兄弟姐妹中同时出现原发性硬化性胆管炎和胆道闭锁的巧合病例？

Coincidental cases of primary sclerosing cholangitis and biliary atresia in siblings?

作者信息

Isoyama K, Yamada K, Ishikawa K, Sanada Y

机构信息

Department of Paediatrics, Showa University Fujigaoka Hospital, Yokohama, Japan.

出版信息

Acta Paediatr. 1995 Dec;84(12):1444-6. doi: 10.1111/j.1651-2227.1995.tb13589.x.

DOI:10.1111/j.1651-2227.1995.tb13589.x

PMID:8645970

Abstract

Familial cases of primary sclerosing cholangitis or biliary atresia have been reported, although genetic influences and immunopathological abnormalities in these diseases are considered to be obscure. We report a case of primary sclerosing cholangitis and biliary atresia in siblings with the observation of HLA-DR antigen expression in the abnormal bile duct epithelial cells.

摘要

虽然原发性硬化性胆管炎或胆道闭锁的家族性病例已有报道，但这些疾病中的遗传影响和免疫病理异常被认为是不明确的。我们报告了一例原发性硬化性胆管炎和胆道闭锁的同胞病例，并观察到异常胆管上皮细胞中有HLA-DR抗原表达。