branched chain ketoaciduria

1 definition found

branched chain ketoaciduria - WordNet (r) 2.1 (2005) :

  branched chain ketoaciduria
      n 1: 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
           [syn: maple syrup urine disease, branched chain ketoaciduria
           ]