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Kawasaki Disease in Germany: A Prospective, Population-based Study Adjusted for Underreporting

Jakob, André MD*; Whelan, Jane MD, PhD, MPH; Kordecki, Matthaeus*; Berner, Reinhard MD; Stiller, Brigitte MD*; Arnold, Raoul MD§; von Kries, Rudiger MD; Neumann, Elena*; Roubinis, Nicholas MSc; Robert, Mirna MD, MPH; Grohmann, Jochen MD*; Höhn, René MD*; Hufnagel, Markus MD

The Pediatric Infectious Disease Journal: February 2016 - Volume 35 - Issue 2 - p 129–134
doi: 10.1097/INF.0000000000000953
Original Studies

Background: National estimates of Kawasaki disease (KD) incidence often do not include incomplete cases (diagnosed based on only laboratory or echocardiographic criteria), and/or they rely on retrospective case reports and data registries where underreporting is known to be a problem.

Methods: We conducted a prospective nationwide KD surveillance study in children younger than 5 years through the hospital-based German Pediatric Surveillance Unit (ESPED). We accounted for underreporting through applying capture–recapture methodology in 2 federal states using hospital discharge records with KD International Statistical Classification of Diseases and Related Health Problems 10th revision code (ie, M30.3). KD diagnosis (complete and incomplete) was established according to the American Heart Association criteria, 2004.

Results: Incidence of KD, corrected for underreporting, was 7.2 of 100,000 in children younger than 5 years in Germany. Underreporting to ESPED was estimated at 37%–44%. Overall, 315 validated KD cases were reported. Of the 64 (20%) incomplete cases, 58% (37/64) were detected through echocardiographic findings and 42% (27/64) through laboratory criteria alone. Incomplete cases were younger than complete cases (1.2 vs. 2.0 years, P = 0.0001) and had more coronary aneurysms (43% vs. 11%, P = 0.0001).

Conclusions: A substantial number of incomplete KD cases were diagnosed based on the laboratory and echocardiographic criteria only. This was particularly the case in relation to infants younger than 1 year—an age group known to have an increased risk of developing coronary aneurysms. In addition, we found a high rate of underreporting to national Pediatric Surveillance Units. We suggest that improved surveillance and development of better diagnostic tests remain a high priority.

From the *Department of Congenital Heart Defects, Heart Center University of Freiburg, Freiburg, Germany; Novartis Pharma BV, Amsterdam, The Netherlands; Clinic and Polyclinic for Pediatrics and Adolescent Medicine, University Children’s Hospital, Technische Universität Dresden, Dresden, Germany; §Department of Pediatric Cardiology, University Hospital Heidelberg, Heidelberg, Germany; Division of Epidemiology, Institute of Social Pediatrics and Adolescent Medicine, Ludwig-Maximilian’s University Munich, München, Germany; and Section of Pediatric Infectious Diseases and Rheumatology, Center for Pediatrics and Adolescent Medicine, University Medical Center Freiburg, Freiburg, Germany.

Accepted for publication September 23, 2015.

Supported by a grant from Novartis Vaccines (to A.J.). J.W., N.R. and M.R. are employees of Novartis group companies. The other authors have no funding or conflicts of interest to disclose.

Address for correspondence: André Jakob, MD, Universitäts Herzzentrum Freiburg-Bad Krozingen, Klinik für Angeborene Herzfehler, Mathildenstraße 1, 79106 Freiburg, Germany. E-mail:

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