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Published on: May 6, 2018
Interventions for idiopathic steroid-resistant nephrotic syndrome in children
Insights
Cyclosporin shows promise in treating childhood steroid-resistant nephrotic syndrome (SRNS), significantly increasing remission rates compared to placebo. Further research is needed to confirm efficacy and explore other treatment combinations for SRNS.
Area of Science:
- Pediatric Nephrology
- Clinical Pharmacology
- Systematic Reviews
Background:
- Most children with nephrotic syndrome respond to corticosteroids.
- Steroid-resistant nephrotic syndrome (SRNS) requires alternative treatments, including immunosuppressants (e.g., cyclophosphamide, cyclosporine) and non-immunosuppressants (e.g., ACE inhibitors).
- Optimal treatment strategies for SRNS with minimal toxicity are yet to be established.
Purpose of the Study:
- To systematically review the benefits and harms of all interventions for children diagnosed with idiopathic steroid-resistant nephrotic syndrome (SRNS).
Main Methods:
- A comprehensive literature search was conducted for published and unpublished randomized controlled trials (RCTs).
- Included trials compared different immunosuppressive or non-immunosuppressive agents against placebo, prednisone, or other agents in children (3 months to 18 years) with SRNS.
- Data from nine RCTs involving 225 children were analyzed using a random effects model, with results expressed as relative risk (RR) and 95% confidence intervals (CI).
Main Results:
- Cyclosporine significantly increased complete remission rates compared to placebo or no treatment (RR 0.64, 95% CI 0.47 to 0.88).
- No significant differences in remission rates were observed between oral cyclophosphamide and prednisone alone, intravenous versus oral cyclophosphamide, or azathioprine and prednisone alone.
- No RCTs were found that compared combination regimens (high-dose steroids, alkylating agents, cyclosporine) against single agents or placebo.
Conclusions:
- Cyclosporine demonstrates potential efficacy in treating pediatric SRNS.
- Further well-designed, adequately powered RCTs are necessary to confirm cyclosporine's effectiveness.
- Additional research is required to evaluate other treatment regimens for SRNS, including combinations of high-dose steroids with alkylating agents or cyclosporine.
Background:
The majority of children, who present with their first episode of nephrotic syndrome, achieve remission with corticosteroid therapy. Children who fail to respond to corticosteroids 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 least toxicity remain to be determined. The aims of this systematic review are to assess the benefits and harms of interventions used to treat idiopathic steroid resistant nephrotic syndrome (SRNS) in children.
Objectives:
We aimed to evaluate the benefits and harms of all interventions for children with SRNS.
Search Strategy:
Published and unpublished randomised controlled trials (RCTs) were identified from the Cochrane Controlled Trials Register, MEDLINE, EMBASE, reference lists of articles and abstracts from conference proceedings.
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 3 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:
Nine RCTs involving 225 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). No RCTs were identified comparing combination regimens comprising high dose steroids, alkylating agents or cyclosporin with single agents, placebo or no treatment.
Reviewers' 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.
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