Hepatic glycogenosis: reversible hepatomegaly in type 1 diabetes

J Paediatr Child Health. 2000 Oct;36(5):449-52. doi: 10.1046/j.1440-1754.2000.00547.x.

Abstract

Objective: To describe the aetiology, clinical features and appropriate treatment for hepatic glycogenosis in poorly controlled type 1 diabetes.

Methods: A review of three adolescents with poor diabetes control, hepatomegaly and elevated serum liver transaminase concentrations.

Results: Symptoms included abdominal pain, anorexia, nausea and vomiting. All had tender hepatomegaly; two had splenomegaly. Liver biopsy was performed on two patients. Histology revealed hepatic glycogenosis in both; one also demonstrated macrovesicular steatosis. With improved glycaemic control, all three showed resolution of their symptoms, organomegaly and elevated serum liver transaminase concentrations.

Conclusions: Insulin-reversible hepatic glycogenosis is the most common cause of hepatomegaly and raised serum liver transaminase concentrations in children and adolescents with type 1 diabetes. Having excluded other causes of hepatic dysfunction, a 4 week therapeutic trial of improved glycaemic control is recommended prior to more invasive investigations.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Biopsy
  • Child
  • Child, Preschool
  • Diabetes Mellitus, Type 1 / complications*
  • Female
  • Glycogen Storage Disease / complications*
  • Glycogen Storage Disease / physiopathology
  • Hepatomegaly / complications*
  • Humans
  • Liver / enzymology
  • Liver / pathology
  • Liver / physiopathology*
  • Male
  • Transaminases / blood

Substances

  • Transaminases