🏥 USMLE Exam Prep

A newborn screening reveals elevated phenylalanine levels. Subsequent testing confirms a deficiency in phenylalanine hydroxylase. Which of the following is the most appropriate long-term dietary intervention for this infant?

A High-protein diet with tyrosine supplementation.
B Phenylalanine-restricted diet with tyrosine supplementation.
C Tyrosine-restricted diet with phenylalanine supplementation.
D High-fat, low-carbohydrate diet.

✓ Correct Answer: Option B

Phenylketonuria (PKU) results from phenylalanine hydroxylase deficiency, preventing phenylalanine conversion to tyrosine. A phenylalanine-restricted diet supplemented with tyrosine is crucial to prevent intellectual disability and other neurological issues.

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