The plasma amino acids in the patient in the previous two questions reveal a marked elevation of leucine, isoleucine, and valine. The most likely diagnosis is

Correct Answer: A

Rationale: Maple syrup urine disease (MSUD) causes elevated branched-chain amino acids (leucine, isoleucine, valine).

Correct Answer: B

Rationale: Albumin infusion is not standard for urea cycle defect crises; others help remove ammonia or provide energy.

Correct Answer: B

Rationale: Zellweger syndrome involves peroxisomal import defects.

Correct Answer: C

Rationale: Elevated VLCFAs confirm X-linked adrenoleukodystrophy.

Correct Answer: B

Rationale: Niemann-Pick primarily causes hepatosplenomegaly.