title: Thiamine-responsive intermittent branched-chain ketoaciduria.
mesh_terms: D000592:Amino Acid Metabolism, Inborn Errors / Q000175:diagnosis / Q000178:diet therapy / Q000188:drug therapy*; D000597:Amino Acids, Branched-Chain / Q000378:metabolism*; D002675:Child, Preschool; D004044:Dietary Proteins / Q000627:therapeutic use; D006801:Humans; D007532:Isoleucine / Q000378:metabolism; D007651:Keto Acids / Q000652:urine*; D007930:Leucine / Q000378:metabolism; D008297:Male; D013831:Thiamine / Q000627:therapeutic use*; D014633:Valine / Q000378:metabolism
chemical_list: D000597:Amino Acids, Branched-Chain; D004044:Dietary Proteins; D007651:Keto Acids; D007532:Isoleucine; D007930:Leucine; D014633:Valine; D013831:Thiamine