branched chain ketoaciduria

Definitions 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
   synonyms: maple syrup urine disease

   type of:

   congenital disease, genetic abnormality, genetic defect, genetic disease, genetic disorder, hereditary condition, hereditary disease, inherited disease, inherited disorder

   a disease or disorder that is inherited genetically

   acetonuria, ketoaciduria, ketonuria

   excessive amounts of ketone bodies in the urine as in diabetes mellitus or starvation