maple syrup urine disease(redirected from Ketoacidemia)
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Related to Ketoacidemia: ketoaciduria
maple syrup urine disease[′mā·pəl ¦sir·əp ′yu̇r·ən di‚zēz]
A hereditary metabolic disorder caused by deficiency of branched-chain keto acid decarboxylase; characterized by the maple-syrup-like odor of urine. Also known as branched-chain ketoaciduria.
McGraw-Hill Dictionary of Scientific & Technical Terms, 6E, Copyright © 2003 by The McGraw-Hill Companies, Inc.