Thiamine-responsive intermittent branched-chain ketoaciduria

J Pediatr. 1979 Apr;94(4):628-31. doi: 10.1016/s0022-3476(79)80036-0.
No abstract available

Publication types

  • Case Reports

MeSH terms

  • Amino Acid Metabolism, Inborn Errors / diagnosis
  • Amino Acid Metabolism, Inborn Errors / diet therapy
  • Amino Acid Metabolism, Inborn Errors / drug therapy*
  • Amino Acids, Branched-Chain / metabolism*
  • Child, Preschool
  • Dietary Proteins / therapeutic use
  • Humans
  • Isoleucine / metabolism
  • Keto Acids / urine*
  • Leucine / metabolism
  • Male
  • Thiamine / therapeutic use*
  • Valine / metabolism

Substances

  • Amino Acids, Branched-Chain
  • Dietary Proteins
  • Keto Acids
  • Isoleucine
  • Leucine
  • Valine
  • Thiamine