Pathology

Genetic and Pediatric Diseases Practice Questions

20 free Genetic and Pediatric Diseases practice questions for the USMLE Step 1, each with the correct answer and a detailed explanation. Open any question below, or take the full set as an interactive quiz.

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All Genetic and Pediatric Diseases questions

20 questions
  1. Q1. Which of the following is the genetic basis of Down syndrome (most common form)?
  2. Q2. Which of these is a classic clinical feature commonly seen in Down syndrome patients?
  3. Q3. Cystic fibrosis (CF) is inherited by which pattern, and what is the primary defect in CF?
  4. Q4. Which organ systems are most characteristically affected in cystic fibrosis, leading to chronic clinical problems?
  5. Q5. Sickle cell disease (SCD) is caused by a mutation in which gene product, and what is the primary pathologic change in red blood cells?
  6. Q6. Which of the following genetic disorders often leads to progressive neurodegeneration in infancy or early childhood due to enzyme deficiency in the central ner…
  7. Q7. Which syndrome is caused by the complete or partial loss of one X chromosome in a genetically female individual and often results in short stature, infertility…
  8. Q8. Which of the following best describes the inheritance pattern of most classical inborn errors like cystic fibrosis, sickle cell disease, and Tay–Sachs disease?
  9. Q9. Marfan syndrome commonly results from mutations in the FBN1 gene. Which tissue abnormality primarily accounts for its characteristic aortic aneurysm risk?
  10. Q10. Which of the following genetic diseases arises from genomic imprinting or uniparental disomy rather than simple gene mutation or chromosome number change?
  11. Q11. Which pediatric genetic condition is most likely when a newborn has exocrine pancreatic insufficiency plus bone marrow dysfunction and short stature (but not c…
  12. Q12. Which of the following statements about chromosomal disorders is TRUE?
  13. Q13. In a pedigree showing an X-linked recessive disease such as Duchenne muscular dystrophy, which parent transmits the defective gene to an affected male child?
  14. Q14. Which metabolic pediatric disease results from inability to degrade an amino acid, leading to intellectual disability unless detected by newborn screening and…
  15. Q15. Which genetic condition tends to have more severe expression of disease in males because of having only one X chromosome?
  16. Q16. A newborn male baby presents with micrognathia, downward slanting palpebral fissures, malformed ears and cleft palate, but normal intelligence. Which disorder…
  17. Q17. Which of the following is a common complication in children with Down syndrome that warrants early cardiac evaluation?
  18. Q18. Which feature differentiates a chromosomal disorder from a single-gene disorder in genetic disease classification?
  19. Q19. Which of the following syndromes is an example of a non-Mendelian, imprinting or uniparental disomy disorder rather than classic autosomal or chromosomal abnor…
  20. Q20. Which pediatric genetic disease is characterized by progressive destruction of motor neurons leading to muscle weakness, often inherited in an X-linked pattern?