Neonatal Isovaleric Acidemia Associated with Hyperammonemia
Isovaleric acidemia (IVA) is an inborn error of leucine metabolism due to a congenital deficiency of isovaleryl-CoA dehydrogenase (Rhead and Tanaka, 1980). Association of significant hyperammonemia with the disease has not been previously described. The present paper reports on a neonate with IVA who developed hyperammonemia. This observation may indicate that secondary derangement of ammonia metabolism can take place in IVA and that IVA should be included in the differential diagnosis of hyperammonemia in the neonatal period.
KeywordsOrotic Acid Calcium Gluconate Maple Syrup Urine Disease Body Odour Isovaleric Acid
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