isovaleric acidemia (medicine)

isovaleric acidemia

A disorder of leucine metabolism characterised by the excessive production of isovaleric acid upon protein ingestion or during infectious episodes; severe metabolic acidosis results from the large quantities of acid formed; autosomal recessive inheritance; due to a deficiency of isovaleryl-CoA dehydrogenase.

Synonym: sweaty feet syndrome.

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isovaleric acidemia (medicine)

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