Glycine therapy in isovaleric acidemia.
Publication/Presentation Date
5-1-1978
Abstract
The therapeutic efficacy of oral glycine was tested in a 3-year-old girl with isovaleric acidemia. An oral leucine load (25 mg/kg) caused a rise of the blood levels of isovaleric, lactic, and pyruvic acids as well as an increase of urinary excretion of the ketone bodies. These changes did not occur when oral glycine (250 mg/kg) was given with the leucine. Glycine supplementation favored the formation of isovalerylglycine, a nontoxic conjugate of isovaleric acid which is excreted rapidly. Excretion of isovalerylglycine rose threefold when leucine and glycine were administered simultaneously. Chronic glycine therapy was tolerated well and may have prevented one episode of ketoacidosis.
Volume
92
Issue
5
First Page
813
Last Page
817
ISSN
0022-3476
Published In/Presented At
Yudkoff, M., Cohn, R. M., Puschak, R., Rothman, R., & Segal, S. (1978). Glycine therapy in isovaleric acidemia. The Journal of pediatrics, 92(5), 813–817. https://doi.org/10.1016/s0022-3476(78)80164-4
Disciplines
Medicine and Health Sciences | Pediatrics
PubMedID
641635
Department(s)
Department of Medicine
Document Type
Article