3-year-old girl had a history of severe growth retardation, hypoglycemia, seizures and hepato-splenomegaly (specimen from autopsy).
- The architecture of the hepatic parenchyma is maintained.
- The hepatocytes are large with centrally placed nuclei. The cytoplasm appears clear, in part coarse granules or large vacuoles with a mucinous content are present (during preparation the excess glycogen is lost).
- Some hepatocytes display macrovesicular steatosis (loss of membrane-bound triglycerides during staining).