Abstract
A case of typical Wilson's disease was reported with clinical and pathomorphological study. An 18 year old man was admitted with liver dysfunction including Kayser-Fleischer ring, hypo-ceruloplasminemia and hypo-copperemia in the serum. Hepatic tissue obtained by open biopsy included 292μg of copper per g dry weight. Histologically, the hepatic tissue showed normonodular cirrhosis demarcated by thin fibrous connective tissue with irregular and focal copper deposition in hepatic parenchymal cells. Electronmicroscopic inspection revealed that Ito cells (Fat Storing cell: FSC), which characteristically include multiple fatty droplets in their cytoplasm, increased in the peri-hepatocellular space with fine fibrogenesis. These finding may support the idea that fibrogenesis in Wilson's disease depends not only on secondary fibrosis, which appears after hepatocytic necrosis, but also on primary fibrosis due to Ito cells.
