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原发性胆汁性胆管炎:一种古老疾病的新疗法。

Primary biliary cholangitis: new treatments for an old disease.

作者信息

Trivedi Hirsh D, Lizaola Blanca, Tapper Elliot B, Bonder Alan

机构信息

Liver Center, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts, USA.

Department of Internal Medicine, St. Elizabeth's Medical Center, Brighton, Massachusetts, USA.

出版信息

Frontline Gastroenterol. 2017 Jan;8(1):29-36. doi: 10.1136/flgastro-2016-100741. Epub 2016 Nov 3.

DOI:10.1136/flgastro-2016-100741
PMID:28839882
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5369441/
Abstract

Primary biliary cholangitis (PBC) is an immunological condition that causes a significant health disturbance and dramatically reduces the quality of life for those affected with the disease. It is a potentially fatal disease that can lead to multiple hepatic and extrahepatic complications. Having adequate therapeutic interventions that can improve the course of the disease is imperative in reducing the associated morbidity and mortality. Ursodeoxycholic acid (UDCA) is the gold standard therapy. However, it has been associated with suboptimal response rates in a significant proportion of patients. Despite UDCA, approximately 35%-40% of individuals with PBC still experience a progression of the disease, leading to liver failure and requiring liver transplantation. Recent studies of new pharmacological approaches have shown beneficial outcomes. Some of these agents can now be applied to a clinical scenario. In this review article, we will outline the new and emerging treatments for PBC.

摘要

原发性胆汁性胆管炎(PBC)是一种免疫性疾病,会严重影响健康,并显著降低患者的生活质量。它是一种潜在的致命疾病,可导致多种肝脏和肝外并发症。拥有能够改善疾病进程的适当治疗干预措施对于降低相关的发病率和死亡率至关重要。熊去氧胆酸(UDCA)是标准治疗方法。然而,在相当一部分患者中,它的有效率并不理想。尽管使用了UDCA,但仍有大约35%-40%的PBC患者病情仍在进展,导致肝衰竭并需要进行肝移植。最近关于新药理学方法的研究已显示出有益的结果。其中一些药物现已可应用于临床。在这篇综述文章中,我们将概述PBC的新兴治疗方法。

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本文引用的文献

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A Placebo-Controlled Trial of Obeticholic Acid in Primary Biliary Cholangitis.奥贝胆酸治疗原发性胆汁性胆管炎的安慰剂对照临床试验。
N Engl J Med. 2016 Aug 18;375(7):631-43. doi: 10.1056/NEJMoa1509840.
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Metabolic Bone Disease in Primary Biliary Cirrhosis.原发性胆汁性肝硬化中的代谢性骨病
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Management of symptom complexes in primary biliary cholangitis.原发性胆汁性胆管炎症状复合体的管理
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ASBT inhibitors in cholangiopathies - Good for mice, good for men?
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Fatigue in Primary Biliary Cirrhosis: Prevalence, Pathogenesis and Management.原发性胆汁性肝硬化中的疲劳:患病率、发病机制及管理
Dig Dis. 2015;33 Suppl 2:109-14. doi: 10.1159/000440757. Epub 2015 Dec 7.
6
Development and Validation of a Scoring System to Predict Outcomes of Patients With Primary Biliary Cirrhosis Receiving Ursodeoxycholic Acid Therapy.原发性胆汁性肝硬化患者接受熊去氧胆酸治疗结局预测评分系统的建立与验证。
Gastroenterology. 2015 Dec;149(7):1804-1812.e4. doi: 10.1053/j.gastro.2015.07.061. Epub 2015 Aug 7.
7
Advances in pharmacotherapy for primary biliary cirrhosis.原发性胆汁性肝硬化药物治疗的进展
Expert Opin Pharmacother. 2015 Apr;16(5):633-43. doi: 10.1517/14656566.2015.998650. Epub 2014 Dec 29.
8
Efficacy of obeticholic acid in patients with primary biliary cirrhosis and inadequate response to ursodeoxycholic acid.奥贝胆酸治疗原发性胆汁性胆管炎患者对熊去氧胆酸应答不足的疗效。
Gastroenterology. 2015 Apr;148(4):751-61.e8. doi: 10.1053/j.gastro.2014.12.005. Epub 2014 Dec 11.
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Dual activation of the bile acid nuclear receptor FXR and G-protein-coupled receptor TGR5 protects mice against atherosclerosis.胆汁酸核受体FXR和G蛋白偶联受体TGR5的双重激活可保护小鼠免受动脉粥样硬化的影响。
PLoS One. 2014 Sep 19;9(9):e108270. doi: 10.1371/journal.pone.0108270. eCollection 2014.
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Levels of alkaline phosphatase and bilirubin are surrogate end points of outcomes of patients with primary biliary cirrhosis: an international follow-up study.碱性磷酸酶和胆红素水平是原发性胆汁性肝硬化患者结局的替代终点:一项国际随访研究。
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