Abstract
We have used glycine for the acute and chronic management of two newborn infants with isovaleric acidemia. Both infants presented with deep coma and serum isovaleric acid levels in excess of 50 mg/dl after several days of treatment with parenteral fluids and protein restriction. After administration of intravenous glycine (250 mg/kg/day) the serum isovaleric acid level declined to less than 1 mg/dl within 3 days. Restoration of normal clinical status occurred within 6 days. Unexpectedly, the sharp decline of the serum isovaleric acid level was not accompanied by an equally sharp rise of urinary isovaleryl-glycine excretion. This suggests that in neonates isovaleryl-glycine may be excreted by the GI tract or that the glycine effect is mediated through a mechanism other than the synthesis of isovalerylglycine. The infants have been treated prophyl-actically with a low protein diet and oral glycine for 3 and 8 months, respectively. Their psychomotor development has been entirely normal. Neither has suffered a relapse even though the older infant has had several intercurrent infections. Glycine treatment in neonates appears to have aided in the management of the acute toxicity of isovaleric acidemia and may have prevented the reaccumulation of isovaleric acid.
Article PDF
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Yudkoff, M., Cohn, R., Blazer-Yost, B. et al. 575 GLYCINE THERAPY FOR THE ACUTE AND CHRONIC MANAGEMENT OF NEONATES WITH ISOVALERIC ACIDEMIA. Pediatr Res 12 (Suppl 4), 459 (1978). https://doi.org/10.1203/00006450-197804001-00580
Issue Date:
DOI: https://doi.org/10.1203/00006450-197804001-00580