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Kawasaki disease


Médecine thérapeutique / Pédiatrie. Volume 14, Number 5-6, 355-61, Septembre-Décembre 2011, Dossier

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Author(s) : Jean-Louis Stéphan, Aurélie Desbrée, Lionel Berthomieu, Claire Gay

Summary : Kawasaki syndrome is an acute, self-limited vasculitis that occurs in children of all ages. The diagnostic may be challenging in children with an incomplete or atypical form of Kawasaki disease (KD) especially in infants younger than 1 year. They are the group most likely to develop coronary artery aneurysms. Intravenous immunoglobulin (IVIG) continues to offer a remarkable combination of efficacy and safety for the treatment of KD. Therapy within the first 10 days of illness reduces the incidence of coronary artery aneurysms by more than 70%. Persistent or recurrent fever after IVIG therapy usually indicates ongoing vasculitis, with increased risk of developing coronary artery aneurysms. While not established by prospective studies, a number of clinical reports support the treatment of children who have not responded to IVIG and still have active KD with one to three daily doses of IVMP (30 mg/kg). Other immunosuppressive therapies, including infliximab, may also be beneficial, although randomised clinical trials are lacking. A better understanding of the aetiology of KD would be helpful in targeting new therapeutic strategies, along with the development of a diagnostic test.

Keywords : Kawasaki syndrome, vasculitis, coronary artery aneurysm

 

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