Hepatic glycogenosis: Reversible hepatomegaly in 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 inc...

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Bibliographic Details
Published in:Journal of paediatrics and child health 2000-10, Vol.36 (5), p.449-452
Main Authors: Munns, Cfj, McCrossin, Rb, Thomsett, Mj, Batch, J
Format: Article
Language:English
Subjects:
Adolescent
Associated diseases and complications
Australia
Biological and medical sciences
Biopsy
Child
Child, Preschool
Diabetes
Diabetes Mellitus, Type 1 - complications
Diabetes. Impaired glucose tolerance
Endocrine pancreas. Apud cells (diseases)
Endocrinopathies
Female
Glycogen Storage Disease - complications
Glycogen Storage Disease - physiopathology
Hepatomegaly
Hepatomegaly - complications
Humans
insulin
Liver - enzymology
Liver - pathology
Liver - physiopathology
Male
Medical sciences
reversible hepatomegaly
Transaminases - blood
type 1 diabetes
Young people
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Summary: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.
ISSN:1034-4810
1440-1754
DOI:10.1046/j.1440-1754.2000.00547.x