title: Hypoglycemia and maple syrup urine disease: defective gluconeogenesis.
mesh_terms: D000409:Alanine / Q000097:blood / Q000652:urine; D000596:Amino Acids / Q000032:analysis; D002845:Chromatography; D004035:Diet Therapy; D005260:Female; D005632:Fructose / Q000627:therapeutic use; D005934:Glucagon / Q000627:therapeutic use; D005943:Gluconeogenesis; D005969:Glutamate Dehydrogenase / Q000378:metabolism; D005971:Glutamates / Q000378:metabolism; D005973:Glutamine / Q000652:urine; D006801:Humans; D007003:Hypoglycemia / Q000150:complications*; D007231:Infant, Newborn; D007328:Insulin / Q000097:blood; D007657:Ketone Bodies / Q000097:blood; D007773:Lactates / Q000097:blood; D008375:Maple Syrup Urine Disease / Q000150:complications*
chemical_list: D000596:Amino Acids; D005971:Glutamates; D007328:Insulin; D007657:Ketone Bodies; D007773:Lactates; D005973:Glutamine; D005632:Fructose; D005934:Glucagon; D005969:Glutamate Dehydrogenase; D000409:Alanine