Hypoglycemia and maple syrup urine disease: defective gluconeogenesis

Pediatr Res. 1973 May;7(5):500-8. doi: 10.1203/00006450-197305000-00003.
No abstract available

Publication types

  • Case Reports

MeSH terms

  • Alanine / blood
  • Alanine / urine
  • Amino Acids / analysis
  • Chromatography
  • Diet Therapy*
  • Female
  • Fructose / therapeutic use
  • Glucagon / therapeutic use
  • Gluconeogenesis*
  • Glutamate Dehydrogenase / metabolism
  • Glutamates / metabolism
  • Glutamine / urine
  • Humans
  • Hypoglycemia / complications*
  • Infant, Newborn
  • Insulin / blood
  • Ketone Bodies / blood
  • Lactates / blood
  • Maple Syrup Urine Disease / complications*

Substances

  • Amino Acids
  • Glutamates
  • Insulin
  • Ketone Bodies
  • Lactates
  • Glutamine
  • Fructose
  • Glucagon
  • Glutamate Dehydrogenase
  • Alanine