Hepatomegaly in Type 1 Diabetes Mellitus: When to Suspect of Glycogenic Hepatopathy?

Abstract

Uncontrolled type 1 diabetes mellitus is associated with recognized short- and long-term complications. The authors describe a case of a female adolescent, with history of poorly controlled type 1 diabetes mellitus, who presented with tender hepatomegaly and elevated liver enzymes. Her growth and pubertal development were appropriated for age. After excluding infectious and autoimmune causes of liver disease, a liver biopsy was performed and histology revealed glycogenosis. Glycogenic hepatopathy is characterized by abnormal glycogen accumulation in hepatocytes. It is a reversible condition, with good glycemic control, and is not known to progress to fibrosis. This entity should be distinct from other causes of hepatomegaly and elevated liver enzymes, in diabetic patients, such as nonalcoholic fatty liver disease. Although glycogenic hepatopathy is a major cause of hepatomegaly in type 1 diabetes mellitus, is rare and likely under-recognized, the diagnosis has implications in the management and outcome. J Med Cases. 2013;4(11):726-728 doi: https://doi.org/10.4021/jmc1516w