maple syrup urine disease

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• noun branched-chain ketoaciduria, an autosomal recessive metabolic disorder affecting branched-chain amino acids and causing the urine to have a distinctive sweet odour

from WordNet 3.0 Copyright 2006 by Princeton University. All rights reserved.

• noun an inherited disorder of metabolism in which the urine has a odor characteristic of maple syrup; if untreated it can lead to mental retardation and death in early childhood