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肝移植治疗原发性胆汁性肝硬化

Authors
Steven Flamm, MD
Fredric D Gordon, MD
Raoul Poupon, MD
Section Editor
Robert S Brown, Jr, MD, MPH
Deputy Editor
Anne C Travis, MD, MSc, FACG, AGAF
Translators
梁锐, 副主任医师，教授

引言

肝移植可成功治疗原发性胆汁性肝硬化(primary biliary cirrhosis, PBC)引起的终末期肝病。近年来因PBC而行肝移植的总体数量略微下降，这很可能反映了早期治疗的获益[1]。然而，对于那些虽行药物治疗但疾病仍然进展的患者，移植仍然是一个重要的选择。在美国，因PBC而行肝移植的患者的平均年龄为53-55岁[1]。

本专题将对关于PBC患者中行肝移植的患者筛选、移植时机及移植结局的问题进行总结。其他关于PBC的发病机制、临床表现、诊断及治疗的问题将在别处讨论。 (参见“原发性胆汁性胆管炎(原发性胆汁性肝硬化)的临床表现、诊断和预后”和“原发性胆汁性肝硬化的治疗概述”和“原发性胆汁性肝硬化的发病机制”和“熊去氧胆酸治疗原发性胆汁性胆管炎(原发性胆汁性肝硬化)的有关试验”)

移植的最佳时机

决定肝移植的最佳时机是一项重要的议题。许多机构已形成了使用临床变量来评估患者生存情况的模型。已建立的模型有两种类型：一类基于纳入研究时的初始数据，另一类使用初始和随访数据。Mayo模型(表 1)使用初始评估数据，且使用最为广泛，但是由于患者的个体差异性，它并不能替代有经验的临床医师的意见[2,3]。通过使用当前的实验室和临床数据，Mayo模型可以预测患者的短期和长期生存情况。该工具用于预期肝功能衰竭，允许临床医师及时将患者转至行肝移植。尽管这些模型对评估PBC患者的生存有特异性，但PBC患者的移植是通过MELD(Model for End-stage Liver Disease)评分来区分优先次序，并且该评分也经常被用于决定何时对患者进行移植(计算器 1)。 (参见“终末期肝病模型(MELD)”)

除了需要考虑MELD评分和Mayo模型外，如果出现下述一种或多种情况，我们建议PBC患者转诊进行移植评估：

●血浆胆红素浓度高于5mg/dL并且仍在升高

            

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Literature review current through: 2017-06 . | This topic last updated: 2016-01-12.
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