Mallory bodies in a patient with type Ia glycogen storage disease.

Mallory bodies were determined by light microscopy and confirmed by electron microscopy in the liver of a 37-yr-old woman with type Ia glycogen storage disease. They were found mainly in the swollen hepatocytes of the centrilobular region in the liver associated with periportal and centrilobular pericellular fibrosis. Ultrastructurally, the shape of small circular structures found in the Mallory body was similar to that of the circularly disorganized rough endoplasmic reticulum seen around the Mallory body. Although Mallory bodies are seen in several liver diseases other than alcoholic liver injury, there has been only one report of finding them in the liver in type I glycogen storage disease.