不同IVIG治疗方案对川崎病冠状动脉病变影响的多中心回顾性研究

Multi-center retrospective research of different intravenous immunoglobulin in treatment coronary lesion in Kawasaki disease

目的探讨静脉丙种球蛋白(IVIG)的不同治疗方案对川崎病(KD)患儿发生冠状动脉病变(CAL)的影响,提出最佳的IVIG治疗建议方案。方法回顾性分析1998—2007年上海地区住院KD患儿1682例的临床资料,男1064例,女618例;发病年龄0.1～18.8岁,平均(2.57±2.33)岁。其中1533例(91.1%)使用IVIG治疗,方案包括①1g/kg×1次;②2g/kg×1次;③0.4～0.5g/kg×5次;④1g/kg×2次;⑤2g/kg×2次;⑥不规则治疗。IVIG治疗的时间窗包括:①病程1～4d;②病程5～10d;③病程>10d。结果在1533例IVIG治疗的KD患儿中,CAL总发生率为20.74%。与此相比较,CAL发生率明显降低者如下:①在病程5～10d应用IVIG治疗1g/kg×2次的KD患儿,其CAL发生率为12.06%;②病程5～10d应用1g/kg×1次者CAL发生率为15.00%;③病程1～4d应用1g/kg×1次者CAL发生率为16.28%;④病程5～10d应用2g/kg×1次者CAL发生率为16.71%。相反,以下几种情况CAL发生率明显高于CAL总发生率:①在病程10d以上应用IVIG者;②使用IVIG2g/kg×2次(因使用1次后效果不佳而再次使用);③其他不规律使用IVIG者。结论在IVIG治疗KD、减少CAL方面,以病程5～10d使用IVIG1g/kg×2次效果最佳;但考虑经济因素和大剂量IVIG潜在的风险,在病程10d以内使用IVIG1g/kg×1次亦是不错的选择。

Objective To evaluate the impacts of different regimens of intravenous immunoglobulin （IVIG） on the coronary artery lesion （CAL） in children with Kawasaki disease （KD）. Methods 1 682 inpatients of KD from 1998 through 2007 in Shanghai were recruited with 1 064 males and 618 females, aged 2.57 ± 2.33 years （0.1 - 18.8 years）. In total cases, there were 1 533 cases （91.1%） with IVIG. There were six different regimens of IVIG, which were 1 g/kg, once; 2 g/kg, once; 0.4- 0.5 g/kg, five times; 1 g/kg, twice; 2 g/kg, twice; and others. The therapeutic windows were within 4 days, from the fifth day to the tenth day, and after 10 days, respectively. Results In a total of 1 533 cases with IVIG, the incidence of CAL was 20.74%. Comparatively, the incidence was lower in patients with following regimens of IVIG： （1） IVIG 1 g/kg twice from the fifth day to the tenth day （the ineidence of CAL was 12.06%） ; （2） IVIG 1 g/kg once from the fifth day to the tenth day （the incidence of CAL was 15.00%） ; （3） IVIG 1 g/kg once within 4 days （the incidence of CAL was 16.28%） ; （4） IVIG 2 g/kg once from the fifth day to the tenth day （The incidence of CAL was 16.71%）. Conversely, the incidence of CAL was higher in patients with the following regimens of IVIG： （1） IVIG after 10 days; （2） IVIG 2 g/kg twice （reused for unfavorable effect）; （3） IGIV others. Conclusions The regimen of IVIG 1 g/kg twice from the fifth day to the tenth day is recommended in children with KD. Taken into consideration of the cost-eflectiveness and risks of huge dosage of IVIG, IV1G 1 g/kg once within 10 days should be an alternative choice.