摘要川崎病(Kawasaki disease,KD)是一种急性系统性血管炎性疾病,好发于5岁以内的亚洲儿童,未及时治疗可发展成冠状动脉损害(coronary artery lesion,CAL).目前大剂量静脉注射丙种球蛋白(intravenous immunoglobulin,WIG)联合阿司匹林口服作为KD的标准治疗方案,可使CAL发生率下降至3％ ～5％,但仍有部分患儿对IVIG不敏感,而这类患儿的CAL发生率较高.现有的Kobayashi评分和Egami评分体系等在临床应用的灵敏度不高,因此有必要探索新的指标包括基因及细胞因子等,建立高灵敏度的评分体系预测川崎病患儿IVIG抵抗的发生,以尽早采取其他治疗措施.该文就目前发现的运用于预测川崎病患儿IVIG无反应的预测指标及评分体系进行综述.

abstractsKawasaki disease (KD),an acute systemic vasculitis with a predilection for Asian children,mainly affects infants and children under 5 years of age.They may develop coronary artery lesions(CAL) if not given treatment in time.Presently the standard therapy of Kawasaki disease is high dose intravenous immunoglobulin (IVIG)combined with oral aspirin,which reduces the incidence rates of coronary artery lesions to 3 ％-5％.However,some KD patients are resistant to IVIG therapy and they are at higher risk of developing coronary artery lesions than responders to IVIG therapy.Owing to the low sensibility of Kobayashi and Egami scoring system,exploring new biomarks such as genes and cytokines is necessary.A high sensitive scoring system based on clinical data should be established to predict IVIG nonresponders.Other therapeutic methods can be adopted for IVIG resistance patients.Predictors and scoring system of intravenous irnmunoglobulin resistance to KD are summarized in this article.