原发性硬化性胆管炎的诊断和治疗

舒慧君; 朱峰

原发性硬化性胆管炎的诊断和治疗

舒慧君,
朱峰

中国医学科学院北京协和医院消化内科

详细信息

中图分类号: R575.6
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出版历程
- 收稿日期: 2011-02-14
- 出版日期: 2011-06-20

Diagnosis and treatment of primary sclerosing cholangitis

摘要

摘要: 原发性硬化性胆管炎(PSC)是一种以胆管的进行性炎症、纤维化和多发性狭窄为主要病理特征的慢性胆汁淤积性肝病,其发病可能与遗传及免疫机制有关,其病变范围可累及肝内和肝外胆管。部分患者具备典型的胆汁淤积表现和PSC的组织学特征,但胆管造影正常,称为小胆管PSC。PSC的病程多呈慢性进行性,最终演变为终末期肝病。60%～80%的PSC患者可并发炎症性肠病,约20%的患者还可并发胆管癌。目前针对PSC的治疗,除肝移植外尚无确切有效的治疗方法。
- 胆管炎 /
- 硬化性
Abstract: Primary sclerosing cholangitis (PSC) is a chronic, cholestatic liver disease characterized by inflammation and fibrosis of both intra-and extra-hepatic bile ducts, leading to the formation of multifocal bile duct strictures and eventually cirrhosis and end-stage liver disease.Genetic and immunologic factors appear to play key roles in disease susceptibility and progression.Patients with histological, biological and clinical features of PSC but with normal cholangiogram are diagnosed as small duct PSC.PSC is strongly associated with inflammatory bowel disease (IBD) and cholangiocarcinoma.The reported frequency of IBD and cholangiocarcinoma in patients with PSC is 60%-80% and 20% respectively.Except for liver transplantation, no specific therapy has been proved effective for PSC.
- cholangitis /
- sclerosing

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参考文献(31)

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