Hepatic glycogenosis: Reversible hepatomegaly in type 1 diabetes

Authors


  • CFJ. Munns, MB BS, Royal Children’s Hospital Foundation/Cressbrook Clinical Fellow in Endocrinology. RB McCrossin, MB BS, FRACP, Executive Director Medical Services. MJ Thomsett, MB BS, FRACP, Senior Visiting Paediatric Endocrinologist. J Batch, MB BS, MD, FRACP, Royal Children’s Hospital Foundation Variety Professor of Paediatrics, Director of Endocrinology.

Correspondence: DrCfjMunns RCHF/Cressbrook Clinical Fellow in Endocrinology, Department of Endocrinology, Royal Children’s Hospital, Herston Road, Herston, Qld 4029, Australia. Email: Munnsc@health.qld.gov.au

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.

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