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Table of Contents > Health Dictionary > Isovaleric acidemia

Isovaleric acidemia

An inborn error of leucine metabolism characterized by psychomotor retardation, a specific odor reminiscent of sweaty feet, vomiting, acidosis, and coma; associated with excessive production of isovaleric acid upon protein ingestion or during infectious episodes; due to a deficiency of isovaleryl-CoA dehydrogenase; severe metabolic acidosis results from the large quantities of acid formed. Autosomal recessive inheritance; two forms are known: the acute neonatal form with fulminant metabolic acidosis and rapid death and the chronic form characterized by intermittent episodes of severe ketoacidosis.
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