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一名幼儿的不完全性和非典型川崎病:对英夫利昔单抗有反应的严重、难治性疾病。

Incomplete and atypical Kawasaki disease in a young infant: severe, recalcitrant disease responsive to infliximab.

作者信息

O'connor Matthew J, Saulsbury Frank T

机构信息

Department of Pediatrics, University of Virginia Health System, Charlottesville, Virginia 22908, USA.

出版信息

Clin Pediatr (Phila). 2007 May;46(4):345-8. doi: 10.1177/0009922806294842.

DOI:10.1177/0009922806294842
PMID:17475994
Abstract

This report describes a 7-week-old infant with incomplete and atypical Kawasaki disease, an acute vasculitis that predominantly affects infants and children. The patient was refractory to 2 doses of intravenous immunoglobulin and to high-dose intravenous methylprednisolone. He became afebrile only after 2 doses of infliximab. His prolonged, recalcitrant course was complicated by the development of peripheral gangrene and giant coronary artery aneurysms. Infants with incomplete and atypical Kawasaki disease are prone to intravenous immunoglobulin treatment failure and are at risk for the development of coronary artery aneurysms. In such patients, we suggest that consideration be given to early aggressive therapy with corticosteroids or infliximab added to intravenous immunoglobulin.

摘要

本报告描述了一名7周大患有不完全性非典型川崎病的婴儿,川崎病是一种主要影响婴幼儿的急性血管炎。该患者对两剂静脉注射免疫球蛋白和高剂量静脉注射甲泼尼龙均无反应。仅在两剂英夫利昔单抗治疗后他才退热。他病程迁延、顽固,并发了外周坏疽和巨大冠状动脉瘤。患有不完全性非典型川崎病的婴儿易于出现静脉注射免疫球蛋白治疗失败,并有发生冠状动脉瘤的风险。对于此类患者,我们建议考虑早期积极使用皮质类固醇进行治疗,或在静脉注射免疫球蛋白基础上加用英夫利昔单抗。

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