A newborn with acute encephalopathy is found to have hypoglycemia and ketonuria.Plasma leucine, isoleucine, and valine levels are markedly elevated, but the plasma ammonia level is normal.This is most likely a case of:
A) Ornithine transcarbamoylase deficiency.
B) Long-chain acyl-coenzyme A (CoA) dehydrogenase deficiency.
C) Methylmalonic acidemia.
D) Vitamin B12 deficiency.
E) Maple syrup urine disease.
Correct Answer:
Verified
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