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Pediatrics · Systemic paediatrics: primary ciliary dyskinesia
Sample itemA child with chronic wet cough since infancy, recurrent otitis media and bronchiectasis has dextrocardia on the chest X-ray. What is the diagnosis?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
A. Kartagener syndrome (primary ciliary dyskinesia)
Kartagener syndrome is the triad of situs inversus, chronic sinusitis and bronchiectasis from defective ciliary motility (dynein arm defects). Males are often infertile.
Option by option
A. Kartagener syndrome (primary ciliary dyskinesia) (correct)
Correct. Situs inversus with bronchiectasis fits.
B. Foreign body aspiration
A foreign body does not cause situs inversus.
C. Cystic fibrosis
CF causes pancreatic insufficiency and does not cause situs inversus.
D. Alpha-1 antitrypsin deficiency
Alpha-1 antitrypsin deficiency causes emphysema and liver disease.
Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.
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