Orthotopic liver transplantation in glycogen storage disease type la: Perioperative glucose and lactate homeostasis

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Glycogen storage disease type 1a (GSD 1a) is a rare inborn error of metabolism. It causes severe fasting intolerance and lactic acidosis due to the deficiency of glucose-6-phosphatase enzyme. Blood glucose and lactate concentrations from 2 patients with GSD 1a were retrospectively compared to a control group of patients with familial amyloid polyneuropathy. Carbohydrate intake and infusions were compared to experimental data based on stable isotope studies. Perioperative lactate concentrations were significantly higher in our 2 patients with GSD 1a (median 15.0 mmol/L; range 9.9-22.0 mmol/L) versus 8 controls. In one patient, despite normal blood glucose concentrations, lactate acidosis was probably caused by a combination of the disease itself, insufficient (par)enteral carbohydrate intake, Ringer lactate infusions, and circulatory insufficiency. Patients with GSD 1a carry an increased risk of lactic acidosis during orthotopic liver transplantation compared to non-GSD patients. Multidisciplinary perioperative care is essential to prevent significant complications.
Original languageEnglish
JournalJournal of Inborn Errors of Metabolism and Screening
Publication statusPublished - 25-Feb-2016


  • Familial amyloid polyneuropathy
  • Glucose blood
  • Glycogen storage disease type la
  • Hypoglycemia
  • Lactic acidemia
  • Liver transplantation
  • glucose
  • lactic acid
  • adult
  • article
  • bleeding
  • carbohydrate intake
  • clinical article
  • controlled study
  • female
  • glucose homeostasis
  • glycogen storage disease type 1
  • glycogen storage disease type 1a
  • human
  • hypoglycemia
  • lactic acidosis
  • liver transplantation
  • male
  • priority journal
  • retrospective study
  • young adult

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