title: A variant form of branched-chain keto aciduria.
mesh_terms: D000596:Amino Acids / Q000097:blood / Q000652:urine; D002653:Child Behavior Disorders / Q000209:etiology; D002675:Child, Preschool; D002869:Chromosome Aberrations; D025063:Chromosome Disorders; D004035:Diet Therapy; D004044:Dietary Proteins / Q000627:therapeutic use; D005260:Female; D006579:Heterozygote; D006801:Humans; D007223:Infant; D008607:Intellectual Disability; D007532:Isoleucine / Q000097:blood / Q000627:therapeutic use; D007651:Keto Acids / Q000097:blood / Q000652:urine; D007930:Leucine / Q000097:blood / Q000627:therapeutic use; D008297:Male; D008375:Maple Syrup Urine Disease / Q000175:diagnosis; D008715:Methionine / Q000627:therapeutic use; D009048:Motor Skills; D010375:Pedigree; D010661:Phenylketonurias / Q000188:drug therapy; D014443:Tyrosine / Q000627:therapeutic use; D014633:Valine / Q000097:blood / Q000627:therapeutic use
chemical_list: D000596:Amino Acids; D004044:Dietary Proteins; D007651:Keto Acids; D007532:Isoleucine; D014443:Tyrosine; D008715:Methionine; D007930:Leucine; D014633:Valine