促皮质素剂量递增法治疗婴儿痉挛的临床研究

Clinical study on treatment of infantile spasms with incremental corticotrophin therapy

目的总结促皮质素(ACTH)剂量递增法治疗婴儿痉挛(IS)的疗效和不良反应,提供新的临床治疗方案。方法收集2016年1月至2018年1月在上海交通大学医学院附属上海儿童医学中心神经内科住院接受ACTH治疗的40例IS患儿的临床资料,进行回顾性研究分析。所有患儿采用静脉滴注ACTH剂量递增治疗方案:起始剂量12.5 U/d,观察3 d,如痉挛发作不消失则加量至25.0 U/d再用3 d,若痉挛仍得不到完全控制则加量至40.0 U/d继续应用,总疗程不超过2周;每个剂量阶段痉挛发作消失者或疗程达到2周时改为泼尼松2 mg/(kg·d)口服,2个月内逐渐减量至停药。患儿治疗前后均进行脑电图检查。结果40例IS患儿经ACTH剂量递增方案治疗,痉挛发作消失率总体为40.0%(16/40例),12.5 U/d治疗阶段为7.5%(3/40例),25.0 U/d治疗阶段为16.2%(6/37例),40.0 U/d治疗阶段为22.6%(7/31例);脑电图高度失律消失率总体为60.0%(24/40例),各剂量阶段分别为5.0%(2/40例)、10.8%(4/37例)和58.1%(18/31例)。37.5%(15/40例)的患儿出现轻度不良反应,主要为呼吸道感染。结论ACTH剂量递增方案治疗IS的短期疗效肯定,不良反应发生率较低。

Objective To summarize the efficacy and adverse reactions of incremental corticotrophin (ACTH) therapy in the treatment of infantile spasms (IS), and to provide new clinical treatment options. Methods The clinical data of 40 children with IS who were hospitalized in the Department of Neurology, Shanghai Children′s Medical Center, Shanghai Jiaotong University School of Medicine, treated with ACTH from January 2016 to January 2018 were collected and retrospectively analyzed.All the children were treated with intravenous infusion of ACTH with an initial dose 12.5 U/d for 3 days.If the spasms did not disappear, dosage of ACTH increased to 25.0 U/d for another 3 days.If the spasms could not yet be fully controlled, the dosage increased to 40.0 U/d, and the total course of treatment did not exceed 2 weeks.If the spasms disappeared at each dose stage or the course of treatment reached to 2 weeks, ACTH would be changed to Prednisone 2 mg/(kg·d) orally, which gradually decreased in 2 months.All children underwent electroencephalogram examination before and after treatment. Results Forty patients with IS were treated with ACTH increa-sing therapy.The disappearance rate of spasms was 40.0%(16/40 cases) totally, with 7.5%(3/40 cases) at the dosage phase of 12.5 U/d, 16.2%(6/37 cases) at the dosage stage of 25.0 U/d, and 22.6%(7/31 cases) at the dosage of 40.0 U/d.The disappearance rate of hypsarrhythmia on electroencephalogram was 60.0%(24/40 cases)generally, and 5.0%(2/40 cases), 10.8%(4/37 cases), 58.1%(18/31 cases), respectively at above different dosage phases, while 37.5%(15/40 cases) of the children had mild adverse reactions, mostly respiratory infections. Conclusions The short-term efficacy of the ACTH incremental therapy in the treatment of IS is positive, and the incidence of adverse reactions is low.