静脉丙种球蛋白无反应性川崎病的治疗及危险因素分析

Retreatment and risk factors of IVIG nonresponsiveness

目的探讨首次丙种球蛋白静脉注射（IVIg）无反应性川崎病（KD）的发生率及危险因素，及其再治疗方法的选择。方法总结2000-2004年北京45家医院KD患儿的临床资料，IVIG无反应性定义为首次IVIG治疗36h后体温仍超过38．5℃。结果1107例患儿纳入研究对象，1092例有急性期治疗资料，1052例（96．3％）接受IVIG治疗，135例对首次IVIG治疗无反应，发生率12．8％（135／1052）。Logistic回归分析发现血沉、GPT、WBC、发病至用IVIG的时间、血浆白蛋白及IVIG治疗剂量，是IVIG无反应性的独立危险因素（P〈0．05）。对IVIG无反应者8例给第2剂2g／kgIVIG，5例热退；114例给1g／kg剂量IVIG治疗，35例（30．7％）热退；11例给400—600mg／kgIVIG，1例（9．1％）热退；2例给糖皮质激素，2例均热退。4种再治疗方法间比较，差异有统计学意义（P=0．015）。第2剂2g／kgIVIG治疗较其它再治疗所需进一步IVIG或激素治疗次数少，体温恢复快。结论约12．8％KD患儿对初次IVIG治疗无反应。血沉、WBC和GPT、血浆白蛋白、IVIG使用方法及起病至用IVIG的时间，是IVIG无反应的独立危险因素。对初次IVIG无反应患儿推荐使用第2剂2g／kg,IVIG，对2次2g／kgIVIG治疗仍无效者可以选用糖皮质激素治疗。

Objective To evaluate the incidence and risk factors of children with refractory Kawasaki disease （KD）. Methods Clinical data of all children with KD in 45 hospitals in Beijing were summarized from 2000 through 2004. Refractory KD was defined as those who remained febrile with a temperature of 〉 38.5℃ 36 hours after initial intravenous immunogiobulin treatment （ IVIG）. Results A total of 1107 patients were included as study subjects. Of them, 1092 patients had data on acute treatment,1052 （96. 3% ） received initial IVIG therapy,135 did not respond to the initial IVIG treatment with an incidence of 12. 8%, Logistic regression revealed that ESR, GPT, WBC, serum albumin, time from onset to IVIG treatment and IVIG dosage were independent risk factors for refractory KD （ P 〈 0. 05 ）. Children with refractory KD were retreated with a second IVIG of 2g/kg in 8 patients with 5 responding （62. 5% ） ,1 g/kg in 114 with 35 responding {30. 7% ） ,and 400 -600 mg/kg in 11 with 1 responding （9. 1% ）. Steriod was used in 2 children with both respon ding （ P = 0. 015）. Conclusion The incidence of refractory KD in Beijing was 12. 8%. ESR,GPT,WBC,serum albumin, the time from onset to IVIG treatment and IVIG dosage are risk factors for refractory KD. Retreatment of children with refractory KD with a 2g/kg IVIG is probably more effective for fever defervescence than a dosage of 1 g/kg or 400 - 600 mg/kg.