Clinical and biochemical-genetic aspects of intermittent branched-chain ketoaciduria. Report of two Scandinavian families.
D000293:Adolescent; D000596:Amino Acids / Q000097:blood / Q000652:urine; D001669:Biochemical Phenomena; D001671:Biochemistry; D002247:Carbon Isotopes; D002648:Child; D002675:Child, Preschool; D002845:Chromatography; D004569:Electroencephalography; D005260:Female; D006801:Humans; D007223:Infant; D007651:Keto Acids / Q000652:urine; D007962:Leukocytes / Q000201:enzymology; D008297:Male; D008375:Maple Syrup Urine Disease / Q000145:classification / Q000235:genetics*; D009664:Norway; D010375:Pedigree; D010507:Periodicity; D010634:Phenobarbital / Q000627:therapeutic use; D012640:Seizures / Q000188:drug therapy
D000596:Amino Acids; D002247:Carbon Isotopes; D007651:Keto Acids; D010634:Phenobarbital