布-加综合征病因和诊疗模式的东西方差异OA
Differences in the etiology and diagnostic and therapeutic mode of Budd-Chiari syndrome between China and Western countries
布-加综合征(BCS)是指肝静脉和/或其开口近端的下腔静脉发生阻塞,继而引发门静脉和/或下腔静脉高压的一组临床综合征.既往研究显示,BCS在不同地区人群中的病因构成、临床分型以及治疗策略存在显著差异.西方国家BCS多继发于骨髓增殖性肿瘤等血栓性疾病,病变以肝静脉阻塞为主;治疗上多遵循"抗凝-开通-分流-肝移植"的阶梯式策略,经颈静脉肝内门体分流术的介入时机相对较早.相比之下,中国BCS更常见于肝后段下腔静脉膜性阻塞及混合型阻塞,经皮腔内血管成形术在治疗体系中占据核心地位.上述差异可能与遗传背景、环境暴露以及医疗实践模式等多重因素相关,并对临床决策路径与预后评估产生重要影响.本文基于文献梳理,系统总结BCS的地域异质性特征,旨在为临床医师提供跨地域的诊疗参考,并为未来围绕BCS地域差异开展国际合作研究提供线索与方向.
Budd-Chiari syndrome(BCS)is a clinical syndrome of portal vein and/or inferior vena cava(IVC)hypertension caused by obstruction of the hepatic veins and/or the IVC proximal to its opening.Previous studies have shown that there are significant differences in the etiology,clinical classification,and therapeutic strategy of BCS across different regions.In Western countries,BCS is often secondary to thrombotic disorders,especially myeloproliferative neoplasms,with hepatic vein obstruction as the main lesion;the treatment of BCS mainly follows a stepwise strategy of anticoagulation,recanalization,shunting,and liver transplantation,with relatively early timing for transjugular intrahepatic portosystemic shunt.In contrast,BCS in China mainly involves membranous obstruction of the retrohepatic IVC and mixed-type obstruction,and percutaneous transluminal angioplasty is the core treatment method for BCS.These differences may be associated with various factors such as genetic background,environmental exposure,and healthcare practice patterns,with a significant impact on clinical decision-making and prognostic assessment.This article systematically summarizes the geographic heterogeneity of BCS through a literature review,so as to provide a cross-regional diagnostic and therapeutic reference for clinicians,as well as clues and directions for international collaborative research on the regional differences of BCS in the future.
王祯;帖君
中国人民解放军空军军医大学西京医院消化病医院,国家消化系统疾病临床医学研究中心,消化系肿瘤整合防治全国重点实验室,西安 710032中国人民解放军空军军医大学西京医院消化病医院,国家消化系统疾病临床医学研究中心,消化系肿瘤整合防治全国重点实验室,西安 710032
布-加综合征病因治疗学
Budd-Chiari SyndromeEtiologyTherapeutics
《临床肝胆病杂志》 2026 (4)
755-760,6
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