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目的分析儿童静脉注射免疫球蛋白(IVIG)不敏感川崎病的临床特点。方法对2005-01—2005-12在重庆医科大学儿童医院住院的233例川崎病的患儿进行病例回顾性分析。结果IVIG不敏感川崎病共31例(占13.3%)。IVIG不敏感川崎病中男女性别比为4.17∶1,明显高于IVIG敏感川崎病1.43∶1(P<0.05)。两组川崎病年龄构成差异无显著性(P>0.05)。IVIG不敏感川崎病中有中度以上冠状动脉扩张或冠状动脉瘤者6例(6/31,19.4%),明显高于IVIG敏感川崎病(5/202,2.4%)(P<0.05)。川崎病患儿发生IVIG耐药的可能危险因素有外周血白细胞、中性粒细胞比例、血红蛋白、C反应蛋白、血沉、血浆白蛋白、乳酸脱氢酶等,分析结果提示血浆白蛋白降低及男性可能与IVIG不敏感川崎病有关,但仅此两项指标尚不能预测IVIG不敏感川崎病的发生。IVIG不敏感川崎病冠脉病变发生危险因素无阳性发现。IVIG不敏感川崎病患儿中14例通过复用IVIG后临床症状得以缓解,仅8例在复用IVIG后症状仍不能控制而加用激素治疗。结论IVIG不敏感川崎病并不少见且较IVIG敏感川崎病更易发生较严重冠状动脉病变,川崎病患儿发生IVIG耐药及IVIG不敏感川崎病冠脉病变发生的危险因素不肯定。复用IVIG及必要时在抗凝基础上加用激素对IVIG不敏感川崎病治疗有较好疗效。

Investigation of IVIG resistant Kawasaki disease

Objective To investigate the prevalence,outcome,risk factors and treatment of IVIG resistant Kawasaki disease.Methods Medical charts of 233 cases with a discharge diagnosis of Kawasaki disease from January 2005 to December 2005 were reviewed retrospectively in Children's Hospital of Chongqing Medical University.A follow-up survey was also given.Results Thirty-one (13.3 percent) of the 233 patients failed to respond to initial treatment with IVIG.The male to female ratio of IVIG resistant Kawasaki disease was 4.17,significantly higher than IVIG responsive Kawasaki disease (1.43) (P<0.05).The age distributions of the 2 groups were not different.The patients who did not respond to IVIG had higher and more severe coronary artery lesions (CAL) compared with IVIG responder (6 of 31 (19.4 percent) versus 5 of 202(2.4 percent)).The following data were obtained and compared between resistance and responder: white blood cell,neutrophil,hemoglobulin,C-reactive protein,erythrocyte sedimentation rate,albumin,and lactate dehydrogenase(LDH).Male patients with decreased albumin may be related with resistance,but they could not be good predictors of resistance.Risk factors of coronary artery leisions with IVIG resistant Kawasaki disease were not found.Additional infusions of IVIG had effects on most IVIG resistant Kawasaki disease (14/31);steroid treatment was given to 8 patients whom additional infusion of IVIG had no effects on.Conclusion IVIG resistant Kawasaki diseases are not rare,and they have higher and more severe coronary artery lesions.The risk factors of IVIG resistance and coronary artery leisions with IVIG resistant Kawasaki disease are not sure.Additional infusion of IVIG and steroid treatment with antiplatelet and anticoagulant medications have good effects on IVIG resistant Kawasaki disease.