Rheumatology Advance Access originally published online on March 1, 2005
Rheumatology 2005 44(8):959-961; doi:10.1093/rheumatology/keh593
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© The Author 2005. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oupjournals.org
EDITORIAL |
Diagnosing Kawasaki syndrome: the need for a new clinical tool
Department of Paediatrics-Rheumatology Unit, University of Firenze, Italy, 1 Division of Rheumatology, Department of Pediatrics, A. I. duPont Hospital for Children, Wilmington, DE and Department of Pediatrics, Thomas Jefferson University, Philadelphia, PA, USA and 2 Department of Paediatrics, University of Firenze, Italy
Correspondence to: G. Simonini, Department of Paediatrics-Rheumatology Unit, Via Pico della Mirandola 24, 50132 Firenze, Italy. E-mail: gabriele.simonini@unifi.it
| The first 150 words of the full text of this article appear below. |
Kawasaki syndrome (KS) is the leading cause of acquired heart disease in children in several parts of the world. It is estimated that up to 3500 children in the US develop KS each year. In the absence of therapy, 1525% will develop coronary artery abnormalities as a result of intense vasculitis [1, 2]. These include aneurysms and ectasia, responsible for the 2% mortality reported in Japanese children with KS before the introduction of intravenous immunoglobulin (IVIG). Smaller aneurysms tend to resolve over a period of 12 yr, but giant aneurysms predispose to risk for myocardial ischaemia and infarction requiring anticoagulation therapy and close follow-up. The timely administration of IVIG has been shown to reduce the incidence of coronary artery disease to about 38% [3] and mortality to less than 0.2% [4]. Rarely, children who subsequently develop coronary artery stenosis will require more aggressive
Diagnosis of atypical/incomplete KS: the clinician's dilemma
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