Related Experiment Video
Updated: Aug 9, 2026
Induction of Nephrotic Syndrome in Mice by Retrobulbar Injection of Doxorubicin and Prevention of Volume Retention by Sustained Release Aprotinin
Published on: May 6, 2018
Interventions for idiopathic steroid-resistant nephrotic syndrome in children
E M Hodson1, D Habashy, J C Craig
1Children's Hospital at Westmead, Centre for Kidney Research, Locked Bag 4001, Westmead, NSW, Australia, 2145. Elisah@chw.edu.au
Insights
Cyclosporin effectively treats steroid-resistant nephrotic syndrome (SRNS) in children, increasing remission rates. Further research is needed to confirm efficacy and explore other treatment combinations for pediatric SRNS.
Area of Science:
- Pediatric Nephrology
- Clinical Pharmacology
- Evidence-Based Medicine
Background:
- Idiopathic steroid-resistant nephrotic syndrome (SRNS) affects children unresponsive to corticosteroids.
- Treatment options include immunosuppressive agents (e.g., cyclophosphamide, cyclosporin) and ACE inhibitors.
- Optimal treatment combinations with minimal toxicity require further investigation.
Purpose of the Study:
- To evaluate the benefits and harms of interventions for pediatric idiopathic SRNS.
- To synthesize evidence from randomized controlled trials (RCTs) on SRNS treatments.
Main Methods:
- Systematic review of RCTs and quasi-RCTs identified through major databases (CENTRAL, MEDLINE, EMBASE) and conference proceedings up to June 2005.
- Inclusion criteria: children (3 months–18 years) with SRNS, comparing different agents or placebo.
- Data analysis involved calculating relative risks (RR) and 95% confidence intervals (CI) using a random effects model.
Main Results:
- Eleven RCTs (312 children) were included. Cyclosporin significantly increased complete remission rates compared to placebo (RR 0.64, 95% CI 0.47–0.88).
- No significant differences in remission rates were found for oral/intravenous cyclophosphamide or azathioprine compared to prednisone alone.
- ACE inhibitors, such as fosinopril, significantly reduced proteinuria (by 0.95 g/24 h).
Conclusions:
- Cyclosporin demonstrates efficacy in achieving remission for pediatric SRNS.
- Further high-quality RCTs are necessary to confirm cyclosporin's effectiveness.
- Additional research is needed to evaluate other treatment regimens, including combinations of high-dose steroids with alkylating agents or cyclosporin.
Background:
The majority of children who present with their first episode of nephrotic syndrome, achieve remission with corticosteroid therapy. Children who fail to respond may be treated with immunosuppressive agents such as cyclophosphamide, chlorambucil or cyclosporin, or with non-immunosuppressive agents such as ACE inhibitors. Optimal combinations of these agents with the least toxicity remain to be determined.
Objectives:
To evaluate the benefits and harms of interventions used to treat idiopathic steroid resistant nephrotic syndrome (SRNS) in children.
Search Strategy:
Randomised controlled trials (RCTs) were identified from the Cochrane Central Register of Controlled Trials (CENTRAL), MEDLINE, EMBASE, reference lists of articles and abstracts from conference proceedings. Date of most recent search: June 2005
Selection Criteria:
RCTs and quasi-RCTs were included if they compared different immunosuppressive agents or non-immunosuppressive agents with placebo, prednisone or other agent given orally or parenterally in children aged three months to 18 years with SRNS.
Data Collection And Analysis:
Two reviewers independently searched the literature, determined trial eligibility, assessed quality, extracted data and entered it in RevMan. For dichotomous outcomes, results were expressed as relative risk (RR) and 95% confidence intervals (CI). Data were pooled using the random effects model.
Main Results:
Eleven RCTs (312 children) were included. Cyclosporin when compared with placebo or no treatment significantly increased the number of children who achieved complete remission (three trials, 49 children: RR for persistent nephrotic syndrome 0.64, 95% CI, 0.47 to 0.88). There was no significant difference in the number of children who achieved complete remission between oral cyclophosphamide with prednisone and prednisone alone (two trials, 91 children: RR 1.01, 95% CI 0.74 to 1.36), between intravenous cyclophosphamide and oral cyclophosphamide (one study, 11 children: RR 0.09, 95% CI 0.01 to 1.39) and between azathioprine with prednisone and prednisone alone (one trial 31 children: RR 1.01, 95% CI 0.77 to 1.32). ACE inhibitors significantly reduced proteinuria (two trials, 70 children). After 12 weeks of treatment fosinopril reduced proteinuria by 0.95 g/24 h (95% CI -1.21 to -0.69). No RCTs were identified comparing combination regimens comprising high dose steroids, alkylating agents or cyclosporin with single agents, placebo or no treatment.
Authors' Conclusions:
Further adequately powered and well designed RCTs are needed to confirm the efficacy of cyclosporin and to evaluate other regimens for idiopathic SRNS including high dose steroids with alkylating agents or cyclosporin.
Related Concept Videos
01:24Nephrotic Syndrome III : Nursing Management
01:26Nephrotic Syndrome II : Assessment and Medical Management
01:24Nephrotic Syndrome I : Introduction
01:28Chronic Kidney Disease III: Interprofessional Care
01:20Acute Kidney Injury V: Interprofessional Care
01:26Pharmacokinetics in Pediatric Patients: Drug Excretion