Clinicopathological studies of primary biliary cirrhosis.

概要

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In order to investigate clinicopathological features of primary biliary cirrhosis (PBC), 66 patients with PBC and 3 patients with chronic active hepatitis (CAH)-PBC mixed type were studied. Most of PBC patients were middle aged women. In laboratory findings of them, increased erythrocyte sedimentation rate (ESR), elevated serum alkaline phosphatase and high level of serum IgM were characteristic. Although the positive rate of serum anti-mitochondrial antibody (AMA) was only 0.6% in various liver diseases other than PBC, the rate in PBC was 81.8%. Therefore, it was suggested that AMA was the specific autoantibody for PBC. Initial symptoms of most patients with symptomatic PBC were pruritus and jaundice. Most of asymptomatic PBC patients were incidentally pointed out liver dysfunction in medical examination. The survival rate of asymptomatic PBC was significantly higher than that of symptomatic PBC in the observation periods. Therefore, the prognosis of asymptomatic PBC was considered to be different from that of symptomatic PBC. According to the immunological studies for families, there were not so distinct relationships between the development of PBC and the genetic predisposition except for a few cases. CAH-PBC mixed type progressed more rapidly than symptomatic or asymptomatic PBC.
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