CAJ | 학술논문

目的前瞻性研究原发性布加综合征(Budd-Chiari syndrome,BCS)的临床特征及病因.方法 2010年9月至2012年1月在我院收治的新发BCS患者,随访至2012年6月.记录所有患者症状、体征、实验室及影像学检查结果、治疗方法、介入治疗后生存期及无症状生存期.结果共149例BCS患者,中位年龄46岁(10 ～ 82岁),男女比例为1.61∶1;14.1％患者为急性或者亚急性BCS,85.9％为慢性BCS,症状持续的中位时间为96个月(1d～ 360个月)；30.9％患者单纯肝静脉受累,5.4％单纯下腔静脉受累,其余63.8％肝静脉及下腔静脉联合受累.80.9％患者至少发现一种病因学因素,最常见是静脉膜性闭塞(61.1％),4.8％患者发现骨髓增殖性疾病(JAK2基因突变),未发现凝血因子V基因突变.144例患者予以经皮血管成形术,技术成功率为95.1％,术后抗凝治疗12个月.介入治疗后18个月生存率及无症状生存率分别为97.8％和92.8％.结论 BCS最常见的病因是静脉内膜性闭塞,而与导致血栓形成的潜在疾病相关性不高；采用经皮血管成形术加术后抗凝的治疗策略能取得非常好的临床预后.
목적 전첨성연구원발성포가종합정(Budd-Chiari syndrome,BCS)적림상특정급병인.방법 2010년9월지2012년1월재아원수치적신발BCS환자,수방지2012년6월.기록소유환자증상、체정、실험실급영상학검사결과、치료방법、개입치료후생존기급무증상생존기.결과 공149례BCS환자,중위년령46세(10 ～ 82세),남녀비례위1.61∶1;14.1％환자위급성혹자아급성BCS,85.9％위만성BCS,증상지속적중위시간위96개월(1d～ 360개월)；30.9％환자단순간정맥수루,5.4％단순하강정맥수루,기여63.8％간정맥급하강정맥연합수루.80.9％환자지소발현일충병인학인소,최상견시정맥막성폐새(61.1％),4.8％환자발현골수증식성질병(JAK2기인돌변),미발현응혈인자V기인돌변.144례환자여이경피혈관성형술,기술성공솔위95.1％,술후항응치료12개월.개입치료후18개월생존솔급무증상생존솔분별위97.8％화92.8％.결론 BCS최상견적병인시정맥내막성폐새,이여도치혈전형성적잠재질병상관성불고；채용경피혈관성형술가술후항응적치료책략능취득비상호적림상예후.
Objective To prospectively study clinical features and etiology in patients with incident BuddChiari syndrome (BCS) in China.Methods Taking consecutive case series of patients with incident BCS as who were diagnosed in our hospital,enrolled from September 2010 to January 2012 as the object of research,and the follow-up was lasting until June 2012.Taking records for all patients' symptoms,signs,laboratory findings,radiology findings,treatment,interventional treatment survival and symptom-free period.Results There are total 149 incident cases of BCS.In which,the median age was 46 years old (range 10 ～ 82); 61.7％ of them were male patients,38.3％ were female patients; 85.9％ of them were chronic,the other patients (14.1％)were diagnosed during acute or subacute periods; the median duration of symptoms before diagnosis was 96 months (range 1 day ～ 360 months).In terms of causes,30.9％ of the patients caused by hepatic venous block,5.4％of them resulted from inferior vena cava block,and the rest 63.8％ were suffered from combined hepatic venous and inferior vena cava block.80.5％ patients have at least one etiological factor,Furthermore,the most common cause was the web (61.1％),only 4.8％ have myeloproliferative diseases (JAK2 V617F mutation),and none Factor V Leiden mutation cases was found.144 patients were treated by percutaneous transluminal angioplasty,the technical success rote was 95.1％,and took oral anticoagulation therapy for 12 months.At 18 months,The survival rate and the symptom-free survival rote after percutaneous transluminal angioplasty were 97.8％ and respectively.Conclusion Web is the most prevalent etiological factor for BCS in China.It is different in western countries; the common reasons of BCS are risk factors related to thrombosis,such as myeloprolifemtive disease and Factor V Leiden mutation,etc.,which are seldom found in Chinese BCS patients.In China,most chronic BCS patients were treated with percutaneous transluminal angioplasty and have excellent clinical outcome.