6

A 45 year old man with a history of spondyloarthritis on celecoxib, golimumab and oral and intra-articular steroids presents with haematemesis and melena and on endoscopy is found to have a large duodenal ulcer with biopsy positive for H. Pylori. Which of the following are TRUE regarding treatment of H. Pylori:

A. Treatment of H Pylori can cause transformation of low grade MALT lymphoma to high grade lymphoma
B. Levofloxacin resistance is more common than clarithromycin resistance
C. Approximately half of H Pylori cases are resistant to metronidazole
D. Colloidal bismuth can replace clarithomycin in second line therapy with equal efficacy

Answer 6

Answer: C - approximately half of H. Pylori cases are resistant to Metronidazole

Feedback
“Metronidazole resistance is common. Levofloxacin < amoxicillin < Clarithromycin in terms of resistance.
Colloidal bismuth is used in 2nd line therapy along with a PPI and antibiotics, but not replacing any antibiotic agent.
Treating H Pylori is first line therapy in low grade gastric MALTs associatd lymphomas “

H. Pylori 1st line treatment = Esomeprazole, Amoxicillin, Clarithromycin for 7 days

• Success rate 85-90% in trials
• Less in real life due to adherence and resistance. Metronidazole resistance is very common (50%), clarithromycin is rare (6-8%), with Amoxicillin extremely rare

2nd line therapy after failure of eradication:
10 days of: 1. PPI BD, 2. Amoxicillin BD, 3. Levofloxacin 500mg BD

Question 7

60 year old, professional dragon boat racer with altered bowel habit and iron deficiency anemia found to have a right sided colorectal cancer. CT staging does not reveal any metastasis. He undergoes a right hemicolectomy and histology demonstrates an adenocarcinoma with vascular invasion but negative nodes. Unfortunately 6 months later he presents with distant metastatic disease. Which of the following is FALSE?

A. Right sided colorectal cancer is more common in patients with germline mismatch repair deficiency
B. Right sided colorectal cancers are more likely to be sensitive to EGFR therapy
C. Patients with stage II disease and high risk features derive a 50% survival benefit from adjuvant chemotherapy
D. FDG-PET scan is not recommended prior to surgery for colorectal cancer to identify distant metastasis

Answer 7

Answer: B - Right sided colorectal cancers are more likely to be sensitive to EGFR therapy

Feedback
Colorectal cancer hot topics: Right vs Left sided and hereditary syndromes.
Right vs left becomes relevant in metastatic setting with:

Right sided cancers unlikely to benefit from EGFR therapy, more likely to have RAS/PIK3CA and BRAF mutations and be more poorly differentiated. They are also more likely to be related to lynch syndrome.

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6089587/

Adjuvant therapy in stage II disease has a ~5% overall survival benefit
PET scans are not standard practice.

Question 8

A 40 year of female is recently diagnosed with hormone positive breast cancer. She has been managed with surgical resection and is now on adjuvant endocrine therapy with tamoxifen. She describes having regular hot flushes. Her past medical history is unremarkable except for a longstanding history of depression which is now recently worsened in the context of her illness. Pharmacotherapy is considered. Which of the following agents would be LEAST suitable to start?

A. Paroxetine
B. Citalopram
C. Venlafaxine
D. Duloxetine

Answer 8

Answer: A - Paroxetine

Feedback
Tamoxifen is metabolised to its active metabolite endoxifen by CYP2D6. Medication that inhibit CYP2D6 can interfere with this. Antidepressants can be used to help treat hot flushes associated with tamoxifen use, however certain antidepressants can lead to less efficacy.

Fluoxetine and paroxetine do this the most and should be avoided

Question 9

A 35 year old gentleman presents to hospital with sudden onset of right hand numbness associated with loss of cool sensation. Over the next 24 hours he develops diploplia, as well as vertigo. He begins to lose balance on mobilisation and is now unable to take a few steps before falling. His past medical history is unremarkable except for a heavy alcohol history drinking 1-2 bottles of wine a night. On examination he has right lateral rectus palsy, sustained nystagmus on lateral gaze, broad base gait. His motor examination is normal. Reflexes are all intact. A MRI is ordered. Where is the most specific abnormality seen in this gentleman’s condition?

A. Third ventricle
B. Mamillary bodies
C. Cerebellum
D. Red nucleus

Answer 9

Answer: B - Mamillary bodies

Feedback
Atrophy of the mamillary bodies is the most specific abnormality seen in Wernicke’s

Question 10

A 60 year old female is recently diagnose with renal cell cancer after presenting with abdominal pain. Her staging scan was performed which demonstrated multiple lung lesions with mediastinal lympadenopathy. EBUS performed demonstrated clear cell carcinoma. She is commenced on sunitib therapy. Which of the following side effects most strongly correlates to response against her disease?

A. Acneiform rash
B. Hypertension
C. Hypothyroidism
D. Bone marrow suppression

Answer 10

Answer: B - Hypertension

Feedback
Sunitib/pazopanib are cause hypertension. This correlates to their activity, especially the diastolic pressure. The acneform rash is more a side effect of the EGFR inhibitors, and correlates to disease activity when used in that setting.

Question 11

Which of the following cancers is LEAST associated with BRCA gene mutations?

A. Pancreatic
B. Prostate
C. Ovarian
D. Thyroid

Answer 11

Answer: D - thyroid

Feedback
BRCA mutations are associated with prostate, pancreas, and ovarian. Thyroid less so

Question 12

A 40 year old man presents to hospital with shortness of breath for the past 6 months. He is found be severely pancytopenic with Hb 60, WCC 2, platelets of 60.

He has no significant past medical history, takes no regular medications, and has always lived in Australia. A bone marrow biopsy is performed which shows a profoundly hypocellular marrow with decrease in all elements, composition mostly of fat and stroma.

A decision is made for initial treatment with immunosuppresive therapy. Which of the following agents can be added to marked improve his overall response rate?

A. G-CSF
B. IL-2
C. Eltrombopag
D. GM-CSF

Answer 12

Answer: C - Eltrombopag

Feedback
Patient has aplastic anemia. Can be treated with stem cell transplant as first line if younger. If a bit older, can try IS first with ATG and cyclosporine. Eltrombopag significantly improves overall response. The other agents have had minimal effect

Aplastic anaemia management

• stem cell transplant if young
• Intensive immunosuppression (if not transplant) = horse anti-thymocyte globulin (ATG), cyclosporine + bone marrow stimulation with Eltrombopag

Question 13

Cisplatin is a medication used in several cancers. Which of the following is the most likely significant adverse effect of cisplatin?

A. Cardiac failure
B. Pulmonary fibrosis
C. Severe diarrhea
D. Hearing loss

Answer 13

Answer: D - hearing loss

Feedback
One of the main side effects of cisplatin is neurological, the two most common manifestations being peripheral neuropathy (sensory) as well as ototoxicity.

Question 14

Which of the following is NOT part of the diagnostic criteria for neurofibromatosis type 1?

A. Lisch nodules
B. Optic glioma
C. Meningiomas
D. Axillary freckling

Answer 14

Answer: C - meningiomas

Feedback
Meningiomas part of NF-2

Question 15

A 30 year old gentleman is seen in respiratory clinic for asthma. This was diagnosed a few years ago after an upper respiratory tract infection. His other past medical history is notable for eczema, allergic rhinitis, as well as severe nasal polyps which have required surgery in the past. Recently he has taken ibuprofen for rotator cuff tear which he found exacerbated his asthma. This was similar when he began to take paracetamol instead. What is the basis of his reaction with paracetamol?

A. Cross reactivity with ibuprofen
B. COX-1 inhibition
C. COX-2 inhibition
D. IgE mediated hypersensitivity reaction

Answer 15

Answer: B - COX-1 inhibition

Feedback
Patient as asthma exacerbated respiratory disease. Mechanism of this disease involves inhibition of COX-1 which inhibits prostaglandin E2. This normally inhibits mast cells from activating. Paracetamol at high doses can also inhibit COX-1 and can lead to AERD. Good review article https://www.nejm.org/doi/full/10.1056/NEJMra1712125

Question 16

A 35 year old women with a history of systemic lupus erythematosus and early pregnancy loss on hydroxychloroquine presents with atraumatic acute onset of left lower limb swelling and pain. Compressive venous doppler ultrasonogrophy reveals a DVT in the proximal popliteal vein. Her lupus anticoagulant testing reveals a strongly positive dilute russell viper venom test (drvvt), positive anti -beta 2 glycoprotein 1 and anti-cardiolipin antibody. She has no contra-indication to anticoagulation and has good medication adherence and understanding. She is not currently desiring fertility and has a progesterone IUD. Which of the following agents is most appropriate anticoagulation strategy?

A. Rivaroxaban
B. Enoxaparin alone
C. Warfarin with enoxaparin bridging, target INR of 2-3
D. Warfarin with enoxaparin bridging, target INR of 3-4

Answer 16

Answer: C - warfarin with enoxaparin bridging, target INR of 2-3

Feedback
“Important to appreciate the escalating risks associated with each of the anti-phospholipid antibodies (LA>B2GP1>aCL for risk of thrombosis). Triple positivity, as in this case, carries the highest risk of thrombosis. The TRAPS study published in Blood in 2018 showed in high risk APS patients with triple positivity rivaroxaban was inferior to warfarin, especially for arterial thrombosis. Therefore this lady should be anticoagulated with warfarin. Prior studies have shown a standard intensity anticoagulation in the first setting is appropriate in most cases. Both enoxaparin and fondaparinux are not ideal for long-term anticoagulation, however enoxaparin maybe used if she were to desire pregnancy.

Question 17

Mantoux test is an example of test based on

A. Type I hypersensitivity reaction
B. Type II hypersensitivity reaction
C. Type III hypersensitivity reaction
D. Type IV hypersensitivity reaction

Answer 17

Answer: D - type IV hypersensitivity reaction

Question 18

Which of the following cell type is most responsible for the interferon response to viral infections?

A. Plasmacytoid dendritic cells
B. Macrophages
C. B cells
D. T cells

Answer 18

Answer: A - plasmacytoid dendritic cells

Feedback
Answer: A
TLR 7 and TLR 9 are important in inducing type 1 IFN during a viral infection, particularly by plasmacytoid dendritic cells.

Question 19

All of the following are functions of C1 Inhibitor EXCEPT:

A. Inhibits conversion of Factor XI to FXIa
B. Inhibits conversion plasminogen to plasmin
C. Inhibits C3b binding to Factor B
D. Inhibits conversion of HMWK to Bradykinin by Kallikrein

Answer 19

Answer: Inhibits C3b binding to Factor B

Feedback
C1 Inhibitor stops - C1 inhibitor is a misnomer - clinically the most important role of C1INH is inhibiting the actions of activated FXII (activated by the contact activation pathway). These are: Coagulation, Fibrinolysis and bradykinin production (by inhibiting Kallekrein cleaving of High molecular weight kiminogen (HMWK) to Bradykinin).

Its roll in Complement pathway is overshadowed by the bradykinin/Angioedema effects.

Question 20

In assessing bone health in patients over 75, which of the following is FALSE?

A. BMD can be ordered at least once in all community dwelling men and women as a public health primary prevention screening measure due to the high prevalence of osteoporosis in this population
B. Calcium supplementation is more efficacious than dietary calcium
C. In treating osteoporosis, BMD is unnecessary where there is already evidence of a minimal trauma vertebral fracture on imaging
D. The FRAX osteoporosis risk calculator combines age, BMD and osteoporosis risk factors to calculate the 10 year probability of fracture

Answer 20

Answer: B - Calcium supplementation is more efficacious than dietary calcium

Feedback
Dietary calcium is just as efficacious with less side effects

Question 21

Regarding furosemide:

A. It is filtered by the glomerulus
B. It is secreted into the lumen in the proximal convoluted tubule via an organic acid transporter
C. It blocks sodium reabsorption by blocked the NaCl channel
D. It acts on the basolateral aspect of the thick ascending loop of Henle

Answer 21

Answer: B - It is secreted into the lumen in the proximal convoluted tubule via an organic acid transporter

Feedback
Frusemide is heavily bound to albumin and therefore cannot be filtered by the glomerulus. It is actively secreted by an organic acid transporter in the proximal convoluted tubule and competes for all other organic acids hence higher doses are required in renal failure when there is a build-up of these organic acids. It binds to the luminal aspect of the thick ascending loop of Henle and binds to the sodium potassium 2 chloride channel (Na-K-2Cl). Other reasons for requiring higher doses of loop diuretics ‘diuretic resistance’ is nephrotic syndrome where filtered albumin binds to secreted frusemide in the lumen and can’t block NKCl2 channel. Also low serum albumin reduces the available bound frusemide so here albumin infusion improves the diuresis if given together.

Question 22

A 40 year old male develops bilateral lower limbs swelling. Further investigations revealed serum albumin of 30, Cr of 90, HbA1C 6%. 24-hour urinary protein showed 3.8g/day. Renal biopsy shows thickened GBM without mesangial proliferation, granular subepithelial deposits of IgG and C3.

Which of the following is TRUE regarding this man’s disease at this time?

A. He should be commenced on perindopril but no immunosuppression
B. He should be commenced on warfarin and perindopril but no immunosuppression
C. He should be offered extended malignancy screening if Anti-Phospholipase A2 receptor Antibodies are present
D. He should be commenced on cyclosporine immunosuppression, in addition to medical management

Answer 22

Answer: B - B. He should be commenced on warfarin and perindopril but no immunosuppression

Feedback
This is likely primary membranous glomerulopathy. 5-20% are associated with malignancy, most commonly a solid tumour (prostate, lung, breast, bladder or GIT).

Presence of Anti-PLA2R reduces the likelihood of malignancy being present (more likely idiopathic).
Anti thrombospondin Type 1 domain-containing 7A (Anti THSD7A) are more likely associated with malignancy (~25%).

Treatment of MN depends on risk of progressive disease:

• High risk = Creatinine >133, progressive renal function decline, severe hypoalbuminaemia
• -> Immunosuppression (e.g. steroids & cyclophosphamide (or Rituximab)
• Low risk = normal kidney function, non-disabling nephrotic syndrome
• -> ACEi/ARB and monitor for 3-6 months for progression

This patient should have medical management of proteinuria - ACEI/ARB + statin. There are some general guides e.g. >3-6 months of >4g proteinuria despite ACEI/ARB.
Adding on empirical warfarin can be considered on a case by case basis without clear guidelines.

Question 23

Regarding commonly prescribed medications in dementia, which of the following describes the correct mechanism of action and assocciated adverse effect?

A. Donepezil is a cholinesterase inhibitor and is associated with tachycardia
B. Rivastigmine is a cholinesterase inhibitor and is associated with constipation
C. Memantine is a non-competitive NMDA antagonist and is associated with hypertension
D. Galantamine is a non-competitive NMDA antagonist and is associated with heart block

Answer 23

Answer: C - Memantine is a non-competitive NMDA antagonist and is associated with hypertension

Feedback
Donepezil, rivastigmine and galantamine are cholinesterase inhibitors. Their common adverse effects include diarrhoea and other GI complaints (but not constipation), insomnia, vivid dreams, bradycardia and conduction disease.

Memantine is a non-competitive NMDA antagonist. It’s major adverse effects include drowsiness, constipation, dizziness and hypertension.

Question 24

A 65 year old male with tophaceous gout is experiencing ongoing frequent gout exacerbations on a monthly basis affecting his toes, ankles and wrists. He has ceased alcohol use and is adherent to a low urate diet. His other medical conditions include hypertension, reflux, type 2 diabetes mellitus. Medications include metformin 1000mg daily, omeprazole 20mg daily, perindopril 5mg daily, aspirin 100mg daily, allopurinol 300mg daily, prednisolone 5mg daily.

Serum urate 0.42 (0.26 - 0.45 mmoL)
Urea 7.2 (3 - 8 mmoL)
Creatinine 75 (53 - 106 umoL)
HbA1c 6.5%

What is the best management strategy to improve his gout?

A. Add in probenacid and stop prednisolone
B. Increase allopurinol to 400mg daily, stop prednisolone and retest serum urate in one month
C. Increase allopurinol to 400mg daily, continue prednisolone for another 2 months and retest serum urate in one month
D. Switch allopurinol to febuxostat

Answer 24

Answer: C - Increase allopurinol to 400mg daily, continue prednisolone for another 2 months and retest serum urate in one month

Question 25

A 66 year old man presents with proximal muscle weakness and pain in all 4 limbs. Other relevant symptoms include Raynaud’s phenomenon, weight loss of 8kg in the past 3 months and progressive breathlessness. He has a history of ischaemic heart disease. His medications include aspirin, metoprolol and perindopril. He was previously on simvastatin which was stopped but did not improve his symptoms. He smokes 25 cigarettes per day on the background of a 55 pack year smoking history.

His blood tests are as follows:
Hb 115 MCV 85
WCC 6.0
Platelets 480

EUC normal
LFTs: Albumin 34, ALT 155 (<35), AST 200 (<38), GGT 75 (<35), ALP 150 (30-110)

Creatine kinase 9400 (53 - 106)

Which of the following autoantibodies is most likely to be positive?

A. Jo-1
B. Ro/SSA
C. Anti-topoisomerase
D. U1-RNP

Answer 25

Answer: A - Jo-1

Feedback
Clinical presentation suggestive of anti-synthetase syndrome, in which anti-Jo1 is the most commonly present autoantibody.

Question 26

Which of the following condition is most strongly associated with a positive ANCA against the myeloperoxidase antigen?

A. Behcet’s disease
B. Eosinophilic granulomatosis with polyangiitis
C. Granulomatosis with polyangiitis
D. Polyarteritis nodosa

Answer 26

Answer: B - Eosinophilic granulomatosis with polyangiitis

Feedback
P-ANCA - perinuclear pattern of staining (actually artefact of alcohol fixation)
- MPO usual antigen
- 70% active MPA, 10% active GPA
- Assoc. with haematuria and GN, low relapse rate
- High levels at presentation, fall with treatment, usually disappear and never recur
- Sometimes low levels in other autoimmune diseases, burnt out GN, ILD and pulmonary infection
- False positive with number of other proteins: lactoferrin, elastase, cathepsin G, catalase

EGPA is ANCA positive in 30-40% of cases, typically MPO - this does not correlate with disease activity.

GPA is typically c-ANCA/PR3 positive - 90% of active cases, correlates with ENT/URT disease.

Behcets and PAN are not ANCA associated vasculitides.

Question 27

Which of the following drug is not associated with an increased risk of osteoporosis?

A. Carbamazepine
B. Phenytoin
C. Heparin
D. Atorvastatin

Answer 27

Answer: D - Atorvastatin

Question 28

A patient with Sjogren’s syndrome presented to the Rheumatology clinic for his yearly review. His investigations revealed: Na 139, K 3, Cl 112, Bicarb 15. Urine studies showed pH 6, Na 15, K 10, Cl 12. The most likely cause for this abnormality is:

A. Type 1 RTA
B. Type 2 RTA
C. Type 3 RTA
D. Type 4 RTA

Answer 28

Answer: A - Type 1 RTA

Type 1 RTA = Distal tubule = Inability to excrete H+
pH >5.5 (no H+) – alkaline
Reabsorb citrate (makes Ca soluble) with renal stones
Causes = autoimmune - Sjogren’s syndrome, SLE, Primary biliary cirrhosis, autoimmune hepatitis

Type 2 RTA = proximal tubule = inability to excrete HCO3
pH < 5.5 (excess H+) – acidic
No renal stones
Fanconi syndrome: glycosuria, phosphaturia, uricaciduria, aminoaciduria
Causes Myeloma/MGUS, Drugs: tenofovir, acetazolamide

Look up here fore more: https://thecurbsiders.com/internal-medicine-podcast/104-renal-tubular-acidosis-kidney-boy-joel-topf-md

Question 29

Which of the following plays the most important role in lowering serum phosphate level?

A. Hypercalcaemia
B. FGF23
C. Parathyroid hormone
D. PHEX endopeptidase

Answer 29

Answer: B - FGF23

Question 30

A 40-year-old woman has recently commenced treatment for severe ankylosing spondylitis. She presents with plantar pustular lesions. Use of which of the following treatments is most likely to have precipitated this condition?

A. Sulphasalazine
B.Adalimumab
C. Methotrexate
D. NSAIDs

Answer 30

Answer: B - Adalimumab

Adalimumab is rarely associated with developing psoriasis including palmoplantar pustulosis.

Question 31

A 74-year-old woman has had increasingly difficult to manage idiopathic Parkinson’s Disease in the last five months, with worsening rigidity and bradykinesia. She has become now verbally aggressive towards nursing home staff and is grabbing at the air. Her current medication regimen consists of levodopa, entacapone, amantadine, lactulose and fludrocortisone. What is the next best management step after doing appropriate examination and investigations to exclude infection and constipation?

A. Start quetiapine
B. Start clozapine
C. Reduce levodopa dose
D. Reduce amantadine dose

Answer 31

Answer: D - reduce Amantadine dose

Feedback
Amantadine in Parkinson’s disease is used early to treat tremor or late to treat peak dose dyskinesia. It has significant anticholinergic side-effects including hallucinations and confusion. Given the lack of reported disabling tremor or peak-dose dyskinesia in this patient this would be the most appropriate medication to initially wean, as the dopaminergic drugs are likely providing the most benefit in terms of her worsening motor symptoms.

Question 32

Which of these features would you not expect in a transfusion related acute lung injury?

A. Hypoxemia
B. Low normal blood pressure
C. Low BNP
D. Improvement with diuretics

Answer 32

Answer: D - improvement with diuretics

TRALI = ARDS/acute hypoxic respiratory failure <6 hours post transfusion.
Diagnose by hypoxia and pulmonary infiltrates on CXR. Often associated fever, hypotension.
Immune driven therefore low BNP
Worsens/poor response with diuretics, improves with fluid

Extra: TACO
Hypoxia with signs of cardiac failure, pulmonary congestion, high BNP and abnormal heart function on echo. Improves with diuretics, worsens with fluid.

Question 33

In essential thrombocytosis, which of these mutations is associated with a milder disease course and better survival?

A. JAK-2
B. C-MPL
C. Calreticulin
D. Triple negative

Answer 33

Answer: C - Calreticulin

Feedback
The calreticulin mutation is present in 40% of essential thrombocytosis. It’s presence is associated with a milder disease course, fewer thrombotic episodes and better survival. They are more likely to present with a lower haemoglobin and WCC, but higher platelet count and are less likely to progress to myelofibrosis.

Question 34

Which of the following are FALSE?

A. Mepolizumab is effective in severe asthma and targets IL-5
B. Omalizumab binds Fcε Receptor 1 and is useful in severe asthma
C. Dupilumab has been shown to be effective in atopic dermatitis and asthma
D. Benralizumab is effective for severe asthma and targets IL-5 receptor

Answer 34

Answer: B False that: B. Omalizumab binds Fcε Receptor 1 and is useful in severe asthma

Question 35

A 79 year old gentleman presents to the emergency department with an altered conscious state. His ECG is shown below (Note: Shows J waves/Obsorn waves). Which of the following electrolyte abnormalities is most likely to be present?

A. Hypercalcemia
B. Hyperkalaemia
C. Hypokalaemia
D. Hypomagnesaemia

Answer 35

Answer: A - hypercalcaemia

Feedback
These are J waves or Osborn waves. Found in patients with significant hypothermia or hypercalcaemia.

The sharp uptake of the J waves can give the impression of a peaked T wave but it should be taller with hyperkalaemia. The presenting history of altered mental state should also guide you towards hypercalcaemia as this usually does not occur with isolated hyperkalaemia.

Question 36

Angiotensin receptor neprilysin inhibitors (ARNI) are a new therapeutic option in heart failure. Which of the following is INCORRECT regarding use of ARNI?

A. Compared with enalapril, sacubitril/valsartan reduced the risk of cardiovascular death or hospitalisation by 20%
B. ANRIs should not be commenced for at least 36 hours after discontinuing an ACE inhibitor
C. ANRI use should be avoided in patients who are on cardiac resynchronisation therapy
D. There is no role in monitoring levels of circulating BNP for patients on treatment with ANRIs

Answer 36

Answer; C -ANRI use should be avoided in patients who are on cardiac resynchronisation therapy

Feedback
These questions with double negatives are meant to be avoided in exams but unfortunately occasionally appear. In a long exam, it is easy to lose concentration and not read the question properly. Questions phrased like this need a bit of extra time and focus to avoid mistakes.

ANRI do reduce CVS mortality. Concurrent use with ACE should be avoided and if patient was on ACE therapy, it should be commenced at least 36hrs after discontinuing ACE inhibitor to minimise the risk of angioedema. It can be used in patients with CRT. While pro-ntBNP levels can give an indication of the degree of heart failure, circulating BNP will be elevated in patients on ANRI. See the link for further details: https://heart.bmj.com/content/heartjnl/102/17/1342.full.pdf

Question 37

A 23 year old man was playing soccer on the field. He was hit in the praecordium by a soccer ball. He collapsed to the ground and CPR was commenced. He was transferred to a tertiary hospital and as part of the subsequent workup underwent a cardiac MRI. Unfortunately, he failed to make significant neurologic recovery and died. Below are the cardiac MRI (upper image) and pathologic findings (lower image). What is the most likely cause of death?

Image shows right ventricle replaced with fibrofatty tissue.

A. Arrhythmogenic right ventricular dysplasia
B. Commotio cordis
C. Dilated cardiomyopathy
D. Hypertrophic obstructive cardiomyopathy

Answer 37

Answer: A - Arrhythmogenic right ventricular dysplasia

Feedback
While the history is suggestive of commotio cordis, a normal structural heart would be expected on MRI and pathology. There is clear abnormality of the right ventricle that has been replaced with fibrofatty tissue. Diagnosis is arrhythmogenic right ventricular dysplasia.

Question 38

Which of the following is the most sensitive test for diagnosing asthma?

A. Methocholine bronchoprovocation test
B. Mannitol bronchoprovocation test
C. Spirometry with bronchodilator reversibility
D. Daily peak expiratory flow rate

Answer 38

Answer: A - Methocholine bronchoprovocation test

Feedback
Methacholine is a direct bronchoprovocation test, which directly constricts airway smooth muscle via receptors on smooth muscle. It is the most sensitive test and has the best NPV for asthma.

Mannitol is an indirect bronchoprovocation test requiring mast cell activation to release histamine and other mediators. Hypertonic saline is an alternative indirect stimulus. This has a better PPV than methacholine - less sensitive but more specific.

Question 39

A patient has been diagnosed with cystic fibrosis. They have a homozygous mutation with a deletion of a phenylalanine residue at position 508 of the CFTR gene on chromosome 7. Which of the following therapeutic options would be most appropriate for this patient?

A. Ivacaftor monotherapy
B. Lumacaftor monotherapy
C. Tezacaftor monotherapy
D. Combination Ivacaftor and Lumacaftor

Answer 39

Answer: D - Combination Ivacaftor and Lumacaftor

Feedback
The question is describing a deltaF508 mutation. DELta for DELETION. F for phenylalanine. This is a Class 2 CFTR mutation.

Ivacaftor is a CFTR potentiator. Ivacaftor monotherapy is effective in CF with the G551D mutation where there is a Class 3 CFTR mutation (surface protein with abnormal function).

The deltaF508 mutation has an issue with trafficking of the CFTR to the cell surface and so requires both a corrector (lumacaftor) and a potentiator (ivacaftor). Tezacaftor is a newer agent for CF but similarly needs combination therapy with ivacaftor to be effective for the deltaF508 mutation.

Question 40

A 59 year old man presented with severe chest pain. There was concern regarding aortic dissection and so he was further investigated with CT angiogram of the chest. He has a background of rheumatic heart disease and had a mechanical mitral valve replacement at age 47 and is on warfarin for this. He is a previous smoker with a 30 pack year history. He is not on any other regular medications. The CT angiogram was negative and his chest pain resolved. It was thought to be musculoskeletal in nature. However, he was noted to have an 10mm nodule in his lung. There was no fat or calcification within the nodule and it was assessed by radiology as being at ‘intermediate’ risk for malignancy based on CT appearance. Which of the following is the LEAST appropriate follow up management?

A. Repeat CT chest in 3 months
B. Repeat CT chest in 12 months
C. FDG PET/CT
D. Percutaneous or endobronchial biopsy

Answer 40

Answer: B - repeat CT chest in 12 months

10mm generally considered large and this man is high risk (smoker etc) so would usually need repeat early follow-up scan or likely assess with PET scan +/- biopsy.

Fleischner guidelines = “consider CT at 3 months, PET/CT or tissue sampling”
https://pubs.rsna.org/doi/full/10.1148/radiol.2017161659

Question 41

Regarding obstructive sleep apnoea, which of the following is TRUE?

A. C-PAP therapy combined with weight loss significantly reduces high sensitivity CRP levels compared with either intervention alone
B. C-PAP therapy in addition with usual care, significantly reduces cardiovascular events in patients with moderate-severe obstructive sleep apnoea and established cardiovascular disease
C. Nocturnal oxygen therapy provides a significant reduction in blood pressure in patients with obstructive sleep apnoea
D. C-PAP therapy has been shown to increase insulin sensitivity in patients with obstructive sleep apnoea and diabetes mellitus

Answer 41

Answer: D - C-PAP therapy has been shown to increase insulin sensitivity in patients with obstructive sleep apnoea and diabetes mellitus

Question 42

Variants in which of the following genes increases susceptibility for Crohn’s disease?

A. PTPN22
B. CARD15/NOD2
C. STAT4
D. TLR7

Answer 42

Answer: B - CARD15/NOD2

Feedback
CARD15/NOD2 gene variants can increase susceptibility to Crohn’s by 20-fold. There were 2 nature articles published regarding this. Also it is in the RACP lectures.

Question 43

Which of the following antibiotics is LEAST likely to cause Clostridium Difficile colitis?

A. Amoxycillin
B. Doxycycline
C. Moxifloxacin
D. Trimethoprim

Answer 43

Answer: B - doxycyline

Feedback
https://www.nejm.org/doi/full/10.1056/nejmra1403772

Tetracyclines are the least likely agents to be associated with C Diff infection

Very common: Clindamycin, Ampicillin/Amoxicillin, Cephalosporins, Fluoroquinolones

Somewhat common: other penicillins, Sulfonamides, Trimethoprim/Bactrim, macrolides

Uncommon: Aminoglycosides, Metronidazole, Tetracyclines, Carbapenems, Daptomycin

Question 44

Regarding inflammatory bowel disease and pregnancy, which of the following is TRUE?

A. Patients with active disease at conception have a 20% chance of having continued or worsening symptoms during pregnancy
B. If a patient has had a flare of Crohn’s disease during a previous pregnancy, they are more likely to have a disease flare with the current pregnancy
C. Relapses of Crohn’s disease are most common during the third trimester of pregnancy
D. Infliximab is compatible with use during conception in women and throughout pregnancy

Answer 44

Answer: D - Infliximab is compatible with use during conception in women and throughout pregnancy

Feedback
Obstetric medicine questions can and will be added to the exam. Inflammatory bowel disease and pregnancy is an example of an important topic (other examples include anticoagulation in pregnancy, valvular heart disease in pregnancy, diabetes in pregnancy, hepatitis B in pregnancy etc.)

Based on current knowledge, infliximab appears safe in pregnancy.
Poor disease control can results in a flare during pregnancy in up to 70% of cases (in any case, much more than just 20%).
Flares of disease during previous pregnancies on the other hand, do not seem to predict flares in future pregnancies.
Relapse of Crohn’s is most likely to occur during first trimester.

Question 45

With regards to Hepatitis D virus (HDV), which of the following is TRUE?

A. Hepatitis D is a double stranded DNA virus
B. Hepatitis D is closely related to hepatitis C and co-infection increases the risk of progression to liver cirrhosis
C. Asymptomatic HDV carriers with persistently normal ALT levels do not require treatment but should be monitored for signs of active liver disease
D. Reinfection with HDV following liver transplantation is frequently associated with recurrence of liver disease

Answer 45

Answer: C - Asymptomatic HDV carriers with persistently normal ALT levels do not require treatment but should be monitored for signs of active liver disease

Feedback
Hepatitis D is a single stranded RNA virus.
It is closely related to hepatitis B not hepatitis C and requires co-infection with hepatitis B. Option C is correct in that asymptomatic HDV carriers with persistently normal ALT do not need active treatment.
While I do not expect any of you to be liver transplant specialists, with application of basic science, you should be able to deduce that option D is incorrect. As mentioned earlier, hepatitis D requires co-infection with hepatitis B. Therefore unless there is concurrent hepatitis B infection (unlikely as transplant livers will be screened for hepatitis B), re-infection post transplant with hepatitis D alone is unlikely to become a chronic issue.

Question 46

Which of the following antipsychotic is LEAST associated with the development of weight gain?

A. Clozapine
B. Haloperidol
C. Quetiapine
D. Risperidone

Answer 46

Answer: B - Haloperidol

Feedback
Typical antipsychotics such as haloperidol are LEAST associated with weight gain. Other examples of antipsychotics with lower risk of weight gain include lurasidone and ziprasidone. Olanzapine has the HIGHEST risk of weight gain.

Question 47

Review a hysteresis loop below. Which of the following explanations would best account for a drug with this loop?

Note: lines with Response vertical and plasma drug concentration horizontal axis.

Shows increased response initially with increased concentration then the response gradually plateaus and the concentration increases. Then the response decreases even as conc increases. Eventually loops back around so lower concentration and lower response

A. Time is required for the drug to be distributed to the site of action
B. Tachyphylaxis
C. The drug has decreased protein binding upon reaching its active site
D. There is the presence of competitive inhibitors in the serum

Answer 47

Answer: B - tachyphylaxis

Question 48

Which of the following psychiatric conditions is associated with the greatest risk of all-cause mortality?

A. Anorexia nervosa
B. Bipolar affective disorder
C. Depression
D. Schizophrenia

Answer 48

Answer: A - anorexia nervosa

Feedback
Eating disorders and substance misuse disorders have the highest mortality rates.

Question 49

Which of the following best describes the pathophysiology of Huntington’s Disease?

A. Expansion of a CTG trinucleotide repeat of DMPK gene on chromosome 19.
B. Expansion of CAG repeats in the HTT gene on chromosome 4
C. Unstable expansion of CGG repeat leading to loss of function mutation in FMR1 gene on X chromosome.
D. Expansion of CTG repeat encoding for ATXN1 on chromosome 1 leading to a toxic gain-of-function

Answer 49

Answer: B - Expansion of CAG repeats in the HTT gene on chromosome 4

Feedback
All correctly refer a separate triplet repeat disorder.
2 is the correct explanation of Huntington’s disease
1 describes myotonic dystrophy.
3 refers to fragile X.
4 is the error in spino-cerebellar ataxia.

Question 50

A 17 year old man presents to you with recurrent sinopulmonary infections. He requests referral to ENT surgeons for operative management. He has had 3-5 episodes of painful sinusitis per year since early adolescence and has missed a significant amount of school because of this. On further questioning, he has had three episodes of pneumonia in his life, one of which required brief admission to hospital. He has had no previous surgical intervention.On examination he is slim but developmentally normal. Tonsils and adenoids are noted to be absent.Flow cytometry: <1% CD19 or CD20 positive cells. CD3 positive cells ~65% Serum IgG 1.2g/L (6.4-14.3g/L) Serum IgA undetectable (0.7-3.0g/L) Serum IgM undetectable (0.2-1.4g/L) Serum IgE undetectable (<2000IU/mLTitres to pneumococcal and H. influenzae: undetectable

A. Cystic fibrosis
B. Common variable immunodeficiency
C. Bruton’s agammaglobulinaemia
D. Kartaagner’s syndrome

Answer 50

Answer: Bruton’s (X-linked) agammaglobulinaemia

Feedback
This is a fairly typical discription of Bruton’s agammaglobulinaemia, although the patient is being diagnosed a little later in life. The recurrent sinopulmonary infections are classic, as are the very low titres of IgM/IgG/IgA with undetectable B cells on flow cytometry. He has not mounted any antibody response despite likely exposure.

Cystic fibrosis is a consideration in recurrent sinopulmonary disease, but would not account for the test results. The same applies to Kartaganer’s.

CVID is the other best fit. In this you would expect to find low antibody levels, although IgM may be normal. One would expect to find normal or slightly low B cells on flow, not absent.

Question 51

A 25 year old man presents to you with unsteadiness and hearing loss over the last 3 months. He has otherwise been well. He was adopted at a young age due to the untimely death of his parents. He complains of intermittent but frequent episodes of vertigo. At times these are very prolonged and he feels his walking remains unsteady in between episodes. He has noted persistent hearing loss particularly in his right ear over the last year. On examination he is alert, normotensive and afebrile. Cranial nerve exam reveals sensorineural hearing loss right>left. There is subtle right facial droop and paraesthesia over the right side of the face with absence of the right corneal reflex. Visual examination is normal but you note posterior subcapsular lenticular opacities. Gait examination reveals mild ataxia. Peripheral motor and sensory exams are otherwise normal. What is the most likely cause of this man’s symptoms?

A. Tuberous sclerosis
B. Von Hippel Lindau syndrome
C. Neurofibromatosis type 1
D. Neurofibromatosis type 2

Answer 51

Answer: C - Neurofibromatosis type 2

Feedback
His presentation describes at least one acoustic neuroma. The sensorineural deafness and the involvement of CN V and VII on the right all suggest a fairly large tumour at the cerebello-pontine angle. (This is potential clinical exam fodder). The posterior subcapsular lenticular opacities are also typical of NF2, which is overall the best explanation for his presentation. MRI confirmation of bilateral acoustic neuromas would meet criteria for diagnosis.

Both versions of NF have autosomal dominant inheritance, family history was witheld here. NF1 is due to a mutation on chromosome 17 and NF2 on 22.

Question 52

Regarding the process of protein synthesis, which of the following is false?

A. Transcription proceeds from the 5’ region to the 3’ region.
B. The promoter region often lies upstream of the first exon.
C. Introns contain meaningless DNA sequences
D. Alteration of the nucleotide at the beginning of the codon is most likely to affect the protein product.

Answer 52

Answer: (False) C - Introns contain meaningless DNA sequences

Question 53

A 45 year old woman is admitted to ICU with a large intracerebral bleed. She is sedated and intubated due to low conscious state, and an extraventricular drain inserted to monitor intracerebral pressure. You are contacted by the after-hours Radiologist to inform you that on reviewing the images, there is evidence of extensive venous sinus thrombosis. What is the most appropriate next step in management?

A. Continue current management
B. Surgical evacuation of the haemorrhage
C. Commence heparin infusion
D. Repeat CTA immediately and commence aspirin if no active bleeding,

Answer 53

Answer: C - commence heparin infusion

Feedback
C is scary but true.

Question 54

A 33 year old woman presents to you with recurrent headaches over the two weeks. She has been generally well prior to this, and gave birth to her first child 1 month ago after an uncomplicated pregnancy. The headaches begin suddenly over seconds, and cause excruciating pain bilaterally. Your patient finds herself agitated and tearful during these episodes, which are associated with nausea, vomiting and photophobia. She has had three such attacks over the last two weeks, each lasting ~20 minutes. MRI demonstrates a small convexity subarachnoid haemorrhage. What is the most likely diagnosis?

A. Migraines
B. Cluster headaches
C. Reversible cerebral vasoconstriction syndrome
D. Leaking berry aneurysm

Answer 54

Answer: C - RCVS

Feedback
This is a typical presentation of RCVS. Further reading below:

Migraines would not account for the small amount of subarachnoid bloods.
This would be very unusual for a leaking aneurysm, which one would expect to be catastrophic event.
Cluster headaches are usually more unilateral, occur in clusters (i.e. nightly pain for weeks but months of reprieve in between) and may have autonomic features.

Question 55

A 52 year old man presents to you with sudden onset of horizontal diplopia. It developed at rest whilst watching TV and has persisted for the last 6 hours unchanged. It is associated with retro-orbital pain behind the right eye. He has a past medical history of type 2 diabetes on insulin, hypertension and hypercholesterolaemia. On examination he appears comfortable. BP is 150/90, HR is 90 and afebrile. His right eye is abducted and inferiorly depressed in primary gaze. He is unable to adduct the right eye, and unable to look superior or inferiorly. You note ptosis on the right side. The right pupil is 4mm compared to 3mm on the left, with a sluggish light response. The left eye examines normally. Cardiorespiratory examination is unremarkable. What is the most likely diagnosis?

A. Medial pontine infarct
B. Diabetic third nerve palsy
C. Posterior communicating artery aneurysm
D. Myaesthenia gravis

Answer 55

Answer: Posterior communicating artery aneurysm

Feedback
PainFUL third nerve palsy should always raise suspicion of a PCOM aneurysm, with or without the additional compelling finding of pupillary involvement. Remember that the pupillary fibres lies on the outside of the third nerve bundle, which makes them especially susceptible to compression but more commonly spared in ischaemia.

Question 56

You are visiting Thailand on a volunteer posting after a recent typhoon 7 days ago. A 35 year old man presents to you feeling generally unwell, with subjective rigors, headache, nausea and diarrhoea. He has also noticed slightly decreased urine output. The village where he lives was flooded in the recent heavy rainfall and other people have also been unwell. He appears generally unwell. You note conjunctival suffusion with scleral icterus bilaterally with no other signs of bleeding. BP is 120/60 and HR is 110. Hb 90 WCC 23 with left shift Platelets 90 Na 130 K 3.2 Creatinine 980 Urea 20 Bilirubin 70 ALP 456 (30-120U/L) GGT 110 (12-64U/L) ALT 523 (0-55U/L) AST 344 Albumin 29 (35-47g/L) INR 2.6 You admit him to the local HDU. During his stay there he develops respiratory distress with haemoptysis requiring FiO2 100 to maintain saturations of 95%. What is the underlying diagnosis?

A. Ebola
B. Hepatitis A
C. Leptospirosis
D. HIV

Answer 56

Answer: C - leptospirosis

Feedback
This question was really just to put tropical diseases (specifically leptospirosis) on your radar. Tropical diseases overall are rare in MCQs.
It’s clearly not Hep A.
Ebola would have more haemorrhagic features.
HIV itself would not cause a fulminant picture like this.

Question 57

A 40 year old man is admitted under the Haematology unit for an allogeneic bone marrow transplant which took place 40 days ago. His underlying condition is acute myeloid leukemia for which he received 7+3 induction (doxorubicin plus cytarabine) to achieve remission a month prior to admission. He received cyclophosphamide and total body irradiation conditioning and methotrexate for GVHD prophylaxis. He continues on cyclosporin. His medications also include pantoprazole 40mg BD and posaconazole 300mg daily. He developed severe haemorrhagic mucositis and hypokaelaemia following the transplant and requires ongoing analgesia and electrolyte replacement. At day 30 he developed high temperatures and became generally unwell. He commenced empiric Tazocin and gentamicin but has remained culture negative. Vancomycin was added on the fourth day and levels have been therapeutic. He continues to spike daily temperatures. Hb 89 WCC 0.01 Platelets 70 Na 140K 3.3Creatinine 120LFTs: normal. What is the most appropriate agent to add next?

A. Fluconazole
B. Meropenem
C. Anidulafungin
D. Linezolid

Answer 57

Answer: C - Anidulafulgin

Feedback
The persistent fevers despite broad spectrum antibiotics in someone so profoundly immunosuppressed raises concern for a fungal infection.
Anidulafungin is a better choice than an -azole in someone who has already been on prophylaxis.

Question 58

Which of the following is not a gram negative coccus?

A. Neisseria meningitidis
B. Enterococcus faecalis
C. Moraxella catarrhalis
D. Bordetella pertussis

Answer 58

Answer: B - enterococcus faecalis

Neiserria meningiditis = gram negative diplococci
Moraxella catarrhalis = gram negative diplococci
Bordetella Pertussis = gram negative coccobacillus
E. Coli = gram negative rod/bacillus

Listeria = Gram positive rod/bacillus

Question 59

Which of the following is an anaerobic gram positive bacillus? *

A. Listeria monocytogenes
B. Corynebacterium diptheriae
C. Bacillus cereus
D. Clostridioides difficile

Answer 59

Answer: D - clostrioides difficile

All of the options are gram positive bacilli. The clostridium species are all anaerobes.

Question 60

Which of the following is NOT a major criterion for the diagnosis of infective endocarditis according to the modified Duke’s criteria?

A. Two separate blood cultures for streptococcus gallolyticus
B. Four positive blood cultures for streptococcus epidermidis over 5 days
C. An IgG antibody titre >1:800 for Coxiella Burnetii
D. Septic emboli to brain with abscess formation

Answer 60

Answer: D - septic emboli to brain with abscess formation

Modified Duke criteria = 2 major criteria or 1 major and 3 minor
Possible IE if 1 major and 1 minor or 3 minor criteria

o Major criteria
 1. Blood cultures positive for IE
• ≥2 positive separate cultures for typical organism
• Single positive culture for Coxiella burnetti or IgG titre > 1:800
• 3 or more out of 4 positive blood cultures for microorganisms usually skin contaminants
 2. Imaging positive for IE
• Vegetation, abscess, valvular perforation or new dehiscence of prosthesis
• Abnormal activity at site of prosthetic valve on PET or CT

 Minor criteria: 1. Predisposing heart condition or IVDU, 2. Fevers, 3. Vascular phenomena (septic emboli, Janeway lesion), 4. Immunological phenomena (GN, Osler’s nodes, RF), 5. Micro not meeting major criterion

Question 61

Which of the following haemoglobin types contain alpha globin chains?

A. HbF
B. HbA
C. HbA2
D. All of the above contain alpha globin chains

Answer 61

Answer: D - All of the above contain alpha globin chains

HbF (foetal) = alpha2, gamma2
HbA (normal adult) = alpha2, beta2
Hb A2 (normal low level variant, increased in B-thalassemia) = alpha2, delta 2

Question 62

Which is false regarding sickle cell anaemia?

A. It is caused by a point mutation on the beta globin gene on chromosome 11 substituting valine for glutamic acid.
B. Hydroxyurea works by increasing HbF concentrations.
C. With the exception of HbS-β0-thalassemia compound heterozygotes are usually more severe than sole inheritance of sickle cell anaemia.
D. All patients with sickle cell disease should begin penicillin prophylaxis within the first three months of life and continue until at least age 5.

Answer 62

Answer: C - With the exception of HbS-β0-thalassemia compound heterozygotes are usually more severe than sole inheritance of sickle cell anaemia.

Feedback
A is true.
B is true.
D is true, patients with sickle cell anaemia often become functionally asplenic in childhood and benefit from penicillin prophylaxis.

C is false. Essentially pts with beta thalassemia will have a higher proportion of normal alpha-globin chains than pts with sickle cell anaemia, thereby reducing the proportion of abnormal beta chains.

Question 63

A 35 year old man with known sickle cell anaemia presents with acute onset of chest pain, respiratory distress and wheeze. He has had similar presentation in the past and is on long term hydroxyurea therapy 2g daily. On examination respiratory rate is 36, oxygen saturations are 85% on room air improving to 95% on 6L by CIG, BP is 130/90, and HR is 115, temperature 37.8. He is in obvious pain. There is a diffuse wheeze on examination of the chest. ECG shows sinus tachycardia with no ischaemic features. Hb 105. WCC 10. Platelets 200. EUC - unremarkable. CXR is shown below (diffuse patchy opacities). In addition to supplemental oxygen and analgesia, what is the next step in management?

A. Blood transfusion
B. Exchange transfusion
C. IV hydrocortisone 100mg
D. Therapeutic enoxaparin

Answer 63

Answer: B - Exchange transfusion

Feedback
Sickle cell chest crisis - severe. This man needs an exchange transfusion. Giving a transfusion alone will increase his haematocrit, which will predispose further to vessel occlusion. if he were very anaemic this might be reasonable.

Steroids are not standard practice.
He should receive VTE prophylaxis but does not need anticoagulation. increase his haematocrit, which will predispose further to vessel occlusion. if he were very anaemic this might be reasonable.

UptoDate:
The need for transfusion and the modality of transfusion (simple versus exchange transfusion) depend upon the severity and the rate of progression of the ACS episode [3,20]. Mild episodes require no transfusion, moderate episodes require simple or exchange transfusion, and severe episodes require exchange transfusion.
There are advantages to exchange transfusion compared with simple transfusion that make exchange transfusion the preferred method in severely affected adults. Exchange transfusion performed by automated erythrocytapheresis allows for the rapid transfusion of large amounts of blood (eg, 6 to 8 units of packed red blood cells for a typical adult), effectively decreasing hemoglobin S percentage while avoiding the hyperviscosity that may occur when hemoglobin levels are raised above 11 g/dL [56].

Question 64

Which of the following is not a potential benefit of splenectomy in the management of a patient with beta thalassemia major?

A. Decreased transfusion requirement
B. Improvement in cytopenias
C. Reduced iron overload
D. Decreased risk of thromboembolism

Answer 64

Answer: D - Decreased risk of thromboembolism

Feedback
This is an obscure question. Splenectomy may be done in haemoglobinopathies to reduce transfusion requirement, alleviate mass effect or in the event of splenic infarction.

There is an increased risk of VTE following this.

Question 65

Which of the following exerts its action by direct inhibition of thrombin?

A. Bivalirudin
B. Apixaban
C. Fondaparinux
D. Danaparoid

Answer 65

Answer: A - Bivalirudin

Feedback
Factor Xa inhibitors: rivaroXaban, apiXaban, fondaparinuX

Anti-thrombin III potentiators: LMWH and danaparoid

Anti-thrombin III potentiator with direct effect on thrombin: heparin

Direct thrombin inhibitors: dabigatran, bivalirudin

Question 66

Regarding the process of cellular replication, which of the following is true?

A. Most cells spend a majority of their life in metaphase.
B. Each somatic cell contains 23 autosomal pairs.
C. Chromosome non-disjunction leads to aneuploidy.
D. Taxane and vinca alkaloid chemotherapeutics are active in telophase.

Answer 66

Answer: C - Chromosome non-disjunction leads to aneuploidy.

Feedback
Most cells spend their life in interphase.
Each somatic cell has 22 autosomal pairs and one allosomal pair (sex chromosome)
Chromosomal non-dysjunction leads to aneuploidy (the correct answer). This is a common mechanism of Downs syndrome.
Taxanes and vinca alkaloids are most active in metaphase, as they interfere with microtubule function.

Question 67

EMQ
Multiple pharmacogenomic markers have been identified which can predict efficacy or adverse effects from specific medications. Please identify the corresponding marker with the condition listed.

A. CYP2C9*3
B. CYP2D6
C. CYP2C19
D. CYP3A5
E. HLA-B*1502
F. HLA-B*5701
G. HLA-B*5801
H. TPMT

1. Abacavir hypersensitivity
2. Steven Johnson syndrome and carbamazepine
3. Drug induced liver injury and Flucloxacillin

Answer 67

Answers:

1. E - HLAB*5701 = abacavir hypersensitivity
2. HLAB*15:02 = SJS and carbamazepine
3. HLA-B*57:01 = Drug induced liver injury and flucloxacillin

NB: HLA- B*58:01 = Allopurinol hypersensitivity in Han Chinese
TPMT for allopurinol metabolism
CYP2D6 metabolises codeine, fluoxetine/paroxetine, Tamoxifen