| BackgroundPrimary nephrotic syndrome(PNS)is one of the common pediatric diseases.It is characterized by hypoalbuminemia,heavy proteinuria,hyperlipidemia and edema.Currently,it is mainly treated with glucocorticoid.According to the clinical response to glucocorticoid PNS was divided into steroid-sensitive nephrotic syndrome(SSNS),steroid-dependent nephrotic syndrome(SDNS),and steroid-resistant nephrotic syndrome(SRNS).And it can also be divided into non frequent relapses and frequent relapses nephrotic syndrome(FRNS)according to the relapses times.SDNS,SRNS and FRNs are collectively referred to as refractory nephrotic syndrome(RNS).For children with RNS are usually used immunosuppressive agents or(and)Cytotoxic agents,while long-term use of immunosuppressive agents can cause adverse reactions such as myelosuppression,gonadoinhibitory,infection and renal toxicity.A new treatment scheme is urgently needed to solve the above problems.In recent years,Adrenocorticotropic hormone(ACTH)has again been used to treat nephrotic syndrome with some clinical results.Traditionally,ACTH plays a role mainly by stimulating the secretion of glucocorticoids.With the deepening of research,people have come to realize that ACTH also has the function of independent glucocorticoids,which also has a good clinical response to the treatment of nephrotic syndrome.ObjectiveTo observe the efficacy and safety of ACTH in the treatment of children with refractory nephrotic syndrome.MethodsRetrospective analysis of children with nephrotic syndrome who received ACTH treatment in pediatric department of the First Affiliated Hospital of Xinxiang Medical University from August 2017 to October 2019 met the diagnostic criteria of steroid-resistant nephrotic syndrome,steroid-dependent nephrotic syndrome and frequently relapse nephrotic syndrome.All the children returned to the hospital every month to use adrenocorticotropic hormone for injection treatment(Shanghai First Biochemical Production,national SFDA approval number:H31022101,Drug specifications: 25 U / tube),the specific usage is 0.4-1u /(kg·d)(total amount ≤ 25U),add 250 ml of 5% glucose solution to intravenous drip for 6h,continuous use for 3 days as a course of treatment,one course per month,so repeatedly apply to ACTH treatment for 2 courses after prednisone reduction and stop.if there is relapse in the course of treatment,continue to use ACTH,but the longest is no more than 8 courses.ACTH was used for 3-8 courses.The levels of serum cortisol,urine routine,urine protein,prednisone dosage,immunosuppressant users,serum creatinine,urea,liver function and blood potassium were recorded before and after treatment.The number of relapses and adverse reactions were observed.Results1 45 children were included,40 males and 5 females.The average age of onset was4.5±2.7 years,including 5 steroid-resistant nephrotic syndrome,15 steroid-dependent nephrotic syndrome,25 frequent relapses nephrotic syndrome.The average course of disease before treatment was 36.07±32.47 months.25 of them were treated with immunosuppressive agents.Before treatment,the average amount of prednisone was 0.30 ±0.30 mg /(kg · d),the average amount of serum cortisol was 4.91 ± 3.43 ug/dl The average course of ACTH was 5.51 ± 1.62(3-8 courses),and the follow-up time was 18.73±4.50 months.2 In 5 children with steroid-resistant nephrotic syndrome,there were 4 patients with complete remission,the remission rate was 80%,1 patient had relapse,5 patients had a significant decrease in urinary protein after the treatment,the difference was statisticallysignificant(P < 0.05),1 patient assisted in prednisone reduction(20%),1 patient with immunosuppressant reduction(20%),and there was no statistical difference before and after prednisone dosage(P > 0.05).3 Among the 15 children with steroid-dependent nephrotic syndrome,8(53.3%)had no relapse.After the treatment,12(80%)patients were assisted in prednisone reduction and 1(6.7%)patient was immunosuppressant reduction.The dosage of prednisone before treatment0.26 ± 0.17 mg /(kg.d)was significantly lower than that after treatment 0.04 ± 0.07 mg /(kg.d)(P < 0.05).4 During the treatment and follow-up period,22(88%)of the 25 children with frequent relapses nephritic syndrome had no relapses.The frequency of relapse before treatment3.20±3.38 times per year was decreased significantly compared with that after treatment0.16±0.37 times per year(P < 0.05).The dosage of prednisone was decreased significantly before and after treatment(P < 0.05).Six children were stopped using immunosuppressants.5 At the end of the course of ACTH,serum cortisol 11.17±4.65 ug/dl and pre-treatment4.91 ± 3.43 ug/dl were paired sample T-test,the difference was statistically significant(P <0.05).6 The number of immunosuppressant users before the treatment was 25,and the number of immunosuppressant users after the treatment was 17,the difference was statistically significant(P < 0.05).7 After the treatment course of ACTH,there was no significant difference in serum creatinine,urea,serum potassium,glutamic-pyruvic transaminase,and glutamic oxalacetic transaminase before and after treatment(P >0.05).8 During the treatment of ACTH,there were 4 cases of skin rash and 1 case of high blood pressure with headache.All of them improved after treatment.ConclusionUsing ACTH to treat children with primary nephrotic syndrome can help the recovery of adrenocortical function,help the reduction of glucocorticoid,immunosuppressive agents anddecrease the number of relapses.At the same time,it has less adverse reactions and high safety. |
