Pavri Tanya M, Herbst Alan, Reddy Rajender, Forde Kimberly A
Tanya M Pavri, Alan Herbst, Rajender Reddy, Kimberly A Forde, Division of Gastroenterology and Hepatology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA 19104, United States.
World J Gastroenterol. 2014 Nov 21;20(43):16236-44. doi: 10.3748/wjg.v20.i43.16236.
To investigate challenges, risk factors, prognostic indicators, and treatment outcomes associated with Budd-Chiari syndrome (BCS) at a tertiary care center.
A retrospective cohort study was conducted at the University of Pennsylvania in patients with a diagnosis of BCS or hepatic vein thrombosis. All patients receiving care at the University of Pennsylvania, and who had at least 2 clinical encounters in the University of Pennsylvania Health system from January 1, 2008 to September 10, 2013 were eligible for study inclusion. Data were extracted from the electronic medical record of each patient, and recorded in a secure Research Electronic Data Capture database. Logistic regression analyses were applied to identify predictors of outcome of liver transplant (LT) or death.
Between January 1, 2008 and September 10, 2013, forty-seven patients were identified. Median age was 42.4 years. Thirty-one (66.0%) were women. A majority were Caucasian (68.1%). At diagnosis, 43 (91.5%) patients had ascites, 27 (57.4%) patients had a hematologic disorder associated with a hypercoagulable state and 26 (55.3%) had cirrhosis. Forty (85.1%) patients were on anticoagulation (AC), 30 (63.8%) of whom were maintained on warfarin. Two patients (4.3%) underwent thrombolytic therapy. A transjugular intrahepatic portosystemic shunt (TIPS) was placed in 21 (44.7%) patients, 19 (90.5%) of whom were also on AC. Twenty-one (44.7%) received AC alone. Over a median of 974 d, 8 (17.0%) patients received LT, and 10 (21.3%) died. The median time from listing to death was 26 mo [interquartile range (IQR) = 16, 65)]. TIPS with AC was utilized more frequently in younger patients (P = 0.02). Age, cirrhosis and chronic kidney disease (CKD) were significant predictors of LT or death.
AC alone was employed as frequently as TIPS with AC, though the latter was used more frequently in younger patients with polycythemia vera. There were no significant differences in treatment outcome regardless of the therapeutic intervention employed. Significant predictors of poor prognosis included age, cirrhosis and CKD.
在一家三级医疗中心研究与布加综合征（BCS）相关的挑战、危险因素、预后指标及治疗结果。
在宾夕法尼亚大学对诊断为BCS或肝静脉血栓形成的患者进行一项回顾性队列研究。所有在宾夕法尼亚大学接受治疗且在2008年1月1日至2013年9月10日期间在宾夕法尼亚大学医疗系统至少有2次临床就诊经历的患者符合研究纳入标准。从每位患者的电子病历中提取数据，并记录在一个安全的研究电子数据采集数据库中。应用逻辑回归分析来确定肝移植（LT）或死亡结局的预测因素。
在2008年1月1日至2013年9月10日期间，共确定了47例患者。中位年龄为42.4岁。31例（66.0%）为女性。大多数为白种人（68.1%）。诊断时，43例（91.5%）患者有腹水，27例（57.4%）患者有一种与高凝状态相关的血液系统疾病，26例（55.3%）有肝硬化。40例（85.1%）患者接受抗凝治疗（AC），其中30例（63.8%）维持使用华法林。2例（4.3%）患者接受了溶栓治疗。21例（44.7%）患者进行了经颈静脉肝内门体分流术（TIPS），其中19例（90.5%）患者也接受了AC治疗。21例（44.7%）患者仅接受AC治疗。在中位974天的时间里，8例（17.0%）患者接受了LT，10例（21.3%）患者死亡。从列入名单到死亡的中位时间为26个月[四分位间距（IQR）=16, 65]。TIPS联合AC在年轻患者中使用更频繁（P = 0.02）。年龄、肝硬化和慢性肾脏病（CKD）是LT或死亡的重要预测因素。
单独使用AC与TIPS联合AC的使用频率相同，尽管后者在患有真性红细胞增多症的年轻患者中使用更频繁。无论采用何种治疗干预，治疗结果均无显著差异。预后不良的重要预测因素包括年龄、肝硬化和CKD。
