pediatrics
Branched Chain Amino Acid Metabolism Disorders
Introduction
Valine, leucine, and isoleucine are branched-chain amino acids; deficiency of enzymes involved in their metabolism leads to accumulation of organic acids with severe Metabolic Acidosis . There are numerous disorders of branched-chain amino acid metabolism (see the Branched-Chain Amino Acid* Metabolism Disorders ) as well as many other 