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目的 探讨国人家族性Budd Chiari综合征 (Budd Chiarisyndrome ,BCS)的影像学特点与介入治疗的有效性。方法 对 4例家族性BCS(两对姐妹 ,来自A、B两个家族 )行血管造影 ,并对 2例行经皮球囊血管成形术 (percutneoustransluminalangioplasty ,PTA) ,2例行内支架置入术。结果 A家族姐妹、B家族姐妹分别为下腔静脉肝后段节段性闭塞和膜性闭塞。A、B家族姐姐分别行内支架置入术 ,妹妹分别行PTA。 4例BCS行介入治疗后下腔静脉回流通畅。A家族姐妹随访 2年后下腔静脉出现再狭窄 ,姐姐再行内支架治疗 ,妹妹再行PTA ;7个月后姐姐因肝功能衰竭死亡 ,其妹妹至今未见复发。B家族姐妹随访 4年未见复发。结论 (1)国人家族性BCS病变类型多样。 (2 )静脉血栓是国人家族性发病的基本病因。 (3)PTA、内支架置入术及严格长期抗凝治疗的综合应用是治疗家族性BCS的有效方法
Objective To explore the imaging characteristics and the effectiveness of interventional therapy of Budd Chiari syndrome (Budd-Chiari syndrome) in Chinese. Methods Four cases of familial BCS (two pairs of sisters, from A and B families) underwent angiography. Two patients underwent percutaneous balloon angioplasty (PTA) and two underwent stent placement . Results A family siblings, B family siblings were segmental occlusion and membranous occlusion of the posterior segment of the inferior vena cava. A, B family sister intracranial stent placement, respectively, sister PTA. 4 cases of BCS after interventional treatment of inferior vena cava reflux patency. A family sisters follow-up after 2 years of restenosis in the inferior vena cava, sister and then stent treatment, sister and then PTA; 7 months after the death of her sister due to liver failure, her sister so far no recurrence. B family sister followed up for 4 years no recurrence. Conclusions (1) There are many types of familial BCS in Chinese. (2) Venous thrombosis is the basic cause of familial onset in China. (3) PTA, stent placement and strict long-term anticoagulant therapy is an effective treatment of familial BCS