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.
Hepatic glycogenosis: Reversible hepatomegaly in type 1 diabetes
Article first published online: 30 SEP 2008
Blackwell Science Asia Pty. Ltd.
Journal of Paediatrics and Child Health
Volume 36, Issue 5, pages 449–452, October 2000
How to Cite
Munns, C., McCrossin, R., Thomsett, M. and Batch, J. (2000), Hepatic glycogenosis: Reversible hepatomegaly in type 1 diabetes. Journal of Paediatrics and Child Health, 36: 449–452. doi: 10.1046/j.1440-1754.2000.00547.x
- Issue published online: 30 SEP 2008
- Article first published online: 30 SEP 2008
- reversible hepatomegaly;
- type 1 diabetes
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.