布加综合征患者的临床特点分析

目的探讨布加综合征(Budd-Chiari syndrome,BCS)的临床特征特点,为临床治疗提供参考依据.方法收集2007年1月至2016年8月28例BCS患者,分析患者的一般资料、临床表现、辅助检查、误诊情况及治疗结局.结果BCS好发于青年,男女发病率大约1.1∶1,临床常见表现为腹胀(57.1%)、消化道出血(28.6%)、腹痛(28.6%)、肝脾肿大(46.4%及71.4%)、胸腹壁静脉曲张(42.9%)、腹腔积液(28.6%)及双下肢水肿(35.7%).肝功能损害多为轻度.彩色多普勒及磁共振血管成像(MRA)诊断符合率为84.6%及90%,19例患者行介入治疗,绝大部分患者预后良好(92.9%),2例患者预后差.结论BCS临床症状复杂,易误诊,彩色多普勒及MRA为重要的影像学检查,介入治疗为首选治疗方法.

Objective To explore the clinical features of Budd-Chiari Syndrome(BCS),improve the diagnosis accuracy and treatment level and reduce the mis-diagnosis rate. Methods A retrospective analysis including general information,clinical manifestations,auxiliary examination, misdiagnosis and therapeutics was performed in 28 patients with Budd-Chiari Syndrome diagnosed in the WenZhou Central Hospital and the First Affiliated hospital of Wenzhou Medical University from 2007 to 2016. Results BCS occurred more frequently in young adults and the male to female ratio was 1.1∶1. The common clinical manifestations were ventosity(57.1%),hemorrhage of digestive tract(28.6%),abdominal pain(28.6%), hepatauxe(46.4%),splenomegaly(71.4%),thoracic abdominal varicose veins(42.9%),seroperitoneum(28.6%)and lower extremity edema (35.7%). Liver function damage was minor. The diagnostic accordance rate of color Doppler and magnetic resonance angiography(MRA)were 84.6% and 90%. 19 cases accepted interventional therapy,and prognoses were fine(92.9%),2 patients with poor prognoses. Conclusion The clinical features of BCS are complex,which often causes misdiagnosing decision. Color Doppler and MRA play an important role in the imaging tests. The interventional therapy is the preferred treatment.