Budd—Chiari综合征151例临床分析

The clinical characteristics of 151 cases of Budd-Chiari syndrome

【摘要】目的总结Budd—Chiari综合征的临床特点。方法对1996--2012年北京大学人民医院收治的151例Budd—Chiari综合征临床资料进行回顾分析。结果患者多以腹胀为主诉就诊，下肢水肿占62．9％（95／151），腹水征阳性占60．9％（92／151），53．0％（80／151）存在典型的白下而上血流的纵行腹壁曲张静脉。丙氨酸转氨酶21．5（15．0，30．0）U／L；天冬氨酸转氨酶30．0（23．8，42．0）U／L；总胆红素31．1（23．3，47．8）txmol／L；血清白蛋白37．5（31．8，41．5）g/L；凝血酶原活动度71％（61％，84％）；白细胞计数5．2（3．5，7．5）×10^9／L；血红蛋白126．5（108．8，144．2）g／L；血小板计数117．0（85．5，155．5）×10^9／L。B超显示68．9％（104／151）患者存在肝脏增大。手术中和（或）血管造影显示，41．1％（62／151）为单纯下腔静脉狭窄或阻塞，15．9％（24／151）为单纯肝静脉狭窄或阻塞，43．0％（65／151）为下腔静脉合并肝静脉狭窄或阻塞，术后证实59．6％（90／151）病变为腔静脉内膜样结构合并血栓形成。结论Budd—Chiari综合征以肝脏、下腔静脉淤血以及门静脉高压为主要临床表现，其肝功能损害轻，与门静脉高压程度不平行。与肝硬化相比，Budd—Chiari综合征脾功能亢进的程度较轻。

Objective To summarize the clinical features of Budd-Chiari syndrome. Methods A total of 151 Budd-Chiari syndrome admitted in Peking University People＇s Hospital from 1996 to 2012 were analyzed retrospectively. Results Abdominal distension was the most common complaint, with 62.9% （95/151）of lower extremity edema, 53.0% （80/151）of typical bottom-up flow of the abdominal wall longitudinal varicose veins and 60. 9% （ 92/151 ） with ascites. Laboratory tests results showed median of alanine aminotransferase （ALT） was 21.5 （ 15.0,30. 0） U/L, aspartate aminotransferase （AST） was 30. 0 （23.8,42. 0） U/L, total bilirubin was 31.1 （23.3,47.8） txmol/L, blood albumin 37.5 was （ 31.8,41.5 ） g/L, prothrombin activity was 71% （61%, 84% ）. WBC was 5.2 （3.5,7.5） ~ 109/L, hemoglobin concentration was 126. 5 （ 108.8,144. 2） g/L, and platelet count was 117.0 （85.5,155.5） ~ 109/L. Abdominal B-ultrasound examination showed hepatomegaly existed in 68.9% （ 104/151 ） patients. Intraoperative angiography and surgical exploration showed that 41.1% （62/151） patients were simple inferior vena cava obstruction or stenosis, 15.9% （24/151）were simple hepatic vein obstruction or stenosis and 43.0% （65/151） suffered from the inferior vena cava combined with hepatic vein stenosis or obstruction. Surgically confirmation of the lesions showed that inferior vena cava membrane-like structure combined with thrombosis was in 59.6% （90/151） cases. Conclusions Liver congestion, inferior vena cava congestion and portal hypertension are the main clinical manifestations of Budd-Chiari syndrome. With slightly liver function injury, liver dysfunction of Budd-Chiari syndrome isn＇t parallel with its portal hypertension.