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Summary
KETOTIC HYPOGLYCEMIA
unknown

Detail information to gene locus by the National Center for Biotechnology Information NCBI:
  • frequent feeding
  • glucose infusion (acute)
  • high-carbohydrate diet
should only be diagnosed after exclusion of disorders of carbohydrate or fat metabolism ! very common pathogenesis unknown
symptoms
hypoglycemia
ketosis
onset, child
vomiting
laboratory finding
Glucose0.501.002.803.80mmol/lserum
Ketone bodies +++urine
Ketone bodies4.0010.00 mmol/lblood
Literature
Huijmans JGet al.Functional hyperactivity of hepatic glutamate dehydrogenase as a cause of the hyperinsulinism/hyperammonemia syndrome: effect of treatmentPediatrics1060596-6002000
Yorifuji Tet al.Hyperinsulinism-hyperammonemia syndrome caused by mutant glutamate dehydrogenase accompanied by novel enzyme kineticsHum Genet1040476-48-791999
Hsu BYet al.Protein-sensitive and fasting hypoglycemia in children with the hyperinsulinism/hyperammonemia syndromeJ Pediatr1380383-3892001
Lehnert Wet al.2-Methyl-3-hydroxybutyryl-CoA dehydrogenase deficiency - new case report15. Jahrestagung der Arbeitsgemeinschaft f+€r P+ñdiatrische Stoffwechselst+Ârungen -APS-7.-9. M+ñrz 2001Fulda002001