A deficiency in one of the critical enzymes involved in the catabolism of the branched-chain amino acids results in neonatal vomiting, lethargy, and poor suckling behavior. Progressive neurological signs include decerebrate posturing. Which one of the following disorders corresponds to this defect?
A) alkaptonuria
B) homocystinuria
C) isovaleric acidemia
D) maple syrup urine disease
E) phenylketonuria (PKU)
Correct Answer:
Verified
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