Skip to main content
main-content

27.06.2017 | Review | Ausgabe 8/2017 Open Access

European Journal of Pediatrics 8/2017

Dissecting Kawasaki disease: a state-of-the-art review

Zeitschrift:
European Journal of Pediatrics > Ausgabe 8/2017
Autoren:
S. M. Dietz, D. van Stijn, D. Burgner, M. Levin, I. M. Kuipers, B. A. Hutten, T. W. Kuijpers
Wichtige Hinweise
Communicated by Peter de Winter

Abstract

Kawasaki disease (KD) is a pediatric vasculitis with coronary artery aneurysms (CAA) as its main complication. The diagnosis is based on the presence of persistent fever and clinical features including exanthema, lymphadenopathy, conjunctival injection, and changes to the mucosae and extremities. Although the etiology remains unknown, the current consensus is that it is likely caused by an (infectious) trigger initiating an abnormal immune response in genetically predisposed children. Treatment consists of high dose intravenous immunoglobulin (IVIG) and is directed at preventing the development of CAA. Unfortunately, 10–20% of all patients fail to respond to IVIG and these children need additional anti-inflammatory treatment. Coronary artery lesions are diagnosed by echocardiography in the acute and subacute phases. Both absolute arterial diameters and z-scores, adjusted for height and weight, are used as criteria for CAA. Close monitoring of CAA is important as ischemic symptoms or myocardial infarction due to thrombosis or stenosis can occur. These complications are most likely to arise in the largest, so-called giant CAA. Apart from the presence of CAA, it is unclear whether KD causes an increased cardiovascular risk due to the vasculitis itself.
Conclusion: Many aspects of KD remain unknown, although there is growing knowledge on the etiology, treatment, and development and classification of CAA. Since children with previous KD are entering adulthood, long-term follow-up is increasingly important.
What is known:
• Kawasaki disease (KD) is a pediatric vasculitis with coronary artery damage as its main complication.
• Although KD approaches its 50th birthday since its first description, many aspects of the disease remain poorly understood.
What is new:
• In recent years, multiple genetic candidate pathways involved in KD have been identified, with recently promising information about the ITPKC pathway.
• As increasing numbers of KD patients are reaching adulthood, increasing information is available about the long-term consequences of coronary artery damage and broader cardiovascular risk.

Unsere Produktempfehlungen

e.Med Interdisziplinär

Kombi-Abonnement

Für Ihren Erfolg in Klinik und Praxis - Die beste Hilfe in Ihrem Arbeitsalltag als Mediziner

Mit e.Med Interdisziplinär erhalten Sie Zugang zu allen CME-Fortbildungen und Fachzeitschriften auf SpringerMedizin.de.

e.Med Pädiatrie

Kombi-Abonnement

Mit e.Med Pädiatrie erhalten Sie Zugang zu CME-Fortbildungen des Fachgebietes Pädiatrie, den Premium-Inhalten der pädiatrischen Fachzeitschriften, inklusive einer gedruckten Pädiatrie-Zeitschrift Ihrer Wahl.

Literatur
Über diesen Artikel

Weitere Artikel der Ausgabe 8/2017

European Journal of Pediatrics 8/2017 Zur Ausgabe

Neu im Fachgebiet Pädiatrie

Mail Icon II Newsletter

Bestellen Sie unseren kostenlosen Newsletter Update Pädiatrie und bleiben Sie gut informiert – ganz bequem per eMail.

Bildnachweise