Wilson disease

Liver biopsy from a 4-year-old female patient whose 10-year-old sister presented with liver failure and cirrhosis secondary to previously unrecognized Wilson disease. Liver biopsy was performed because serum ceruloplasmin was low (4.0 mg/dL) and serum aminotransferases were repeatedly two to three times normal.

(A) Low power shows portal fibrosis, mild portal inflammation, and fatty infiltration (Masson trichrome).