Liver abnormalities in patients with Gaucher's disease

摘要: Clinical and liver histopathological observations are described in 25 patients with Gaucher's disease. Although 24 had hepatomegaly, the majority abnormalities of serum transaminases, alkaline phosphatase, BSP clearance, only 3 evidence portal hypertension complications advanced Liver biopsies showed scattered foci Gaucher cells 5 prominent centrilobular accumulation 14 patients. Three cirrhosis, which was associated extensive replacement by storage cells. The severity correlated occurrence other severe wide spectrum disease should be considered evaluating trials therapeutic enzyme replacement.