Massive hepatic fibrosis in Gaucher's disease: clinico- pathological and radiological features

摘要

Hepatomegaly is frequent in patients with type 1 Gaucher's diseaseand is associated with infiltration Of the liver with pathologicalmacrophages. Most Patients suffer no significant clinicalconsequences, But a few develop portal hypertension which mayProgress to parenchymal liver failure. We describe Four patients withGaucher's disease who have Developed portal hypertension.