Jain Supriya S, Harahsheh Ashraf S, Lee Simon, Raghuveer Geetha, Dahdah Nagib, Khoury Michael, Portman Michael A, Wehrmann Melissa, Sabati Arash A, Fabi Marianna, Thacker Deepika, Misra Nilanjana, Hicar Mark D, Choueiter Nadine F, Elias Matthew D, Dionne Audrey, Orr William B, Szmuszkovicz Jacqueline R, Tierney Seda Selamet, Garrido-Garcia Luis Martin, Dallaire Frederic, Sundaram Balasubramanian, Prasad Deepa, Harris Tyler H, Braunlin Elizabeth, Cooke Elisa Fernandez, Manlhiot Cedric, Farid Pedrom, McCrindle Brian W
Department of Pediatrics, Division of Cardiology, New York Medical College-Maria Fareri Children's Hospital at Westchester Medical Center, Valhalla, New York, USA.
Division of Cardiology, Department of Pediatrics, Children's National Hospital, George Washington University School of Medicine and Health Sciences, Washington, DC, USA.
Can J Cardiol. 2025 Apr;41(4):740-748. doi: 10.1016/j.cjca.2024.11.027. Epub 2024 Nov 30.
While clinical overlap between Kawasaki disease (KD) and multisystem inflammatory syndrome in children (MIS-C) has been evident, information regarding those presenting with shock has been limited. We sought to determine associations with shock within and between diagnosis groups.
The International KD Registry enrolled contemporaneous patients with either KD or MIS-C from 39 sites in 7 countries from January 1, 2020, to January 1, 2023. Demographics, clinical features and presentation, management, laboratory values, and outcomes were compared between the diagnosis and shock groups.
Shock at presentation was noted for 19 of 672 KD patients (2.8%) and 653 of 1472 MIS-C patients (44%; P < 0.001). Within both groups, patients with shock were significantly more likely to be admitted to the intensive care unit, to receive inotropes, and to have greater laboratory abnormalities indicative of hyperinflammation and organ dysfunction, including abnormal cardiac biomarkers. Patients with KD and shock had a greater maximum coronary artery z score (median +2.62) vs KD patients without shock (+1.36; P < 0.001) and MIS-C patients with shock (+1.45 [vs +1.32 for MIS-C patients without shock]; P < 0.001). They were also more likely to have large coronary artery aneurysms. In contrast, MIS-C patients with shock had lower left ventricular ejection fraction (mean 51.6%) vs MIS-C patients without shock (56.6%; P < 0.001) and KD patients with shock (56.7% [vs 62.8% for KD patients without shock]; P = 0.04).
Although patients with KD presenting with shock are clinically similar to patients with MIS-C, especially those with shock, they have more severe coronary artery involvement, whereas MIS-C patients with shock have lower left ventricular ejection fraction.
虽然川崎病（KD）和儿童多系统炎症综合征（MIS-C）之间的临床重叠很明显，但关于出现休克的患者的信息有限。我们试图确定诊断组内和诊断组之间与休克的关联。
国际川崎病注册研究纳入了2020年1月1日至2023年1月1日期间来自7个国家39个地点的同期KD或MIS-C患者。比较了诊断组和休克组的人口统计学、临床特征和表现、治疗、实验室检查值及预后。
672例KD患者中有19例（2.8%）初诊时出现休克，1472例MIS-C患者中有653例（44%）出现休克（P<0.001）。在两组中，出现休克的患者更有可能入住重症监护病房、接受血管活性药物治疗，并且有更多表明炎症反应过度和器官功能障碍的实验室异常，包括心脏生物标志物异常。KD合并休克的患者冠状动脉最大Z值（中位数+2.62）高于无休克的KD患者（+1.36；P<0.001）以及合并休克的MIS-C患者（+1.45[无休克的MIS-C患者为+1.32]；P<0.001）。他们也更有可能出现大型冠状动脉瘤。相比之下，合并休克的MIS-C患者左心室射血分数较低（平均51.6%），低于无休克的MIS-C患者（56.6%；P<0.001）以及合并休克的KD患者（56.7%[无休克的KD患者为62.8%]；P=0.04）。
虽然出现休克的KD患者在临床上与MIS-C患者相似，尤其是那些出现休克的患者，但他们的冠状动脉受累更严重，而合并休克的MIS-C患者左心室射血分数较低。
