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Isovalericacidemia Appearing as Diabetic Ketoacidosis

KATHLEEN M. WILLIAMS, MD; VIRGINIA H. PEDEN, MD; RICHARD E. HILLMAN, MD
Am J Dis Child. 1981;135(11):1068-1069. doi:10.1001/archpedi.1981.02130350068023.
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Isovalericacidemia (IVA) is a rare metabolic disorder of leucine metabolism characterized by recurrent bouts of vomiting associated with dehydration, acidosis, ketonuria, coma, and a peculiar "sweaty feet" body odor. The course may be rapidly progressive, with death in early infancy, or may be prolonged, with evidence of mental retardation if untreated.

Laboratory features that may be associated with IVA include neutropenia, thrombocytopenia, hypocalcemia, hyperammonemia, and hyperglycinemia. To our knowledge, hyperglycemia severe enough to simulate diabetic ketoacidosis has not been reported.

Report of a Case.—A boy was born at term to a 13-year-old girl, who put him up for adoption. No family history is known.

Recurrent vomiting began at 3 weeks of age. Pyloric stenosis was diagnosed and a pyloromyotomy was done. Transient ketonuria was noted at that time.

His subsequent course was marked by intermittent episodes of vomiting brought on by infections or excessive protein ingestion. A "funny odor"

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