454 Hepatic Glycogenosis in Type I Diabetes Mellitus: Report of Two Cases and Review of the Literature

Aim Hepatic glycogenosis is an underrecognized cause of serum transaminase elevations in poorly controlled type I diabetes mellitus, which has a relatively benign course with appropriate treatment.1,2 Objective of this study is to describe the aetiology, clinical presenting symptoms and treatment op...

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Published inArchives of disease in childhood Vol. 97; no. Suppl 2; p. A133
Main Authors Groot, LW de, Geven, WB
Format Journal Article
LanguageEnglish
Published London BMJ Publishing Group Ltd and Royal College of Paediatrics and Child Health 01.10.2012
BMJ Publishing Group LTD
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Summary:Aim Hepatic glycogenosis is an underrecognized cause of serum transaminase elevations in poorly controlled type I diabetes mellitus, which has a relatively benign course with appropriate treatment.1,2 Objective of this study is to describe the aetiology, clinical presenting symptoms and treatment options. Methods A report of two adolescents with poor controlled diabetes mellitus, hepatomegaly and serum transaminase elevations and a literature review. Results Both cases presented with abdominal pain and hepatomegaly, combined with nausea and dyspeptic complaints. Laboratory investigation revealed marked elevation of serum transaminase levels. Synthetic function of the liver stayed intact. Abdominal ultrasound showed isolated, homogenous hepatomegaly, without other abdominal abnormalities. In one case liver biopsy was performed, showing hepatic glycogenosis. Other causes for hepatomegaly were excluded. With improved diabetic control all complaints improved within three weeks, with normalisation of serum transaminase levels. Review of literature that hepatic glycogenosis, not frequently described, is an important complication of type I diabetes mellitus. Hepatic glycogenosis as result of glycogen storage in hepatocytes, caused by periods of hyperglycaemia and frequent insulin boluses. This process is reversible with improved glycaemic control.1,2 Conclusions Hepatic glycogenosis is a important complication of type I diabetes mellitus which can be reversible with the proper treatment. Therefore, medical attention is necessary. References C.F.J. Munns et al. Hepatic glycogenosis: Reversible hepatomegaly in type 1 diabetes. Journal of Paediatrics and Child Health 2000; 36:449–452. M. Torbenson et al. Glycogenic Hepatopathy, an underrecognized hepatic complication of diabetes mellitus. The American Journal of Surgical Pathology 2006; 30:508–513.
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ISSN:0003-9888
1468-2044
DOI:10.1136/archdischild-2012-302724.0454