肝静脉血栓型布-加综合征患者的临床特征

摘要

目的 研究肝静脉(HV)血栓型布-加综合征(BCS)患者的临床特征.方法 以2010年6月至2012年12月我院连续收治的16例HV血栓型BCS患者作为研究对象,并与同期连续收治的132例其他类型BCS患者进行对比研究.回顾性分析两组患者住院及随访资料.随访截止时间为2013年6月,中位随访时间为24个月(6 ～ 36个月).采用独立样本t检验及Wilcoxon W秩和检验分析定量资料组间差异性,采用x2检验或Fisher确切概率法分析定性资料组间差异性,采用Kaplan-Meier法计算患者生存率及复发率.结果 HV血栓型BCS患者年龄、症状持续时间、白蛋白水平、脾脏直径及生存率等指标均低于其他类型BCS患者;HV血栓型BCS患者腹水比例、平均住院时间、天冬氨酸转氨酶、丙氨酸转氨酶、糖类蛋白抗原-125、总胆红素、Rotterdam BCS预后分级及介入术后复发率等指标均高于其他类型BCS患者.以上各个指标组间差异性均有统计学意义(P＜0.05).结论 与其他类型的BCS相比,HV血栓型BCS是一类发病年龄轻,症状和肝脏损伤重,介入治疗难度较大,预后差的急性BCS.%Objective To study the clinical features and prognosis of patients with primary BuddChiari syndrome (BCS) caused by hepatic vein thrombosis.Method 16 patients with primary BCS caused by hepatic vein thrombosis treated in our hospital between June 2010 to December 2012 were used as the study group while 132 patients with primary BCS caused by other causes were used as the control group.A retrospective study was then employed to analyze the clinical data of the two groups of patients during hospitalization and on follow-up.The study was censored in June 2013.The median follow-up was 24 months (range,6 months to 36 months).The difference in quantitative data between the 2 groups were analyzed using the independent-samples t test and the Wilcoxon W rank sum test,and the difference in qualitative data were analyzed using the Chi-square test and the Fisher's exact test.The survival rates and recurrence rates were calculated using the Kaplan-Meier method.Result The study group was significantly lower than the control group in age,duration of symptoms,albumin level,diameter of spleen and survival rate,but it was significantly higher in the proportion of patients with ascites,average hospitalization time,alanine transaminase,aspartate aminotransferase,total bilirubin,carbohydrate antigen-125 and recurrence rate after percutaneous transluminal angioplasty.The differences were significant (P ＜ 0.05).The Rotterdam BCS prognosis grades of the study group were:9 patients grade Ⅱ and 7 patients grade Ⅲ.In the control group,there were 65 patients with grade Ⅰ,51 patients with grade Ⅱ,and 16 patients with grade Ⅲ.The prognosis grade of the study group was significantly higher than the control group (P ＜ 0.05).Conclusion When compared to the patients with BCS due to other causes,patients with BCS caused by hepatic vein thrombosis were more common in the young,most of them were diagnosed in the acute period,they had worse clinical outcomes and had more severe clinical symptoms and liver damage.