Real-word adrenocorticotropic hormone treatment for childhood-onset nephrotic syndrome

BackgroundCurrent first-line anti-proteinuric treatments do not produce a satisfactory therapeutic effect in a considerable number of patients with nephrotic syndrome (NS). Interest in adrenocorticotropic hormone (ACTH) for the treatment of NS has recently been revived. The present study investigate...

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Main Authors: Ying Wang, Xiqiang Dang, Xiaochuan Wu, Yongzhen Li, Qingnan He, Xiaoyan Li
Format: Article
Language:English
Published: Frontiers Media S.A. 2023-03-01
Series:Frontiers in Pediatrics
Subjects:
Online Access:https://www.frontiersin.org/articles/10.3389/fped.2023.1044075/full
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author Ying Wang
Ying Wang
Xiqiang Dang
Xiqiang Dang
Xiaochuan Wu
Xiaochuan Wu
Yongzhen Li
Yongzhen Li
Qingnan He
Xiaoyan Li
Xiaoyan Li
author_facet Ying Wang
Ying Wang
Xiqiang Dang
Xiqiang Dang
Xiaochuan Wu
Xiaochuan Wu
Yongzhen Li
Yongzhen Li
Qingnan He
Xiaoyan Li
Xiaoyan Li
author_sort Ying Wang
collection DOAJ
description BackgroundCurrent first-line anti-proteinuric treatments do not produce a satisfactory therapeutic effect in a considerable number of patients with nephrotic syndrome (NS). Interest in adrenocorticotropic hormone (ACTH) for the treatment of NS has recently been revived. The present study investigated the efficacy and safety of ACTH treatment in children with frequent relapsing NS (FRNS), steroid-dependent NS (SDNS), and steroid-resistant NS (SRNS).MethodThe ACTH treatment group was comprised of NS patients receiving ACTH treatment. Patients with serum cortisol concentrations <85.3 nmol/L and who had not received ACTH treatment previously were enrolled in the control group from January 2018 to January 2021. The maintenance dose of prednisone, the number of disease recurrences, the time of first disease relapse, immunosuppressant use, serum cortisol levels, and adverse events were recorded in both groups.ResultsFifty-one patients were included in the ACTH group, and twenty-one patients were enrolled in the control group. Concurrent treatment with one or more immunosuppressive and/or cytotoxic treatments occurred in 92.2% and 85.7% of patients in the ACTH and control groups, respectively, throughout the study period. A greater reduction in the prednisone maintenance dose was observed in the ACTH group compared with the control group after 1 year of follow up (0.603 ± 0.445 mg/kg vs. 0.267 ± 0.500 mg/kg, p = 0.006). During the one-year study period, fewer participants experienced one or more disease relapses in the ACTH group (45.1%) compared to the control group (76.2%, odds ratio = 3.896, p = 0.016). The number of disease recurrences per patient in the ACTH group was less than that in the control group (median difference = −1, p = 0.006). The mean length of remission was 8.902 m and 7.905 m in the ACTH group and control group, respectively. A log-rank test showed a longer relapse free survival for patients in the ACTH group (p = 0.046), but the Breslow test showed no significant difference between groups (p = 0.104). Ten patients in the ACTH group successfully discontinued all drug therapies. No patients in the control group were able to discontinue drug therapy as of February 2022.ConclusionACTH, combined with multiple drugs, is effective at reducing the prednisone maintenance dose and may effectively prevent disease relapses in childhood NS.
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spelling doaj.art-392a5d7a8d57430db504e815496b02ab2023-03-09T07:12:06ZengFrontiers Media S.A.Frontiers in Pediatrics2296-23602023-03-011110.3389/fped.2023.10440751044075Real-word adrenocorticotropic hormone treatment for childhood-onset nephrotic syndromeYing Wang0Ying Wang1Xiqiang Dang2Xiqiang Dang3Xiaochuan Wu4Xiaochuan Wu5Yongzhen Li6Yongzhen Li7Qingnan He8Xiaoyan Li9Xiaoyan Li10Department of Pediatrics, The Second Xiangya Hospital, Central South University, Changsha, ChinaDepartment of Pediatrics Nephrology, Children's Medical Center, The Second Xiangya Hospital, Central South University, Changsha, ChinaDepartment of Pediatrics, The Second Xiangya Hospital, Central South University, Changsha, ChinaDepartment of Pediatrics Nephrology, Children's Medical Center, The Second Xiangya Hospital, Central South University, Changsha, ChinaDepartment of Pediatrics, The Second Xiangya Hospital, Central South University, Changsha, ChinaDepartment of Pediatrics Nephrology, Children's Medical Center, The Second Xiangya Hospital, Central South University, Changsha, ChinaDepartment of Pediatrics, The Second Xiangya Hospital, Central South University, Changsha, ChinaDepartment of Pediatrics Nephrology, Children's Medical Center, The Second Xiangya Hospital, Central South University, Changsha, ChinaDepartment of Pediatrics, The Third Xiangya Hospital, Central South University, Changsha, ChinaDepartment of Pediatrics, The Second Xiangya Hospital, Central South University, Changsha, ChinaDepartment of Pediatrics Nephrology, Children's Medical Center, The Second Xiangya Hospital, Central South University, Changsha, ChinaBackgroundCurrent first-line anti-proteinuric treatments do not produce a satisfactory therapeutic effect in a considerable number of patients with nephrotic syndrome (NS). Interest in adrenocorticotropic hormone (ACTH) for the treatment of NS has recently been revived. The present study investigated the efficacy and safety of ACTH treatment in children with frequent relapsing NS (FRNS), steroid-dependent NS (SDNS), and steroid-resistant NS (SRNS).MethodThe ACTH treatment group was comprised of NS patients receiving ACTH treatment. Patients with serum cortisol concentrations <85.3 nmol/L and who had not received ACTH treatment previously were enrolled in the control group from January 2018 to January 2021. The maintenance dose of prednisone, the number of disease recurrences, the time of first disease relapse, immunosuppressant use, serum cortisol levels, and adverse events were recorded in both groups.ResultsFifty-one patients were included in the ACTH group, and twenty-one patients were enrolled in the control group. Concurrent treatment with one or more immunosuppressive and/or cytotoxic treatments occurred in 92.2% and 85.7% of patients in the ACTH and control groups, respectively, throughout the study period. A greater reduction in the prednisone maintenance dose was observed in the ACTH group compared with the control group after 1 year of follow up (0.603 ± 0.445 mg/kg vs. 0.267 ± 0.500 mg/kg, p = 0.006). During the one-year study period, fewer participants experienced one or more disease relapses in the ACTH group (45.1%) compared to the control group (76.2%, odds ratio = 3.896, p = 0.016). The number of disease recurrences per patient in the ACTH group was less than that in the control group (median difference = −1, p = 0.006). The mean length of remission was 8.902 m and 7.905 m in the ACTH group and control group, respectively. A log-rank test showed a longer relapse free survival for patients in the ACTH group (p = 0.046), but the Breslow test showed no significant difference between groups (p = 0.104). Ten patients in the ACTH group successfully discontinued all drug therapies. No patients in the control group were able to discontinue drug therapy as of February 2022.ConclusionACTH, combined with multiple drugs, is effective at reducing the prednisone maintenance dose and may effectively prevent disease relapses in childhood NS.https://www.frontiersin.org/articles/10.3389/fped.2023.1044075/fullnephrotic syndromeadrenocorticotropic hormonechildrenprednisone maintenance doserelapse
spellingShingle Ying Wang
Ying Wang
Xiqiang Dang
Xiqiang Dang
Xiaochuan Wu
Xiaochuan Wu
Yongzhen Li
Yongzhen Li
Qingnan He
Xiaoyan Li
Xiaoyan Li
Real-word adrenocorticotropic hormone treatment for childhood-onset nephrotic syndrome
Frontiers in Pediatrics
nephrotic syndrome
adrenocorticotropic hormone
children
prednisone maintenance dose
relapse
title Real-word adrenocorticotropic hormone treatment for childhood-onset nephrotic syndrome
title_full Real-word adrenocorticotropic hormone treatment for childhood-onset nephrotic syndrome
title_fullStr Real-word adrenocorticotropic hormone treatment for childhood-onset nephrotic syndrome
title_full_unstemmed Real-word adrenocorticotropic hormone treatment for childhood-onset nephrotic syndrome
title_short Real-word adrenocorticotropic hormone treatment for childhood-onset nephrotic syndrome
title_sort real word adrenocorticotropic hormone treatment for childhood onset nephrotic syndrome
topic nephrotic syndrome
adrenocorticotropic hormone
children
prednisone maintenance dose
relapse
url https://www.frontiersin.org/articles/10.3389/fped.2023.1044075/full
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