丙种球蛋白无反应性川崎病的治疗研究

Treatment of Kawasaki disease patients with non-responsiveness to high-dose gamma-globulin therapy

目的探讨对大剂量静脉注射丙种球蛋白(IVIG)无反应性川崎病(KD)的临床特点,以及再治疗方案的选择。方法收集2007年1月至2009年12月入院的140例IVIG无反应性的KD患儿,将患儿随机分为4组对照组(单用阿司匹林),IVIG追加治疗组(阿司匹林基础上加2 g.kg-1.d-1IVIG),泼尼松组(阿司匹林基础上加甲泼尼龙20～30 mg.kg-1.d-1)及乌司他丁治疗组(阿司匹林基础上加乌司他丁5 000 U.kg-1),比较各组的临床特点及治疗效果。结果单用阿司匹林治疗的对照组患儿,心脏冠状动脉病变(CAD)并发率明显高于IVIG追加治疗组、泼尼松组及乌司他丁组。且恢复时间较长。IVIG追加治疗组、泼尼松组及乌司他丁组治疗有效率明显高于对照组,但3组之间无统计学差异,且3组患儿CAD并发率相似。结论 IVIG无反应性川崎病较IVIG敏感性川崎病更易发生CAD和严重并发症;对IVIG无反应性川崎病可以用IVIG追加治疗、糖皮质激素或合用乌司他丁,都可以取得较好的疗效。

Objective To investigate the clinical characteristics and treatment plan of high-dose intravenous immunoglobulin（IVIG）non-responsive Kawasaki disease（KD）.Methods A total of 140 KD patients with non-responsiveness to high-dose gamma-globulin therapy hospitalized from January 2007 to December 2009 were included in the study.All the KD patients were divided into 4 groups.The control group took aspirin orally only.The IVIG group were given intravenous immunoglobulin at 2 g·kg-1·d-1.The prednisone group were given intravenous meprednisone at 20-30 mg·kg-1·d-1.The ulinastatin group were given intravenous ulinastatin 5 000 U·kg-1.The clinical characteristics and therapeutic effect were analysed.Results The therapeutic effect rate in the control group was lower than that of the other groups.The incidence rate of coronary artery lesion was higher in the control group than the other groups.But there was no difference between the 3 groups in the effective rate and the incidence rate.Conclusion KD patients with IVIG non-responsiveness are vulnerable to coronary artery lesion and severe complications.Additional IVIG,steroid therapy and ulinastatin therapy may be used for IVIG non-responsive KD patients.