Nokea
Recognized features in Ehlers-Danlos syndrome include:
- A. Mutation in fibrillin gene
- B. Short stature
- C. Keratoconus
- D. Easy scarring
Correct Answer: D
Rationale: Easy scarring: Ehlers-Danlos syndrome is a connective tissue disorder characterized by hyperelastic skin, joint hypermobility, and easy bruising and scarring.
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Clinical manifestations of hypoplastic left-heart syndrome in the 1st 48 hr of life include
- A. always obvious color
- B. deep-blue skin
- C. bounding peripheral pulses
- D. occasional association with Turner syndrome
Correct Answer: C
Rationale: Bounding peripheral pulses are not typical in hypoplastic left-heart syndrome.
The following drugs may cause long Q-T syndromes (LQTS) EXCEPT
- A. trimethoprim/sulfamethoxazole
- B. erythromycin
- C. imipramine
- D. pentostam
Correct Answer: D
Rationale: Pentostam is not known to cause long Q-T syndromes.
These causes often cause secretory diarrhoea except:
- A. Lactose intolerance
- B. E. coli infection
- C. Congenital chloridorrhoea
- D. Blind loop syndrome
Correct Answer: A
Rationale: The correct answer is A because lactose intolerance causes osmotic diarrhoea, not secretory diarrhoea. The other options (b-e) are causes of secretory diarrhoea.
Which statement by a parent of an infant with congestive heart failure (CHF) who is being sent home on digoxin indicates the need for further education?
- A. I will give the medication at regular 12-hour intervals.
- B. If he vomits, I will not give a make-up dose.
- C. If I miss a dose, I will not give an extra dose.
- D. I will mix the digoxin in some formula to make it taste better.
Correct Answer: D
Rationale: Mixing digoxin with formula can lead to incomplete ingestion and inadequate dosing. Digoxin must be given in its entirety at the prescribed times.
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.