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Volume 48, Number 1, February 2015

Clinical manifestations of Kawasaki disease shock syndrome: A case–control study 

Pei-Shin Chen, Hsin Chi, Fu-Yuan Huang, Chun-Chih Peng, Ming-Ren Chen, Nan-Chang Chiu

Received: December 6, 2012    Revised: March 15, 2013    Accepted: June 24, 2013   


Corresponding author:

* Corresponding author. Department of Pediatrics, Mackay Memorial Hospital, 92, Section 2, Chung-Shan North Road, Taipei, Taiwan.
E-mail address: (N.-C. Chiu). 


Background and purpose: 

Kawasaki disease shock syndrome (KDSS) is a severe condition related to Kawasaki disease (KD), and sometimes it is difficult to diagnose. This is a case–control study to ascertain the clinical presentations, risk factors, and clinical outcomes of children who had KDSS. 



Children who were hospitalized during 2001–2011 with the diagnosis of KD combined with hypotension, sepsis, or shock were retrospectively reviewed and were defined as case patients. For each case patient, three season-matched patients diagnosed as having KD with normal blood pressure were identified to serve as control patients. Demographic characteristics, clinical presentations, laboratory features, therapies, and outcomes were analyzed. 



Nine KDSS patients and 27 control patients were identified. The average age of patients with KDSS was 3.2 ± 3.2 years. Compared with controls, KDSS patients were less likely to have a diagnosis of KD at admission (22.2% vs. 66.7%) and had a higher risk of coronary artery dilatation (77.8% vs. 11.1%). Risk factors for KDSS included higher neutrophil counts and proportions of bands, higher C-reactive protein (CRP), and lower platelet counts. All case patients received aspirin therapy; eight patients received intravenous immunoglobulin therapy, with two receiving more than one course. Seven KDSS patients required fluid resuscitation, and eight patients required vasoactive infusions. 



Patients with KDSS may have uneven clinical course and may be misdiagnosed in the beginning. They may have more prominent inflammatory markers in the early phase and higher risk of coronary artery dilatation. 


Key words:

Children, Kawasaki disease, Kawasaki disease shock syndrome, Risk factors