Darwish Murad Sarwa, Valla Dominique-Charles, de Groen Piet C, Zeitoun Guy, Hopmans Judith A M, Haagsma Elizabeth B, van Hoek Bart, Hansen Bettina E, Rosendaal Frits R, Janssen Harry L A
Department of Gastroenterology and Hepatology, Erasmus Medical Center Rotterdam, Rotterdam, The Netherlands.
Hepatology. 2004 Feb;39(2):500-8. doi: 10.1002/hep.20064.
Budd-Chiari syndrome (BCS) is a rare disorder that is characterized by hepatic venous outflow obstruction. The aim of this study was to assess determinants of survival and to evaluate the effect of portosystemic shunting. In this international multicenter study, 237 patients with BCS, diagnosed between 1984 and 2001, were investigated. Univariate, multivariate, and time-dependent Cox regression analyses were performed. Overall survival at 1, 5, and 10 years was 82% (95% CI, 77%-87%), 69% (95% CI, 62%-76%), and 62% (95% CI, 54%-70%), respectively. Encephalopathy, ascites, prothrombin time, and bilirubin were independent determinants of survival. A prognostic classification combining these factors could identify three classes of patients (classes I-III). The 5-year survival rate was 89% (95% CI, 79%-99%) for class I, 74% (95% CI, 65%-83%) for class II, and 42% (95% CI, 28%-56%) for class III. Anticoagulants were administered to 72%; only for patients in class I was this associated with a trend toward improved survival (relative risk [RR], 0.14; 95% CI, 0.02-1.21). Portosystemic shunting was performed in 49% of the patients (n = 117); only for patients in class II, time-dependent analyses suggested an improved survival (RR, 0.63; 95% CI, 0.26-1.49). In conclusion, at the time of diagnosis, patients with BCS can be classified into good (I), intermediate (II), and poor (III) prognostic classes, according to simple baseline clinical and laboratory parameters. Our results suggest an improved survival after surgical portosystemic shunting for patients with an intermediate prognosis (class II).
布加综合征（BCS）是一种以肝静脉流出道梗阻为特征的罕见疾病。本研究的目的是评估生存的决定因素，并评估门体分流术的效果。在这项国际多中心研究中，对1984年至2001年间诊断的237例布加综合征患者进行了调查。进行了单因素、多因素和时间依赖性Cox回归分析。1年、5年和10年的总生存率分别为82%（95%CI，77%-87%）、69%（95%CI，62%-76%）和62%（95%CI，54%-70%）。肝性脑病、腹水、凝血酶原时间和胆红素是生存的独立决定因素。结合这些因素的预后分类可识别出三类患者（I-III类）。I类患者的5年生存率为89%（95%CI，79%-99%），II类为74%（95%CI，65%-83%），III类为42%（95%CI，28%-56%）。72%的患者接受了抗凝治疗；仅I类患者的生存有改善趋势（相对风险[RR]，0.14；95%CI，0.02-1.21）。49%的患者（n = 117）进行了门体分流术；仅II类患者的时间依赖性分析显示生存有所改善（RR，0.63；95%CI，0.26-1.49）。总之，在诊断时，根据简单的基线临床和实验室参数，布加综合征患者可分为预后良好（I类）、中等（II类）和较差（III类）三类。我们的结果表明，手术门体分流术后，预后中等（II类）的患者生存有所改善。
