Branched-Chain Amino Acids, Quantitative (Plasma)
This test is used to monitor the treatment of patients with maple syrup urine disease (MSUD), an autosomal recessive disorder of branched-chain amino acid metabolism. For new patients presenting with symptoms suggestive of MSUD (neurologic decompensation accompanied by metabolic acidosis and ketosis), please order quantitative amino acids (plasma) and urine organic acids.
