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[川崎病的医学治疗现状与新挑战]

[Present situation and new challenges for medical treatment of Kawasaki disease].

作者信息

Ikeda Kazuyuki, Hamaoka Kenji

出版信息

Nihon Rinsho. 2014 Sep;72(9):1523-9.

Abstract

To predict resistance to intravenous immunoglobulin (MIG) treatment in patients with severe Kawasaki disease (KD), Gunma, Kurume, and Osaka risk scores were established. Using these scores, prospective randomised trials were performed, and addition of predni- solone or intravenous methylprednisolone -pulse to the standard regimen of IVIG improves coronary artery outcomes in severe KD. Adding the evidences of new and existing therapies, such as infliximab, cyclosporin A, ulinastatin, and plasma exchange, the clinical guideline for medical treatment of the acute phase of KD was revised in 2012. Additional large cohort studies are needed to clarify the applicability of intensive initial treatment. Challenges for the future are: 1) clarification of etiology and establishment of specific therapy, 2) establishment of personalized therapy using genetic markers related to the severity of KD.

摘要

为预测重症川崎病(KD)患者对静脉注射免疫球蛋白(IVIG)治疗的耐药性,制定了群马、久留米和大阪风险评分。利用这些评分进行了前瞻性随机试验,在IVIG标准治疗方案中加用泼尼松龙或静脉注射甲泼尼龙冲击治疗可改善重症KD的冠状动脉结局。结合英夫利昔单抗、环孢素A、乌司他丁和血浆置换等新的和现有的治疗证据,2012年修订了KD急性期的临床治疗指南。需要更多大型队列研究来阐明强化初始治疗的适用性。未来的挑战包括:1)明确病因并建立特异性治疗方法;2)利用与KD严重程度相关的基因标志物建立个性化治疗方案。

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