Darwish Murad Sarwa, Plessier Aurelie, Hernandez-Guerra Manuel, Fabris Federica, Eapen Chundamannil E, Bahr Matthias J, Trebicka Jonel, Morard Isabelle, Lasser Luc, Heller Joerg, Hadengue Antoine, Langlet Philippe, Miranda Helena, Primignani Massimo, Elias Elwyn, Leebeek Frank W, Rosendaal Frits R, Garcia-Pagan Juan-Carlos, Valla Dominique C, Janssen Harry L A
Erasmus MC University Hospital Rotterdam, Rotterdam, the Netherlands.
Ann Intern Med. 2009 Aug 4;151(3):167-75. doi: 10.7326/0003-4819-151-3-200908040-00004.
The Budd-Chiari syndrome (BCS) is hepatic venous outflow obstruction. What is known about the syndrome is based on small studies of prevalent cases.
To characterize the causes and treatment of incident BCS.
Consecutive case series of patients with incident BCS, enrolled from October 2003 to October 2005 and followed until May 2006.
Academic and nonacademic hospitals in France, Spain, Italy, Great Britain, Germany, Belgium, the Netherlands, Portugal, and Switzerland.
Persons older than 16 years with definite hepatic outflow obstruction diagnosed by imaging. Persons with hepatic outflow obstruction due to heart failure, sinusoidal obstruction syndrome, cancer, or liver transplantation were excluded.
Signs and symptoms; laboratory and imaging findings; diagnosis; treatment; and overall, transplantation-free, and intervention-free survival.
163 incident cases of BCS were identified. Median follow-up was 17 months (range, 0.1 to 31 months). Most patients (84%) had at least 1 thrombotic risk factor, and many (46%) had more than 1; the most common was myeloproliferative disorders (49% of 103 tested patients). Patients were mainly treated with anticoagulation (140 patients [86%]), transjugular intrahepatic portosystemic shunting (56 patients [34%]), or liver transplantation (20 patients [12%]), and 80 patients (49%) were managed noninvasively. Only 3 patients underwent surgical shunting. The survival rate was 87% (95% CI, 82% to 93%) at 1 year and 82% (CI, 75% to 88%) at 2 years.
Treatment was not standardized across all centers, and data on important clinical variables were missing for some patients.
Most patients with BCS have at least 1 thrombotic risk factor, and many have more than 1; myeloproliferative disorders are most common. One- and 2-year survival rates are good with contemporary management, which includes noninvasive therapies (anticoagulation and diuretics) and invasive techniques. Transjugular intrahepatic portosystemic shunting seems to have replaced surgical shunting as the most common invasive therapeutic procedure.
Fifth Framework Programme of the European Commission.
布加综合征（BCS）是肝静脉流出道梗阻。目前对该综合征的了解基于对现患病例的小型研究。
描述新发BCS的病因及治疗方法。
2003年10月至2005年10月纳入的新发BCS患者的连续病例系列研究，并随访至2006年5月。
法国、西班牙、意大利、英国、德国、比利时、荷兰、葡萄牙和瑞士的学术及非学术医院。
年龄大于16岁、经影像学确诊为明确肝流出道梗阻的患者。因心力衰竭、窦性梗阻综合征、癌症或肝移植导致肝流出道梗阻的患者被排除。
体征和症状；实验室及影像学检查结果；诊断；治疗；以及总体生存率、无移植生存率和无干预生存率。
共识别出163例新发BCS病例。中位随访时间为17个月（范围0.1至31个月）。大多数患者（84%）至少有1个血栓形成危险因素，许多患者（46%）有不止1个；最常见的是骨髓增殖性疾病（103例接受检测的患者中占49%）。患者主要接受抗凝治疗（140例患者[86%]）、经颈静脉肝内门体分流术（56例患者[34%]）或肝移植（20例患者[12%]），80例患者（49%）接受非侵入性治疗。仅3例患者接受了外科分流术。1年生存率为87%（95%CI，82%至93%），2年生存率为82%（CI，75%至88%）。
所有中心的治疗方法未标准化，部分患者缺少重要临床变量的数据。
大多数BCS患者至少有1个血栓形成危险因素，许多患者有不止1个；骨髓增殖性疾病最为常见。采用包括非侵入性治疗（抗凝和利尿剂）及侵入性技术在内的现代治疗方法，1年和2年生存率良好。经颈静脉肝内门体分流术似乎已取代外科分流术，成为最常见的侵入性治疗手段。
欧盟委员会第五框架计划
