Liver Unit, Queen Elizabeth Hospital, Birmingham, UK.
Expert Rev Gastroenterol Hepatol. 2012 Dec;6(6):731-44. doi: 10.1586/egh.12.56.
Budd-Chiari syndrome is a rare disorder caused by hepatic venous outflow obstruction and resulting hepatic dysfunction. Despite a lack of prospective randomized trials, much progress has been made in its management over the last 20 years. The main goals of treatment are to ameliorate hepatic congestion and prevent further thrombosis. The selective use of anticoagulation, vascular stents, transjugular intrahepatic portosystemic stent-shunt and liver transplant has resulted in a significant increase in survival. The diagnosis, initial management and long-term follow-up of patients with Budd-Chiari syndrome is reviewed. The concept of individualization of treatment and a stepwise approach to invasive procedures is also discussed.
布加综合征是一种罕见的疾病，由肝静脉流出道阻塞引起，导致肝功能障碍。尽管缺乏前瞻性随机试验，但在过去 20 年中，其治疗取得了很大进展。治疗的主要目标是改善肝脏充血和预防进一步的血栓形成。抗凝、血管支架、经颈静脉肝内门体分流术和肝移植的选择性应用导致生存率显著提高。本文回顾了布加综合征患者的诊断、初始治疗和长期随访。还讨论了治疗个体化和侵袭性操作逐步处理的概念。
