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Five Years of Kawasaki Disease in the Netherlands: A National Surveillance Study

Tacke, Carline E. MD*; Breunis, Willemijn B. MD, PhD*; Pereira, Rob Rodrigues MD, PhD; Breur, Johannes M. MD, PhD; Kuipers, Irene M. MD, PhD*; Kuijpers, Taco W. MD PhD*

The Pediatric Infectious Disease Journal: August 2014 - Volume 33 - Issue 8 - p 793–797
doi: 10.1097/INF.0000000000000271
Original Studies

Background: The aim of this study was to evaluate the incidence, disease presentation, treatment and cardiac outcome of Kawasaki disease (KD) in The Netherlands.

Methods: The national Dutch Pediatric Surveillance Unit was used to prospectively register new KD cases from 2008 through 2012. Questionnaires were sent to pediatricians to obtain clinical information.

Results: Nationwide 341 cases were reported during the 5-year study period, of which 319 questionnaires (93.0%) were returned. The mean incidence of KD was estimated to be 5.8/100,000 children <5 years of age. The median age at disease onset was 2.4 years (range 0.1–14.6 years) and 79.2% of cases were <5 years of age. The male-to-female ratio was 1.5 to 1. Incomplete KD was diagnosed in 22.3% of cases and these cases were significantly younger than complete cases [median: 1.1 (0.1–13.7) vs. 2.8 (0.2–14.6) years, P < 0.001]. In total, 308 patients (96.6%) received intravenous immunoglobulins (IVIG). Retreatment with IVIG was given in 71 (23.1%) and additional steroid treatment in 17 patients (5.5%). During the acute phase, coronary artery aneurysms developed in 43 cases (13.5%). Multivariate logistic regression analysis showed that male gender, delay of treatment (>10 days) and IVIG retreatment were independent risk factors for coronary artery aneurysms development.

Conclusions: This prospective study of KD in The Netherlands revealed a mean annual incidence of 5.8/100,000 children <5 years of age. Clinicians should consider the diagnosis of KD in young (male) children with persistent inexplicable fever to start IVIG treatment within 10 days to prevent development of coronary artery aneurysms.

Supplemental Digital Content is available in the text.

From the *Emma Children’s Hospital, Academic Medical Center, Amsterdam; TNO, Division of Healthy Living, Leiden; and Department of Paediatric Cardiology, Wilhelmina Children’s Hospital, University Medical Center, Utrecht, The Netherlands.

Accepted for publication January 10, 2014.

This study was sponsored by the Stinafo Foundation (The Hague, The Netherlands), which had no role in the study design, data collection and analysis, writing of the report or the decision to submit the report for publication. The authors have no other funding or conflict of interest to disclose.

Supplemental digital content is available for this article. Direct URL citations appear in the printed text and are provided in the HTML and PDF versions of this article on the journal’s website (

Address for correspondence: Carline E. Tacke, MD, Emma Children’s Hospital, Academic Medical Center, Meibergdreef 9 (H7-270), 1105 AZ Amsterdam, The Netherlands. E-mail:

© 2014 by Lippincott Williams & Wilkins, Inc.