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Kawasaki Disease (Mucocutaneous...
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PEDIATRICS Vol. 105 No. 6 June 2000, p. e78

ELECTRONIC ARTICLE:
Initial Intravenous Gammaglobulin Treatment Failure in Kawasaki Disease

Received Sep 2, 1999; accepted Jan 11, 2000.

Carol A. Wallace, James W. French, Stuart J. Kahn, and David D. Sherry

From the Department of Pediatrics, University of Washington; and Children's Hospital and Regional Medical Center, Seattle, Washington.

Objectives.  To determine initial intravenous gammaglobulin (IVIG) treatment failures in Kawasaki disease (KD) and to report the outcome of retreatment and our use of pulse intravenous (IV) methylprednisolone and cyclophosphamide in patients with persistent KD.

Study Design.  Retrospective analysis of the treatment and response of children with KD over 3 years.

Results.  Fifty (77%) of 65 patients completely responded to a single treatment with IVIG (2 g/kg). Fifteen patients (23%) required retreatment; 10 patients fully responded but 5 had persistent disease (3 developed coronary aneurysms and 4 developed coronary artery thrombosis). Four of these 5 patients with persistent disease were treated with pulse IV methylprednisolone and 2 were also treated with IV cyclophosphamide. There was no progression of coronary aneurysms and no deaths. No initial patient characteristics predicted IVIG treatment failure or the development of coronary aneurysms.

Conclusion.   Nearly 23% of patients with KD may require retreatment and 8% may develop coronary aneurysm. Additional antiinflammatory therapy, such as IV methylprednisolone and IV cyclophosphamide, may be helpful in treating persistent KD.  Key words:  corticosteroids, vasculitis, Kawasaki disease, heart disease, gammaglobulin therapy, cyclophosphamide.


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