Nokea
A child aged 10 days has ambiguous genitalia:
- A. If a buccal smear is chromatin negative, there is a serious risk of an Addisonian crisis
- B. A raised urinary output of pregnanetriol would confirm a diagnosis of CAR
- C. The finding of the genotype 45 XO would reliably explain the anomaly
- D. If testicles were present in the 'labia', an acceptable explanation would be Klinefelter's syndrome
Correct Answer: B
Rationale: A raised urinary pregnanetriol indicates congenital adrenal hyperplasia (CAR). Chromatin-negative buccal smear suggests male genotype, and 45 XO is Turner syndrome, not related to ambiguous genitalia. Klinefelter's syndrome does not explain testicles in the labia.
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Surgical repair of VSD should be considered at the time of diagnosis
- A. small supracristal VSD
- B. VSD with heart failure
- C. hemodynamically restrictive VSD
- D. significant VSD
Correct Answer: B
Rationale: Significant or symptomatic VSDs warrant early surgical intervention.
Which among the following is not a feature of low flow - low gradient severe aortic stenosis with preserved left ventricular function?
- A. Mean trans aortic pressure gradient < 40 mm Hg
- B. Aortic valve area < 1 cm²
- C. Stroke volume > 35 ml/m²
- D. Left ventricular ejection fraction > 50%
Correct Answer: C
Rationale: Stroke volume is typically reduced, not increased, in low flow - low gradient severe aortic stenosis.
In congenital hypertrophic pyloric stenosis:
- A. Conjugated hyperbilirubinaemia may be seen
- B. Hypokalaemia is due to vomiting
- C. Blood pH is high and urinary pH is low
- D. First-born males are commonly affected
Correct Answer: B
Rationale: Hypokalaemia in congenital hypertrophic pyloric stenosis is typically due to the loss of potassium through vomiting.
How much bilirubin can be bound to 1 gm of albumin?
- A. 8.5 mg
- B. 9.8 mg
- C. 10.5 mg
- D. 13.6 mg
Correct Answer: A
Rationale: 1 gram of albumin can bind approximately 8.5 mg of bilirubin, which is important in managing neonatal jaundice.
List THREE medical management plans for biatrial enlargement.
- A. Loop diuretics (carefully)
- B. Beta-blockers (carefully)
- C. Anticoagulation
- D. All of the above
Correct Answer: D
Rationale: These are standard treatment options aimed at managing heart function, reducing fluid buildup, and preventing complications like clot formation.