Genetic and Pediatric Diseases · USMLE Step 1

Which of the following genetic diseases arises from genomic imprinting or uniparental disomy rather than simple gene mutation or chromosome number change?

  1. Down syndrome
  2. Prader–Willi syndrome
  3. Sickle cell disease
  4. Cystic fibrosis
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Correct answer: Prader–Willi syndrome

Prader–Willi syndrome results from absence of active paternal genes on chromosome 15 (due to paternal deletion or maternal uniparental disomy), an imprinting disorder — unlike typical single-gene or chromosomal disorders.

Difficulty: Medium Question 10 of 20

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