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儿童川崎病休克综合征(Kawasaki disease shock syndrome，KDSS)是川崎病(Kawasaki disease，KD)的一种危重状态，表现为末梢循环灌注障碍和低血压。休克原因主要包括细胞因子失调、心肌功能障碍及血管炎伴持续毛细血管渗漏。与KD比较，KDSS发热时间更长；炎症反应更严重；D-二聚体水平增高更明显；心血管系统异常发生率更高；多器官功能障碍更常见；静脉免疫球蛋白无反应比例及糖皮质激素使用比例更大。KDSS早期临床表现及实验室检查结果不典型，诊断难度较大，容易发生漏诊或误诊。治疗主要包括抗休克及抗炎治疗，以纠正休克、缓解急性期炎症及改善长期预后为目标。KDSS发生及发展与多因素相关。“,”Kawasaki disease shock syndrome (KDSS)is a critical state of Kawasaki disease(KD)characterized by peripheral circulatory perfusion disorder and hypotension.The main causes of shock include cytokine imbalance, myocardial dysfunction and vasculitis with continuous capillary leakage.Compared with KD, KDSS shows longer fever duration, more severe inflammatory reaction, more obvious increase of D-dimer level, higher incidence of cardiovascular system abnormalities and multiple organ dysfunction, and higher proportion of unresponsive IVIG and glucocorticoid use.The early clinical manifestations and laboratory examination results of KDSS are not typical, so it is difficult to make an earlier diagnosis.The therapy of KDSS mainly includes anti-shock therapy and anti-inflammatory therapy may be associated with faster remission of shock and inflammation and better improvement of long-term prognosis.Many factors are related to the occurrence and development of KDSS.