临床外科杂志 ›› 2020, Vol. 28 ›› Issue (6): 593-595.doi: 10.3969/j.issn.1005-6483.2020.06.030

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布加综合征诊治现状

  

  1. 西安交通大学第一附属医院消化内科
  • 出版日期:2020-06-20 发布日期:2020-06-20

Current status of diagnosis and treatment of Budd Chiari syndrome

  • Online:2020-06-20 Published:2020-06-20

摘要: 布加综合征(BCS)是一种少见的由肝静脉流出道阻塞导致的门静脉和(或)下腔静脉高压的疾病,其病因包括高凝状态、骨髓增殖性疾病和环境条件等。BCS诊断首推彩色多普勒超声,其次CT或MR,其治疗的主要目的是改善肝淤血和防止进一步血栓形成,目前介入治疗已成为首选,原位肝移植可作为补救性治疗措施。本文就布加综合征的流行病学、病因及发病机制、诊断、治疗及长期随访进行综述。

关键词: 布加综合征, 球囊扩张, 经颈静脉肝内门体分流术

Abstract: BuddChiari syndrome is rare disorder by hepatic venous outflow obstraction and resulting portal hypertension and(or)hypertension of infeior vena cava.Etiological factors include hypercoagulable conditions,myeloproliferative desenses and enviroment conditions.The diagnosis is establisbed by demonstrating obstruction of hepatic venous outflow by ultrasound,computed tomography or magnetic resonance.The main goals of treatment are to ameliorate hepatic congestion and prevent further thrombosis.The selective use of anticoagulation,revascularization,transjugular intrahepatic portosystemic shunt has resulted in a significant increase.Orthotopic liver transplant should be used as a rescue therapy.The epidemiology,etiology and pathogenesis,diagnosis,mangement and longterm followup of patiens with budd-Chiari syndrome is reviewed.

Key words: BuddChiari syndrome, balloon dilatation, transjugular intrahepatic portosystemic shunt

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