目的　探讨川崎病休克综合征（KDSS）的临床特点。方法　回顾分析2014年6月至2017年10月期间住院诊 断为KDSS患儿的临床资料，并以同一时期确诊为川崎病（KD）但血流动力学稳定的患儿作为对照组，分析比较两组患儿 的临床特点。结果　与119例KD患儿相比，21例KDSS患儿的颈部淋巴结肿大及不完全KD发生率较高，中性粒细胞比例、 C反应蛋白、D-二聚体、 N末端脑钠肽前体水平较高，血小板及血清白蛋白水平较低，易发生首剂丙种球蛋白无反应及冠 状动脉损害，总热程及住院时间较KD患儿明显延长，差异均有统计学意义（P<0.05）。 结论　KDSS患儿具有更强烈的炎 症反应，存在更明显的高凝状态，易出现丙种球蛋白抵抗。

Objective　To explore the clinical characteristics of Kawasaki disease shock syndrome (KDSS). Method The clinical data of hospitalized children diagnosed with KDSS between June 2014 and October 2017 were retrospectively analyzed. The children diagnosed with Kawasaki disease (KD) at the same time with stable hemodynamics were selected as controls. The clinical characteristics of the two groups of children were analyzed and compared. Results　Compared with 119 KD children, 21 KDSS children had higher percentage of cervical lymph node enlargement and incomplete KD, higher levels of neutrophils, C-reactive protein, D-dimer and N-terminal brain natriuretic peptide precursor, lower platelet count and lower serum albumin level; children with KDSS were more likely to have no response to the first dose of gamma globulin, to suffer from coronary artery damage and to have longer total fever course and hospitalization time than children with KD. The differences were statistically significant (P<0.05). Conclusion　Children with KDSS have more intense inflammatory reaction, more obvious hypercoagulability, and are prone to gamma globulin resistance