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挪威原发性硬化性胆管炎患者肝移植的适应症及预后

Indications and Outcomes in Liver Transplantation in Patients With Primary Sclerosing Cholangitis in Norway.

作者信息

Andersen Ina M, Fosby Bjarte, Boberg Kirsten M, Clausen Ole P F, Jebsen Peter, Melum Espen, Line Pål D, Foss Aksel, Schrumpf Erik, Karlsen Tom H

机构信息

Division of Cancer Medicine, Surgery and Transplantation, Department of Transplantation Medicine, Norwegian PSC Research Center, Oslo University Hospital, Rikshospitalet, Oslo, Norway.

Division of Cancer Medicine, Surgery and Transplantation, Department of Transplantation Medicine, Norwegian PSC Research Center, Oslo University Hospital, Rikshospitalet, Oslo, Norway.; Section for Transplantation Surgery, Department of Transplantation Medicine, Oslo University Hospital, Rikshospitalet, Oslo, Norway.

出版信息

Transplant Direct. 2015 Oct 19;1(9):e39. doi: 10.1097/TXD.0000000000000548. eCollection 2015 Oct.

Abstract

BACKGROUND

Primary sclerosing cholangitis (PSC) is 1 of the leading causes of liver transplantation (LTX) in Scandinavia, and an increasing number of PSC patients have been transplanted in Norway during the last 2 decades. This trend is partly attributable to the recently established practice in Norway of offering LTX to PSC patients with cholangiocellular dysplasia. Based on the controversy associated with this practice, we herein aimed to report the main features and outcomes of our LTX program in PSC.

METHODS

The primary indication for LTX (quality of life/end-stage liver disease or suspected neoplasia) was retrospectively determined for 222 patients undergoing LTX for PSC or other autoimmune liver diseases (primary biliary cirrhosis/autoimmune hepatitis) with at least 5 years of follow-up.

RESULTS

In PSC patients impaired quality of life (43.5%) and end-stage liver disease (38.4%) were the most frequent indications for LTX, whereas suspected neoplasia accounted for 18.1%. The proportion of PSC patients with manifest encephalopathy, variceal bleeding, or ascites declined over time. In patients with suspected neoplasia as the primary indication for LTX (n = 25), neoplasia was confirmed in the explanted liver in 20 patients (80%). Five-year survival rates for PSC patients transplanted between 2001 and 2009 were 91.9% for patients receiving LTX due to impaired quality of life or end-stage liver disease and 83.3% for suspected neoplasia.

CONCLUSIONS

The PSC patients are increasingly listed for LTX at an earlier stage of their liver disease. In patients with suspected neoplasia before LTX, 5-year survival was acceptable, despite confirmation of neoplasia in 80% of the liver explants.

摘要

背景

原发性硬化性胆管炎(PSC)是斯堪的纳维亚地区肝移植(LTX)的主要原因之一,在过去20年中,挪威接受移植的PSC患者数量不断增加。这一趋势部分归因于挪威最近确立的向患有胆管细胞发育异常的PSC患者提供LTX的做法。基于与这种做法相关的争议,我们在此旨在报告我们的PSC患者LTX计划的主要特征和结果。

方法

回顾性确定222例因PSC或其他自身免疫性肝病(原发性胆汁性肝硬化/自身免疫性肝炎)接受LTX且至少随访5年的患者的LTX主要指征(生活质量/终末期肝病或疑似肿瘤形成)。

结果

在PSC患者中,生活质量受损(43.5%)和终末期肝病(38.4%)是最常见的LTX指征,而疑似肿瘤形成占18.1%。有明显肝性脑病、静脉曲张出血或腹水的PSC患者比例随时间下降。以疑似肿瘤形成为LTX主要指征的患者(n = 25)中,20例(80%)在切除的肝脏中证实有肿瘤形成。2001年至2009年间接受LTX的PSC患者,因生活质量受损或终末期肝病接受LTX的患者5年生存率为91.9%,疑似肿瘤形成的患者为83.3%。

结论

PSC患者在肝病早期越来越多地被列入LTX名单。在LTX前疑似肿瘤形成的患者中,尽管80%的肝脏切除标本证实有肿瘤形成,但5年生存率仍可接受。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d03b/4946487/49eddc8f591d/txd-1-e39-g004.jpg

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