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原发性硬化性胆管炎是一种难治性疾病。

Primary sclerosing cholangitis as an intractable disease.

作者信息

Tamura Sumihito, Sugawara Yasuhiko, Kokudo Norihiro

机构信息

Artificial Organ and Transplantation Division, Department of Surgery, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan.

出版信息

Intractable Rare Dis Res. 2012 Feb;1(1):13-7. doi: 10.5582/irdr.2012.v1.1.13.

Abstract

Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of unknown origin which eventually results in liver cirrhosis. The disease is reported to be more common among the Western population than in the Asian population. Asian experience remains limited. Diagnosis and treatment standards in the Far East have largely followed those in the West, including liver transplantation. Unlike in the West, however, recent reports from Japan have presented with a higher recurrence rate of PSC after liver transplantation, suggesting the intractable nature of the disease even after the replacement of the entire affected organ.

摘要

原发性硬化性胆管炎(PSC)是一种病因不明的慢性胆汁淤积性肝病,最终会导致肝硬化。据报道,该疾病在西方人群中比在亚洲人群中更为常见。亚洲的相关经验仍然有限。远东地区的诊断和治疗标准在很大程度上遵循了西方的标准,包括肝移植。然而,与西方不同的是,日本最近的报告显示,肝移植后PSC的复发率较高,这表明即使更换了整个受影响的器官,该疾病仍然难以治疗。

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