Branched-Chain Amino Acid-Free Parenteral Nutrition in the Treatment of Acute Metabolic Decompensation in Patients with Maple Syrup Urine Disease
Year: 1991
Type: article
Abstract: MAPLE syrup urine disease is a rare inborn error of branched-chain amino acid and branched-chain keto acid metabolism due to decreased branched-chain α-keto acid dehydrogenase-complex activity.1 The branched-chain keto acids, which are the substrates in this irreversible enzyme reaction, are derived from transamination of the branched-chain amino acids leucine, isoleucine, and valine. Consumption of branched-chain amino acids in the form of dietary protein in excess of an age-dependent daily requirement for protein synthesis results in the accumulation of branched-chain amino acids and branched-chain keto acids in the tissues and body fluids of patients in whom there is little or no . . .
Source: New England Journal of Medicine
Authors: Gerard T. Berry, Randall Heidenreich, Paige Kaplan, Frederick Levine, Alice Mazur, Michael J. Palmieri, Marc Yudkoff, Stanton Segal
Institution: Children's Hospital of Philadelphia
Language: English
Subfield: Clinical Biochemistry
Domain: Life Sciences
Open Access status: bronze
Award: G3442710165
OpenAlex ID: W2074551630