Glycogenic Hepatopathy

Glycogenic hepatopathy results from the pathologic accumulation of excess glycogen within the liver and is most commonly associated with poorly controlled type I diabetes mellitus. Additional patient groups that can be affected by glycogenic hepatopathy include those with type II diabetes mellitus, urea cycle defects, and drug effect. The clinical presentation typically includes varying degrees of hepatomegaly, abdominal pain, and elevated transaminases. Occasionally, the transaminase elevations can be dramatic and reach levels of greater than 10 times the upper limit of normal. The liver shows diffusely pale staining hepatocytes on routine H&E stains and excessive glycogen accumulation on PAS stains. Abundant glycogenated nuclei and megamitochondria can be seen, but there should be little or no inflammation, mild or absent fatty change, and no significant fibrosis. Other clinical and histologic lesions associated with abnormal glycogen accumulation include genetic glycogen storage disease, glycogen storing foci, and pseudo-ground glass change.