isovalericacidemia /iso·va·ler·ic·ac·i·de·mia/ (i″so-vah-ler″ik-as″ĭ-de´me-ah) an aminoacidopathy due to a defect in the pathway of leucine catabolism, characterized by elevated levels of isovaleric acid in the plasma and urine, causing a characteristic odor of sweaty feet, severe acidosis and ketosis, lethargy, convulsions, pernicious vomiting, psychomotor retardation, and in severe cases coma and death.
i·so·va·le·ric·ac·i·de·mi·a (  s -v -lîr -k s-d  m-, -l r-)n. An inherited disorder of leucine metabolism marked by excessive production of isovaleric acid upon protein ingestion or during infectious episodes and resulting in severe metabolic acidosis. |
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