Abstract
Background In the therapy of Kawasaki disease (K.D.), We sometimes fail to prevent the coronary artery aneurysm formation despite high dose immunoglobulin (Iγ -Gl) administration. I have already reported that polymorphonuclear leukocyte elastase(PMN-elastase) plays an important role in provoking the angitis in the acute phase of K.D. and that by giving a PMN-elastase inhibitor, urinastatin (UTI) in the early stage of K.D., it is able to decrease vascular injury and to prevent aneurysm formation. Aim The aim of this study is to evaluate the efficacy of new protocol of UTI therapy for the acute phase of K.D. Subjects The subjects were consecutive 44 cases with K.D. fulfilling the clinical criteria for diagnosis within 7 days of onset of illness, that had not been treated with corticosteroid, Aspirin or Iγ-Gl. Methods All cases were administered 50,000U/1hr d.I.v. x 6 per day of UTI daily until the child became afebril and CRP was improved less than 1.0mg/dl. Iγ-Gl was additionally administered only to the case that showed recurrence of fever and other symptoms and reincrease of CRP and WBC during UTI therapy. Results Thirty eight cases were able to be therapied by UTI alone, and six cases needed UTI and one or two times of additional 1.0g/kg of Iγ-Gl. In only three of the six cases, echocardiographic abnormalities of coronary arteries were detected. Two cases showed mild transient dilatation and in another case, medium sized aneurysms developed. The other 41 cases (93%) showed no coronary artery involvement. Conclusion The new UTI therapy proved to be a valuable and useful alternative for the acute phase of K.D.
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Nakano, M. Evaluation of New Urinastatin Therapy for the Acute Phase of Kawasaki Disease. Pediatr Res 53, 164 (2003). https://doi.org/10.1203/00006450-200301000-00065
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DOI: https://doi.org/10.1203/00006450-200301000-00065