Genetic and Pediatric Diseases · USMLE Step 1

Cystic fibrosis (CF) is inherited by which pattern, and what is the primary defect in CF?

  1. Autosomal dominant; defective collagen synthesis
  2. Autosomal recessive; defective CFTR chloride channel
  3. X-linked recessive; absent dystrophin
  4. Mitochondrial inheritance; defective oxidative phosphorylation
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Correct answer: Autosomal recessive; defective CFTR chloride channel

CF is an autosomal recessive disease caused by mutations in the CFTR gene, leading to defective chloride ion channels; this results in thick, sticky mucus production, especially affecting lungs and pancreas.

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