Definition of Branched chain ketoaciduria

1. 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.




Medical Definition of Branched chain ketoaciduria

1. Hereditary disease due to deficiency of an enzyme involved in amino acid metabolism, characterised by urine that smells like maple syrup. (12 Dec 1998)

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Lexicographical Neighbors of Branched Chain Ketoaciduria

branch register
branch registers
branch stacking
branch tee
branch to atrioventricular node
branch to sinuatrial node
branch to trigeminal ganglion
branch water
branched
branched-chain
branched-chain fatty-acid-kinase
branched-chain fatty acid synthetase
branched calculus
branched chain
branched chain acyl-CoA oxidase
branched chain ketoaciduria (current term)
branched chain ketonuria
brancher
brancher deficiency glycogenosis
brancher glycogen storage disease
brancheries
branchers
branchery
branches
branches of government
branches of segmental bronchi
branchia
branchiae
branchial
branchial apparatus

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