門脈圧亢進症を初発症状とする早期の原発性胆汁性肝硬変(PBC)の病理組織学的検討

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Portal hypertension in patients with early primary biliary cirrhosis (PBC) is infrequent but is wellknown.<BR>Clinical and histological examinations were performed on 4 patients clinically diagnosed as idiopathic Portal hypertension (IPH) or liver cirrhosis (LC) and diagnosed as early PBC on the basis of histological findings. All the patients were women with a mean age of 58.5 years. Laboratory data showed elevation of serum alkaline phosphatase and immunoglobulin M with a mean value of 834 IU/l and 539 mg/dl. Antimitochondrial antibodies were positive in all patients. Histological findings of the liver specimen revealed chronic non-suppurative destructive cholangitis (CNSDC) in all cases. corresponding to Scheuer's stage I-II without cirrhotic changes. Abnormal vascular channels also described in the liver with IPH were present. There was a similarity between IPH and PBC, suggesting an association in their pathogenesis.