Initial intravenous gammaglobulin treatment failure in Kawasaki disease

Initial intravenous gammaglobulin treatment failure in Kawasaki disease
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DOI:
10.1542/peds.105.6.e78
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发表时间:
2000-06-01
期刊:
影响因子:
8
通讯作者:
Sherry, DD
Sherry, DD
中科院分区:
医学2区
文献类型:
--
作者:
Wallace, CA;French, JW;Sherry, DD

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目标。目的:确定川崎病(KD)首次静脉注射丙种球蛋白(IVIG)治疗失败的情况,并报告再治疗的结果以及我们对持续性KD患者使用脉冲静脉注射(IV)甲基强的松龙和环磷酰胺的结果。对3年以上川崎病患儿的治疗及疗效进行了回顾性分析。65名患者中有50名(77%)对静脉注射丙种球蛋白(2g/kg)的单一治疗完全有效。15名患者(23%)需要再次治疗;10名患者完全缓解,但5名患者有持续性疾病(3名患者发展为冠状动脉动脉瘤,4名患者发展为冠状动脉血栓)。5例顽固性疾病患者中4例接受甲基强的松龙冲击治疗,2例同时接受环磷酰胺冲击治疗。没有冠状动脉动脉瘤的进展,也没有死亡。患者的初始特征不能预测IVIG治疗失败或冠状动脉动脉瘤的发展。近23%的KD患者可能需要再次治疗,8%的患者可能会发展为冠状动脉瘤。附加的抗炎治疗,如静脉注射甲基强的松龙和静脉注射环磷酰胺,可能对治疗持续性KD有帮助。
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.