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European Journal of Pediatrics

, Volume 176, Issue 8, pp 995–1009

First Online: 27 June 2017Received: 24 February 2017Revised: 11 May 2017Accepted: 15 May 2017


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.

KeywordsKawasaki disease Coronary artery aneurysms Intravenous immunoglobulins Genetics AbbreviationsABCC4ATP-binding cassette, subfamily C, member 4

AHAAmerican Heart Association


CAACoronary artery aneurysms

CABGCoronary artery bypass grafting

CIMTCarotid intima-media thickness

cMRICardiac MRI

DCDendritic cells

GWASGenome-wide association study

HRQOLHealth-related quality of life

ITPKCInositol-triphosphate 3-kinase

IVIGIntravenous immunoglobulins

JSCJapanese Circulation Society

KDKawasaki disease

NFATNuclear factor of activated T cells

PCAPercutaneous coronary intervention

SNPSingle nucleotide polymorphism

SPEStreptococcal pyrogenic exotoxin

TGF-βTranscription growth factor beta

TIE-2Angiopoietin receptor

TSST-1Toxic shock syndrome toxin-1

VEGFVascular endothelial growth factor

This Review refers to the article http:-dx.doi.org-10.1007-s00431-017-2943-7.

Communicated by Peter de Winter

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Author: S. M. Dietz - D. van Stijn - D. Burgner - M. Levin - I. M. Kuipers - B. A. Hutten - T. W. Kuijpers

Source: https://link.springer.com/


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