Baby with macrocephaly and severe intrauterine growth retardation:

Questions 45

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Pediatric Genetic Questions

Question 1 of 5

Baby with macrocephaly and severe intrauterine growth retardation:

Correct Answer: B

Rationale: Digynic (B, maternal triploidy) fits. Rationale: 2 maternal + 1 paternal sets (69, XXX/XXY) cause small fetus, macrocephaly; diandric has large placenta.

Question 2 of 5

You are asked to consult about a 2-month-old girl with hypotonia, seizures, and an elevated plasma lactate (8 mM/L, normal deficiency. Which of the following is the most likely mode of inheritance in this infant?

Correct Answer: C

Rationale: Same as Q10; autosomal recessive (C) for PDH deficiency. Rationale: Recessive inheritance fits sporadic female cases; mitochondrial (maternal) or X-linked (male bias) are less likely without family history or sex skew.

Question 3 of 5

Initial therapy for the girl described in Question 4 should include all of the following EXCEPT

Correct Answer: D

Rationale: Nonspecific vaginitis treatment focuses on hygiene (A), sitz baths (B), mild soaps (C), Metronidazole (D). is unnecessary. Rationale: Metronidazole treats bacterial vaginosis (e.g., Gardnerella), not nonspecific irritation; hygiene measures address the cause.

Question 4 of 5

The next diagnostic test for the patient in Question 11 is

Correct Answer: C

Rationale: Bitemporal hemianopsia and hyperprolactinemia indicate a pituitary lesion; head CT (C) or MRI confirms this. Rationale: Head imaging detects prolactinomas; abdominal/pelvic scans (A, B) and biopsy (D) are irrelevant to pituitary pathology.

Question 5 of 5

All the following are true in autosomal dominant disorder EXCEPT

Correct Answer: D

Rationale: Autosomal dominant traits affect autosomes (A), require one mutant allele (B), have 50% inheritance (C), and allow male-to-male transmission (D). Male:female ratio is ~1:1, not 2:1 Rationale: Sex bias suggests X-linked, not AD.

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