A Case of Probable Hepatic Glycogenosis Accompanying Poorly Controlled Type 1 Diabetes Mellitus

The major causes of hepatomegaly and elevated transaminases in patients with diabetes mellitus are glycogenosis or fatty liver and non-alcoholic steatohepatitis (NASH). Although hepatic glycogenosis is common in this population, hepatomegaly or elevated transaminase are more often attributed to steatosis than to glycogen excess, and so as glycogenosis may be considered a rare cause of these findings. Accordingly, information on adult diabetic patients with glycogen-induced hepatomegaly is scant. The accumulation of excessive amounts of glycogen in the hepatocytes is a consequence of intermittent episodes of hyperglycemia and hypoglycemia and the use of excessive insulin. Hepatic glycogenosis occurs in patients with poorly controlled type 1 or type 2 diabetes; whereas steatosis is associated with obesity and insulin resistance. The distinction between steatosis and glycogenosis is important. Steatosis may progress to fibrosis and cirrhosis, whereas glycogenosis improves dramatically in response to the institution of euglycemic control. In addition, true diagnosis avoids the unnecessary and invasive investigations. We present a case clinically suggesting hepatic glycogenosis that led to symptomatic hepatomegaly and markedly elevated serum aminotransferases in patient with type 1 diabetes mellitus. Turk Jem 2012; 16: 79-81