長期経過観察した原発性硬化性胆管炎の１例

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  • A long-term follow-up case of primary sclerosing cholangitis.

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A long-term followed-up case of primay sclerosing cholangitis (PSC), 30 y. o. male, is reported. In 1979, he admitted to our hospital for 4-year lasted general malaise and itching. On admission, laboratory findings suggested a prominant cholestatic pattern without hyperbilirubinemia. The antimitochondrial antibody, HBsAg or HBsAb were negative. ERCP demonstrated typical "constricturing and beaded" appearances and diverticular outpouchings diffusely in the biliary tree without biliary stones. The liver histology showed portal fibrosis accompanied with atypical ductular proliferation and periductal concentric fibrosis of septal bile ducts. The diagnosis of PSC was made and he was followed-up for about 6 years, during which the levels of GOT and ALP gradually elevated, resulting to show occasional short-lasting jaundice. ERCP after the 6-year follow-up, essentially unchanged, indicated an advance in the stenotic changes of the bile ducts. Findings in the liver histology, including hydropic changes and orcein-positive copper-binding proteins in hepatocytes, also indicated an advance in the cholestatic changes. By the colonoscopic examination and mucosal biopsy, a focal non-specific colitis located in the entire colon but in the rectum was demonstrated. In the present case, the biliary lesions in PSC indeed advanced during the 6-year follow -up period, but this advance seems to be rather gradual when compared to ever reported cases in Japan.

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