The management of liver disease in people with congenital bleeding disorders: guidance from EAHAD, EHC, ISTH, WFH.

IF 5.5 2区医学 Q1 HEMATOLOGY

Journal of Thrombosis and Haemostasis Pub Date : 2024-09-11 DOI:10.1016/j.jtha.2024.08.018

Vincenzo LA Mura,Massimo Colombo,Graham R Foster,Paolo Angeli,Wolfgang Miesbach,Robert Klamroth,Glenn F Pierce,Brian O'Mahony,Ming Y Lim,Virginia Hernandez-Gea,Michael Makris,Flora Peyvandi

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引用次数: 0

Abstract

People with bleeding disorders (PWBD) have been exposed to the risk of developing chronic viral hepatitis and cirrhosis after replacement therapy. At today, the advent of new pharmacological strategies for the control of hemostasis and the efficacious antiviral therapies against HCV and HBV have significantly reduced this risk. However, the real success for liver health in this clinical setting is based on other risk factors, among them, the severity of liver disease at time of HBV/HCV antiviral therapy and the exposure to highly prevalent factors of chronic liver damage (e.g.; metabolic dysfunction and/or alcohol) that can cause a residual risk of complications such as hepatocellular carcinoma, portal hypertension, liver insufficiency. With this background, a group of experts selected among hepatologists, PWBD treaters and patient representatives, produced this practical multisociety guidance for the protection of liver health and the prevention and management of liver complications in PWBD based on the most updated protocols of care.

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先天性出血性疾病患者的肝病管理：来自 EAHAD、EHC、ISTH 和 WFH 的指南。

出血性疾病患者（PWBD）在接受替代治疗后，面临着罹患慢性病毒性肝炎和肝硬化的风险。如今，控制止血的新药策略以及针对丙型肝炎病毒（HCV）和乙型肝炎病毒（HBV）的高效抗病毒疗法的出现大大降低了这一风险。然而，在这种临床环境下，肝脏健康的真正成功取决于其他风险因素，其中包括 HBV/HCV 抗病毒治疗时肝脏疾病的严重程度，以及是否暴露于慢性肝损伤的高发因素（如代谢功能障碍和/或酒精），这些因素会导致肝细胞癌、门静脉高压症、肝功能不全等并发症的残余风险。在此背景下，一组从肝病专家、肺结核患者治疗者和患者代表中挑选出来的专家，根据最新的护理方案，为保护肝脏健康、预防和管理肺结核患者的肝脏并发症制定了这份实用的多协会指南。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Journal of Thrombosis and Haemostasis 医学-外周血管病

CiteScore

24.30

自引率

3.80%

发文量

321

审稿时长

1 months

期刊介绍： The Journal of Thrombosis and Haemostasis (JTH) serves as the official journal of the International Society on Thrombosis and Haemostasis. It is dedicated to advancing science related to thrombosis, bleeding disorders, and vascular biology through the dissemination and exchange of information and ideas within the global research community. Types of Publications: The journal publishes a variety of content, including: Original research reports State-of-the-art reviews Brief reports Case reports Invited commentaries on publications in the Journal Forum articles Correspondence Announcements Scope of Contributions: Editors invite contributions from both fundamental and clinical domains. These include: Basic manuscripts on blood coagulation and fibrinolysis Studies on proteins and reactions related to thrombosis and haemostasis Research on blood platelets and their interactions with other biological systems, such as the vessel wall, blood cells, and invading organisms Clinical manuscripts covering various topics including venous thrombosis, arterial disease, hemophilia, bleeding disorders, and platelet diseases Clinical manuscripts may encompass etiology, diagnostics, prognosis, prevention, and treatment strategies.