山西省布加综合征169例临床分析

目的分析布加综合征（Budd—Chiari syndrome，BCS）的临床特点，为临床诊断提供经验。方法整理山西省169例布加综合征患者的首次入院资料，对主要临床表现、肝功能、超声、影像学检查结果等进行统计描述和统计推断。结果山西省169例BCS患者病变类型以下腔静脉型为主（59．76％），混合型次之（29．59％），肝静脉型最少（10．65％）。临床表现以乏力、纳差、腹胀、腹痛为就诊主要症状，以下肢肿胀、下肢静脉曲张、胸腹壁静脉曲张、肝脾大、腹水为就诊主要体征，各型中出现频率不同。肝静脉型与非肝静脉型组间比较ALT、AST、GGT差异有统计学意义。临床漏诊、误诊率较高（54．44％），其中肝静脉型最多（83．33％）。结论布加综合征临床表现多样，漏诊、误诊现象常见，诊断除参考临床表现外，应结合肝功能、彩超、血管造影结果；肝穿刺活检具有确诊价值，尤其对于肝静脉型。

Analysis of 169 cases of Budd-Chiari syndrome in Shanxi province

Objective To analyze the clinical features of Budd-Chiari syndrome （BCS） for providing the experiences of clinical diagnosis. Methods The clinical data of 169 cases of BCS from Shanxi province were analyzed from the respects of the main clinical man- ifestation,liver function, ultrasound and imaging results. Results The incidence of inferior vena cava type was the highest （59.76%） ,followed by mixed type（29.59% ） and hepatic vein type （ 10.65% ）. The main clinical manifestations included fatigue, anorexia, abdominal distension, abdominal pain, lower limb edema,lower limbs varicosity, chest and belly wall varicosity, hepatospleno- megaly and ascites. The frequencies of these clinical features were different in different types of BCS. There was statistically significant difference in ALT,AST,GGT between hepatic vein type and non-hepatic vein type. The rate of misdiagnosis and missed diagnosis was high （54.44%）, and hepatic vein type accounted for 83.33%. Conclusion The clinical manifestation of BCS is varied. The misdiagnosis and missed diagnosis are common, so the correct diagnosis should comprehensively consider the results of liver function, ultrasound, angiography and clinical manifestations. Liver biopsy can confirm the disease, especially for hepatic vein type.