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Cirrhosis in Wilson Disease

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Wilson disease is an uncommon autosomal recessive disorder due to mutations in the ATP7B gene, which disrupt normal hepatic copper transport, leading to copper accumulation in hepatocytes and progression from steatosis to chronic hepatitis to cirrhosis (as seen here). The small cirrhotic nodules are separated by fibrous septa containing chronic inflammatory infiltrates and ductular structures. This image also shows the characteristic steatosis, ballooning degeneration, focally dense eosinophilic cytoplasm, and cholestasis seen in untreated Wilson disease.

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