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系统性毛细血管渗漏综合征(systemic capillary leak syndrome,SCLS)是指不明原因反复出现由于毛细血管内皮细胞损伤,血管通透性增加而引起毛细血管外漏,大量血浆蛋白渗透到组织间隙,从而出现低蛋白血症、低血容量休克、血液浓缩等临床表现的一组综合征[1]。具体表现为血压下降、全身广泛性水肿、低白蛋白血症,严重时引起肺、心、肾功能衰竭,其他少见并发症包括骨筋膜室综合征、横纹肌溶解等,常伴有异常的单克隆免疫球蛋白病。常死于呼吸、循环衰竭[2]。自1960年Clarkson等[3]首次报道至今约不到150个案例报道[4]。其发病率近年增高,本文就SCLS的临床研究进展进行综述。
Systemic capillary leak syndrome (systemic capillary leak syndrome, SCLS) refers to the unexplained recurrence due to capillary endothelial cell damage, vascular permeability caused by capillary leakage, a large number of plasma protein penetration into the tissue gap, thus A hypoproteinemia, hypovolemic shock, blood concentration and other clinical manifestations of a syndrome [1]. Specific manifestations of decreased blood pressure, generalized systemic edema, hypoalbuminemia, severe pulmonary, heart and renal failure, other rare complications include compartment syndrome, rhabdomyolysis, often accompanied by abnormal single Clone immunoglobulin disease. Often died of breathing, circulatory failure [2]. Less than 150 cases have been reported since Clarkson et al [3] first reported in 1960 [4]. Its incidence in recent years increased, this paper reviews the progress of clinical research of SCLS.