摘要目的:观察促皮质素(ACTH)治疗儿童频复发型肾病综合征(FRNS)的疗效和不良反应，探讨ACTH在儿童FRNS中的可行性治疗方案。方法:选择2017年11月至2018年6月在新乡医学院第一附属医院儿科接受ACTH治疗的32例FRNS患儿，所有患儿行连续ACTH 3～8个疗程治疗方案(每天激素用量≤0.5 mg/kg时予补充0.4 U/kg ACTH，连续应用3 d后激素减量至1.25～5.00 mg，连续应用3 d为1个疗程，至激素减停后继续应用ACTH治疗2个疗程)，记录ACTH治疗后随访期间内复发次数、泼尼松维持剂量、免疫抑制剂使用状况、晨8时血清皮质醇等相关实验指标及不良反应，随访观察8～15个月。结果:32例患儿中，发病年龄(53.47±25.81)个月，病程(35.25±23.87)个月，激素顺利减停无复发22例(68.75%)，激素减停后复发7例(21.87%)，激素未减停复发3例(9.37%)。与ACTH治疗前比较，泼尼松用量明显减少[(0.08±0.14) mg/(kg·d)比(0.23±0.23) mg/(kg·d) ]，差异有统计学意义( t＝3.661， P<0.05)；免疫抑制剂应用例数明显下降[42.38%(12/32例)比58.62%(17/32例)， χ2＝ 14.500， P<0.05]；晨8时血清皮质醇水平升高[(11.78±4.64) μg/dL比(4.42±3.13) μg/dL，差异有统计学意义( t＝7.340， P<0.05)。随访期间效果稳定，其中2例出现1次全身荨麻疹，1例输注后出现头痛。 结论:ACTH治疗儿童FRNS安全有效，且不良反应发生少，有一定的临床意义。

abstractsObjective:To observe the efficacy and adverse reactions of adrenocorticotropic hormone (ACTH) in the treatment of recurrent frequently relapsing nephrotic syndrome (FRNS), and explore the feasibility of treatment of ACTH in children.Methods:From November 2017 to June 2018, in the First Affiliated Hospital of Xinxiang Medical University of a total of 32 cases of FRNS ACTH therapy were all the role of ACTH consecutive 3-8 courses of treatment (when the dosage of prednisone was less than or equal to 0.5 mg/kg, 0.4 U/kg ACTH should be used every day.After 3 days of continuous application, the dosage of Prednisone should be reduced to 1.25-5.00 mg.ACTH was used for 3 days as a course of treatment, and continued to use ACTH for 2 courses until corticosteroid stopped). The number of recurrence, Prednisone maintenance dose, immunosuppressive use, serum cortisol and other relevant experimental indicators and adverse reactions were recorded during the follow-up period after ACTH treatment, and were followed up for 8-15 months.Results:Among the 32 children, the onset age (53.47±25.81) months, the course of disease (35.25±23.87) months, 22 patients (68.75%) had no recurrence after corticosteroid withdrawal, 7 patients (21.87%) had recurrence after corticosteroid withdrawal, and 3 patients (9.37%) had recurrence during corticosteroid withdra-wal.Compared with before ACTH treatment, Prednisone was significantly decreased[(0.08±0.14) mg/(kg·d) vs. (0.23±0.23) mg/(kg·d)], and the difference was statistically significant ( t=3.661, P<0.05), the number of immunosuppressant cases decreased significantly[42.38%(12/32 cases) vs. 58.62%(17/32 cases), χ2= 14.500, P<0.05]. Serum cortisol was measured at 8 Am increased significantly[(11.78±4.64) μg/dL vs. (4.42±3.13) μg/dL, t=7.340, P<0.05]. The results were stable during follow-up, with 2 patients presenting with systemic urticaria once and 1 patient presenting with headache after infusion. Conclusions:ACTH therapy is safe and effective in hormone therapy for child with FRNS, and less adverse reactions.