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Meta-Analysis
. 2024 Nov 8;11(11):CD002290.
doi: 10.1002/14651858.CD002290.pub6.

Non-corticosteroid immunosuppressive medications for steroid-sensitive nephrotic syndrome in children

Nicholas G Larkins  1 , Deirdre Hahn  2 , Isaac D Liu  3 , Narelle S Willis  4   5 , Jonathan C Craig  5   6 , Elisabeth M Hodson  5
Affiliations
Meta-Analysis

Non-corticosteroid immunosuppressive medications for steroid-sensitive nephrotic syndrome in children

Nicholas G Larkins et al. Cochrane Database Syst Rev. .

Abstract

Background: About 80% of children with steroid-sensitive nephrotic syndrome (SSNS) have relapses. Of these children, half will relapse frequently, and are at risk of adverse effects from corticosteroids. While non-corticosteroid immunosuppressive medications prolong periods of remission, they have significant potential adverse effects. Currently, there is no consensus about the most appropriate second-line agent in children with frequently relapsing SSNS. In addition, these medications could be used with corticosteroids in the initial episode of SSNS to prolong the period of remission. This is the fifth update of a review first published in 2001 and updated in 2005, 2008, 2013 and 2020.

Objectives: To evaluate the benefits and harms of non-corticosteroid immunosuppressive medications in SSNS in children with a relapsing course of SSNS and in children with their first episode of nephrotic syndrome.

Search methods: We searched the Cochrane Kidney and Transplant Register of Studies up to October 2024 through contact with the Information Specialist using search terms relevant to this review. Studies in the Register are identified through searches of CENTRAL, MEDLINE, and EMBASE, conference proceedings, the International Clinical Trials Registry Platform (ICTRP) Search Portal and ClinicalTrials.gov.

Selection criteria: Randomised controlled trials (RCTs) or quasi-RCTs were included if they involved children with SSNS and compared non-corticosteroid immunosuppressive medications with placebo, corticosteroids or no treatment; different non-corticosteroid immunosuppressive medications, or different doses, durations or routes of administration of the same non-corticosteroid immunosuppressive medication.

Data collection and analysis: Two authors independently assessed study eligibility, risk of bias and extracted data from the included studies. Statistical analyses were performed using a random-effects model and results expressed as risk ratio (RR) for dichotomous outcomes or mean difference (MD) for continuous outcomes with 95% confidence intervals (CI). Confidence in the evidence was assessed using the Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach.

Main results: We identified 58 studies (122 reports) randomising 3720 children. Half were multicentre studies, and most studies were undertaken in South and East Asia (28 studies) and Europe (20 studies). The numbers of children randomised ranged from 14 to 211. Risk of bias assessment indicated that 32 and 33 studies were at low risk of bias for sequence generation and allocation concealment, respectively. Eleven studies were at low risk of performance bias and 13 were at low risk of detection bias. Forty-eight and 36 studies were at low risk of incomplete and selective reporting, respectively. Rituximab with or without prednisone compared with placebo with or without prednisone probably reduces the number of children experiencing relapse at six months (5 studies, 182 children: RR 0.22, 95% CI 0.11 to 0.43) and 12 months (3 studies, 108 children: RR 0.38, 95% CI 0.13 to 1.09) (moderate certainty), may increase the number with severe infusion reactions (4 studies, 162 children: RR 5.21, 95% CI 1.19 to 22.89; low certainty), but not severe infection or arthropathy (low certainty). Rituximab compared with tacrolimus probably reduces the risk of relapse at 12 months (4 studies, 238 children: RR 0.64, 95% CI 0.42 to 0.96) and may reduce the risk of relapse when compared with low dose mycophenolate mofetil (MMF) (1 study, 30 children: RR 0.17, 95% CI 0.04 to 0.62). Rituximab followed by MMF for 500 days reduces the risk of relapse compared with rituximab followed by placebo for 500 days (1 study, 78 children: RR 0.29, 95% CI 0.13 to 0.63; high certainty). Rituximab probably does not differ from ofatumumab in the riisk of relapse and 12 months (1 study, 140 children: RR 1.03, 95% CI 0.75 to 1.41; moderate certainty) or in adverse events. MMF and levamisole (1 study, 149 children: RR 0.90, 95% CI 0.70 to 1.16) may have similar effects on the number of children who relapse at 12 months (low certainty). Cyclosporin compared with MMF may reduce the risk of relapse at 12 months (3 studies, 114 children: RR 1.57, 95% CI 1.08 to 2.30) (low certainty). Levamisole compared with steroids or placebo may reduce the number of children with relapse during treatment (8 studies, 474 children: RR 0.52, 95% CI 0.33 to 0.82) (low certainty). Preliminary data from single studies indicate that levamisole and prednisone compared with prednisone alone may delay the onset of relapse after the initial episode of SSNS and that levamisole compared with increasing prednisone administration from alternate day to daily at the onset of infection may reduce the risk of relapse with infection (low certainty). Cyclosporin compared with prednisone may reduce the number of children who relapse (1 study, 104 children: RR 0.33, 95% CI 0.13 to 0.83) (low certainty). Alkylating agents compared with cyclosporin may make little or no difference to the risk of relapse during cyclosporin treatment (2 studies, 95 children: RR 0.91, 95% CI 0.55 to 1.48) (low certainty evidence) but may reduce the risk of relapse at 12 to 24 months (2 studies, 95 children: RR 0.51, 95% CI 0.35 to 0.74) (low certainty). Alkylating agents (cyclophosphamide and chlorambucil) compared with prednisone probably reduce the number of children who experience relapse at six to 12 months (6 studies, 202 children: RR 0.44, 95% CI 0.32 to 0.60) and at 12 to 24 months (4 studies, 59 children: RR 0.20, 95% CI 0.09 to 0.46) (moderate certainty).

Authors' conclusions: New studies incorporated in this review update indicate that rituximab compared with prednisone, tacrolimus, or MMF is a valuable additional agent for managing children with relapsing SSNS. Comparative studies of CNIs, MMF, and levamisole suggest that CNIs may be more effective than MMF and that levamisole may be similar in efficacy to MMF. Important new studies suggest that MMF prolongs remission following rituximab, that levamisole may prevent infection-related relapse more effectively than changing from alternate-day to daily prednisone and that levamisole and prednisone compared with prednisone alone may prolong the time to first relapse. There are currently 23 ongoing studies which should improve our understanding of how to treat children with frequently relapsing SSNS.

Trial registration: ClinicalTrials.gov NCT01716442 NCT02132195 NCT01092962 NCT02818738 NCT01268033 NCT02394119 NCT04494438 NCT04402580 NCT02438982 NCT02390362 NCT05599815 NCT05627557 NCT03560011 NCT01895894 NCT04585152 NCT02972346 NCT04048161 NCT05786768 NCT05843968 NCT05914155 NCT06079788 NCT03899103.

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Conflict of interest statement

  1. Nicolas G Larkins: no relevant interests were disclosed

  2. Deirdre Hahn: no relevant interests were disclosed

  3. Isaac D Liu: no relevant interests were disclosed

  4. Narelle S Willis: no relevant interests were disclosed

  5. Jonathan C Craig: no relevant interests were disclosed

  6. Elisabeth M Hodson: no relevant interests were disclosed

Update of

References

References to studies included in this review

Abeyagunawardena 2006a {published data only}
    1. Abeyagunawardena AS, Trompeter RS. Efficacy of levamisole as a single agent in maintaining remission in steroid dependant nephrotic syndrome [abstract no: COD.OC.5]. Pediatric Nephrology 2006;21(10):1503. [CENTRAL: CN-00602129] - PubMed
Abeyagunawardena 2006b {published data only}
    1. Abeyagunawardena AS, Trompeter RS. Intravenous pulsed vs oral cyclophosphamide therapy in steroid dependant nephrotic syndrome [abstract no: COD.PP 54]. Pediatric Nephrology 2006;21(10):1535.
Abeyagunawardena 2007 {published data only}
    1. Abeyagunawardena A. Intravenous pulsed cyclophosphamide versus vincristine therapy in steroid dependant nephrotic syndrome: a randomised controlled trial [abstract no: 550P]. Pediatric Nephrology 2007;22(9):1547.
Ahn 2018 {published data only}
    1. Ahn YH, Kang HG, Kim SH. Efficacy and safety of rituximab in children with refractory nephrotic syndrome: a multicenter clinical trial [abstract]. Kidney Research & Clinical Practice 2014;33(2):A1. [CENTRAL: CN-01657515]
    1. Ahn YH, Kim SH, Han KH, Choi HJ, Cho H, Lee JW, et al. Efficacy and safety of rituximab in childhood-onset, difficult-to-treat nephrotic syndrome: a multicenter open-label trial in Korea. Medicine 2018;97(46):e13157. [MEDLINE: ] - PMC - PubMed
    1. Anh YH, Kim SH, Han KH, Cho HY, Shin JI, Cho MH, et al. Efficacy and safety of rituximab in children with refractory nephrotic syndrome: a multicenter clinical trial [abstract no: O39]. Pediatric Nephrology 2013;28(8):1361. [EMBASE: 71126980]
Alatas 1978 {published data only}
    1. Alatas H, Wirya IG, Tambunan T, Himawan S. Controlled trial of chlorambucil in frequently relapsing nephrotic syndrome in children (a preliminary report). Journal of the Medical Association of Thailand 1978;61 Suppl 1:222-8. [MEDLINE: ] - PubMed
Al‐Saran 2006 {published data only}
    1. Al-Saran K, Mirza K, Al-Ghanam G, Abdelkarim M. Experience with levamisole in frequently relapsing, steroid-dependent nephrotic syndrome. Pediatric Nephrology 2006;21(2):201-5. [MEDLINE: ] - PubMed
    1. Al-Saran K, Mirza K. Experience with levamisole in FR/SD childhood nephrotic syndrome in a large Saudi center [abstract no: PO03]. Pediatric Nephrology 2004;19(9):C93. [CENTRAL: CN-00583544]
APN 1982 {published and unpublished data}
    1. Arbeitsgemeinschaft fur Padiatrische Nephrologie. Effect of cytotoxic drugs in frequently relapsing nephrotic syndrome with and without steroid dependence. New England Journal of Medicine 1982;306(8):451-4. [MEDLINE: ] - PubMed
    1. Brodehl J, Krohn HP, Ehrich JH. The treatment of minimal change nephrotic syndrome (lipoid nephrosis): cooperative studies of the Arbeitsgemeinschaft fur Padiatrische Nephrologie (APN). Klinische Padiatrie 1982;194(3):162-5. [MEDLINE: ] - PubMed
APN 2006 {published data only}
    1. Arbeitsgemeinschaft fur Padiatrische Nephrologie. Results of the nephrotic syndrome study VIII of the APN: new standard treatment versus new standard treatment plus 8 weeks cyclosporin A [abstract]. Pediatric Nephrology 1999;13:C26. [CENTRAL: CN-00636143]
    1. Hoyer PF, Brodehl J. Initial treatment of idiopathic nephrotic syndrome in children: prednisone versus prednisone plus cyclosporine A: a prospective, randomized trial. Journal of the American Society of Nephrology 2006;17(4):1151–7. [MEDLINE: ] - PubMed
    1. Hoyer PF. Results of the nephrotic syndrome study VIII of the APN: new standard treatment versus new standard treatment plus 8 weeks cyclosporin A [abstract]. Journal of the American Society of Nephrology 1999;10(Program & Abstracts):104A. [CENTRAL: CN-00550522]
    1. Hoyer PF, Arbeitsgemeinschaft fur Padiatrische Nephrologie. The initial treatment of idiopathic nephrotic syndrome with prednisone and cyclosporin A: preliminary results of a therapeutic trial [abstract no: P147]. Pediatric Nephrology 1995;9(6):C91. [CENTRAL: CN-00401335]
ATLANTIS 2018 {published data only}
    1. Wang CS, Travers C, McCracken C, Leong T, Gbadegesin R, Quiroga A, et al. Adrenocorticotropic hormone for childhood nephrotic syndrome: the ATLANTIS randomized trial. Clinical Journal of the American Society of Nephrology: CJASN 2018;13(12):1859-65. [MEDLINE: ] - PMC - PubMed
Baluarte 1978 {published data only}
    1. Baluarte HJ, Hiner L, Gruskin AB. Chlorambucil dosage in frequently relapsing nephrotic syndrome: a controlled clinical trial. Journal of Pediatrics 1978;92(2):295-8. [PMID: ] - PubMed
Banerjee 2024 {published data only}2015/10/006257
    1. Banerjee S, Sengupta J, Sinha R, Chatterjee S, Sarkar S, Akhtar S, et al. Daily compared with alternate-day levamisole in pediatric nephrotic syndrome: an open-label randomized controlled study. Pediatric Nephrology 2024;39(10):2969-77. [PMID: ] - PubMed
BAPN 1991 {published and unpublished data}
    1. Anonymous. Levamisole for corticosteroid-dependent nephrotic syndrome in childhood. British Association for Paediatric Nephrology. Lancet 1991;337(8757):1555-7. [MEDLINE: ] - PubMed
Barratt 1970 {published data only}
    1. Barratt TM, Soothill JF. A controlled trial of cyclophosphamide in steroid sensitive relapsing nephrotic syndrome of childhood [abstract]. Nephron 1971;8:95. [CENTRAL: CN-00253521] - PubMed
    1. Barratt TM, Soothill JF. Controlled trial of cyclophosphamide in steroid-sensitive relapsing nephrotic syndrome of childhood. Lancet 1970;296(7671):479-2. [MEDLINE: ] - PubMed
Barratt 1973 {published data only}
    1. Barratt TM, Cameron JS, Chantler C, Ogg CS, Soothill JF. Comparative trial of 2 weeks and 8 weeks cyclophosphamide in steroid-sensitive relapsing nephrotic syndrome of childhood. Archives of Disease in Childhood 1973;48(4):286-90. [MEDLINE: ] - PMC - PubMed
Barratt 1977 {published data only}
    1. Barratt TM, Cameron JS, Chantler C, Counahan R, Ogg CS, Soothill JF. Controlled trial of azathioprine in treatment of steroid-responsive nephrotic syndrome of childhood. Archives of Disease in Childhood 1977;52(6):462-3. [MEDLINE: ] - PMC - PubMed
Baudouin 2022 {published data only}
    1. Baudouin V, Lahoche A, Taque S, Vrillon I, Decramer S, Cloarec S, et al. Mycophenolate mofetil (MMF) versus cyclophosphamide (CYC) to prevent relapse in children with steroid-dependent nephrotic syndrome (SDNS): a multicentrer, randomized, controlled trial [abstract no: O-27]. Pediatric Nephrology 2023;38(7):2268-9. [DOI: 10.1007/s00467-022-05865-y] [EMBASE: 641737183] - DOI
    1. Baudouin V, Lahoche A, Vrillon I, Decramer S, Cloarec S, Roussey G, et al. Mycophenolate mofetil (MMF) versus cyclophosphamide (CYC) to prevent relapse in children with steroid-dependent nephrotic syndrome (SDNS): a multicentrer, randomized, controlled trial [abstract no: P1-96]. Pediatric Nephrology 2022;37(11):2857. [DOI: 10.1007/s00467-022-05630-1] [EMBASE: 639293145] - DOI
Cerkauskiene 2005 {published data only}
    1. Cerkauskiene R, Kaltenis P. Comparative study of prednisolone alone and prednisolone plus fusidic acid in the treatment of children with steroid-responsive nephrotic syndrome. Medicina (Kaunas, Lithuania) 2005;41 Suppl 1:26-30. [MEDLINE: ] - PubMed
Chiu 1973 {published data only}
    1. Chiu J, McLaine PN, Drummond KN. A controlled prospective study of cyclophosphamide in relapsing, corticosteroid-responsive, minimal-lesion nephrotic syndrome in childhood. Journal of Pediatrics 1973;82(4):607-13. [MEDLINE: ] - PubMed
Dayal 1994 {published data only}
    1. Dayal U, Dayal AK, Shastry JC, Raghupathy P. Use of levamisole in maintaining remission in steroid-sensitive nephrotic syndrome in children [Erratum in: Nephron 1994;67(4):507]. Nephron 1994;66(4):408-12. [MEDLINE: ] - PubMed
Donia 2005 {published data only}
    1. Donia A, Ammar H, Moustafa F, Sobh M. Long-term efficacy of two unconventional adjunctive therapies in minimal change nephrotic children [abstract no: SP062]. In: 41st Congress. European Renal Association. European Dialysis and Transplantation Association; 2004 May 15-18; Lisbon, Portugal. 2004:37. [CENTRAL: CN-00509161]
    1. Donia AF, Ammar HM, El-Agroudy A, Moustafa F, Sobh MA. Long-term results of two unconventional agents in steroid-dependent nephrotic children. Pediatric Nephrology 2005;20(10):1420-5. [MEDLINE: ] - PubMed
Dorresteijn 2008 {published data only}
    1. Dorresteijn E, Kist-van Holthe J, Levtchenko E, Nauta J, Hop W, Heijden B. Randomized controlled trial of mycophenolate mofetil (MMF) versus cyclosporine A (CsA) in children with frequently relapsing nephrotic syndrome [abstract no: 28FC]. Pediatric Nephrology 2007;22(9):1442. [CENTRAL: CN-00636133]
    1. Dorresteijn E, Van Elburg R, Nauta J, Heijden B. Intestinal permeability in patients treated with mycophenolate mofetil (MMF) for nephrotic syndrome (NS) [abstract no: 355]. Pediatric Nephrology 2010;25(9):1865-6. [EMBASE: 70438457]
    1. Dorresteijn EM, Kist-van Holthe JE, Levtchenko EN, Nauta J, Hop WC, Heijden AJ. Mycophenolate mofetil versus cyclosporine for remission maintenance in nephrotic syndrome. Pediatric Nephrology 2008;23(11):2013-20. [MEDLINE: ] - PMC - PubMed
Gellermann 2013 {published data only}61976169
    1. Gellermann J, Querfeld U. Frequently relapsing steroid-sensitive nephrotic syndrome (SSNS) in children and adolescents: treatment with mycophenolate mofetil (MMF) vs. cyclosporin A (CsA) [abstract no: OS3-FRI-172]. Pediatric Nephrology 2011;26(9):1579. [EMBASE: 70530472]
    1. Gellermann J, Schaefer F, Querfeld U. Serum suPAR levels are modulated by immunosuppressive therapy of minimal change nephrotic syndrome. Pediatric Nephrology 2014;29(12):2411-4. [MEDLINE: ] - PubMed
    1. Gellermann J, Weber L, Pape L, Tonshoff B, Hoyer P, Querfeld U, et al. Mycophenolate mofetil versus cyclosporin A in children with frequently relapsing nephrotic syndrome. Journal of the American Society of Nephrology 2013;24(10):1689-97. [MEDLINE: ] - PMC - PubMed
    1. Querfeld U, Gellermann J. A randomized cross-over multicenter trial of mycophenolate mofetil versus cyslosporin A in children with frequently relapsing nephrotic syndrome [abstract no: LB-OR04]. Journal of the American Society of Nephrology 2011;22(Abstracts):1-2B. [CENTRAL: CN-01912567] - PMC - PubMed
Geng 2018 {published data only}
    1. Geng HY, Ji LN, Chen CY, Tu J, Li HR, Bao R, et al. Mycophenolate mofetil versus cyclosporine A in children with primary refractory nephrotic syndrome. Zhonghua Erke Zazhi 2018;56(9):651-6. [MEDLINE: ] - PubMed
Grupe 1976 {published data only}
    1. Grupe WE, Makker SP, Ingelfinger JR. Chlorambucil treatment of frequently relapsing nephrotic syndrome. New England Journal of Medicine 1976;295(14):746-9. [MEDLINE: ] - PubMed
Gruppen 2015 {published data only}23853712
    1. Gruppen M, Bouts A, Niaudet P, Schurmans T, Knops N, Ranguelov N, et al. The levamisole study in steroid sensitive idiopathic nephrotic syndrome (SSINS): inclusion completed [abstract no: P150]. Pediatric Nephrology 2012;27(9):1712-3. [EMBASE: 71386368]
    1. Gruppen M, Davin JC, Bouts A. Levamisole increases the time to relapse in children with steroid-sensitive idiopathic nephrotic syndrome: Results of a multi-center, double-blind, placebo-controlled, randomized clinical trial [abstract no: FP-S25-11]. Pediatric Nephrology 2016;31(10):1753. [EMBASE: 612479674]
    1. Gruppen M, Merkus M, Bouts A, Offringa M, Davin J. First aid in performing international paediatric clinical drug trials using the experience of the levamisole trial [abstract no: P219]. Pediatric Nephrology 2008;23:1651.
    1. Gruppen MP, Bouts AH, Davin JC. The levamisole study in steroid sensitive idiopathic nephrotic syndrome (SSINS): completion is approaching [abstract no: PS2-FRI-537]. Pediatric Nephrology 2011;26(9):1682. [EMBASE: 70530801]
    1. Gruppen MP, Bouts AH, Jansen-van der Weide MC, Merkus MP, Zurowska A, Maternik M, et al. A randomized clinical trial indicates that levamisole increases the time to relapse in children with steroid-sensitive idiopathic nephrotic syndrome. Kidney international 2018;93(2):510-8. [MEDLINE: ] - PubMed
Iijima 2014 {published data only}000000008
    1. Iijima K, Nakamura MS, Saito M, Ohashi Y, Yoshikawa N. Cyclosporine C2 monitoring for frequent-relapsing nephrotic syndrome in children: a multicenter randomized controlled trial [abstract no: LB-PO3157]. Journal of the American Society of Nephrology 2011;22(Abstracts):6B. [CENTRAL: CN-01912566]
    1. Iijima K, Sako M, Oba MS, Ito S, Hataya H, Tanaka R, et al. Cyclosporine C2 monitoring for the treatment of frequently relapsing nephrotic syndrome in children: a multicenter randomized phase II trial. Clinical Journal of the American Society of Nephrology: CJASN 2014;9(2):271-8. [MEDLINE: ] - PMC - PubMed
    1. Nakamura MS, Iijima K, Oba MS, Honda M, Nakamura H, Nagata M, et al. Cyclosporine C2 monitoring for the treatment of frequently relapsing nephrotic syndrome in children: a multicenter randomized trial [abstract no: P-SUN191]. Pediatric Nephrology 2013;28(8):1596.
Iijima 2014a {published data only}
    1. Iijima K, Sako M, Nozu K, Mori R, Tuchida M, Kamei K, et al. Rituximab for childhood-onset, complicated, frequently relapsing nephrotic syndrome or steroid-dependent nephrotic syndrome: a multicentre, double-blind, randomised, placebo-controlled trial. Lancet 2014;384(9950):1273-81. [MEDLINE: ] - PubMed
    1. Iijima K, Sako M, Nozu K, Tsuchida N, Tanaka R, Ishikura K, et al. Multicenter, double-blind, placebo-controlled, randomized trial of rituximab for the treatment of childhood-onset refractory nephrotic syndrome [abstract no: O-41]. Pediatric Nephrology 2013;28(8):1362-3. [EMBASE: 71126982]
    1. Iijima K, Sako M, Nozu K. Rituximab treatment for nephrotic syndrome in children. Current Pediatric Reports 2015;3(1):71-7. [MEDLINE: ] - PMC - PubMed
    1. Iijima K, Sako M, Tsuchida N, Ohashi Y. Multicenter double-blind, randomized, placebo-controlled trial of rituximab for the treatment of childhood-onset refractory nephrotic syndrome [abstract no: SA-PO1102]. Journal of the American Society of Nephrology 2014;23(Abstracts):5B. [CENTRAL: CN-01912564]
    1. Iijima K, Tsuchida N, Sako M. Multicenter double-blind, randomized, placebo-controlled trial of IDEC-C2B8 for the treatment of childhood-onset complicated nephrotic syndrome: clinical study protocol Number: RCRNS-01 Version: 4.0. www.med.kobe-u.ac.jp/pediat/pdf/rcrn01.pdf (accessed 3 October 2024). [CENTRAL: CN-01658465]
Iijima 2022 {published data only}000014347
    1. Horinouchi T, Sako M, Nakanishi K, Ishikura K, Ito S, Nakamura H, et al. Study protocol: mycophenolate mofetil as maintenance therapy after rituximab treatment for childhood-onset, complicated, frequently-relapsing nephrotic syndrome or steroid-dependent nephrotic syndrome: a multicenter double-blind, randomized, placebo-controlled trial (JSKDC07). BMC Nephrology 2018;19(1):302. [MEDLINE: ] - PMC - PubMed
    1. Iijima K, Sako M, Oba M, Tanaka S, Hamada R, Sakai T, et al. Mycophenolate mofetil after rituximab for childhood-onset complicated frequently-relapsing or steroid-dependent nephrotic syndrome. Journal of the American Society of Nephrology 2022;33(2):401-19. [MEDLINE: ] - PMC - PubMed
Ishaq 2023 {published data only}
    1. Ishaq S, Imran M, Raza H, Rashid K, Zafar MS, Ahmed M. Comparison of efficacy of levamisole versus mycophenolate mofetilin children with frequently relapsing steroid sensitive nephrotic syndrome. Pakistan Journal of Medical Research 2023;62(1):32-5.
Ishikura 2008 {published data only}C000000014
    1. Ishikura K, Ikeda M, Hattori S, Yoshikawa N, Sasaki S, Iijima K, et al. Effective and safe treatment with cyclosporine in nephrotic children: a prospective, randomized multicenter trial. Kidney International 2008;73(10):1167-73. [MEDLINE: ] - PubMed
    1. Ishikura K, Ikeda M, Hattori S, Yoshikawa N, Sasaki S, Ijima K, et al. A 2-year, prospective, randomized, multicenter trial of cyclosporine in children with frequently relapsing nephrotic syndrome [abstract no: 497P]. Pediatric Nephrology 2007;22(9):1531. [CENTRAL: CN-00653771]
    1. Ishikura K, Yoshikawa N, Nakazato H, Sasaki S, Nakanishi K, Matsuyama T, et al. Morbidity in children with frequently relapsing nephrosis: 10-year follow-up of a randomized controlled trial. Pediatric Nephrology 2015;30(3):459-68. [MEDLINE: ] - PubMed
ISKDC 1970 {published data only}
    1. Abramowicz M, Barnett HL, Edelmann CM Jr, Greifer I, Kobayashi O, Arneil GC, et al. Controlled trial of azathioprine in children with nephrotic syndrome. A report for the International Study of Kidney Disease in Children. Lancet 1970;1(7654):959-61. [MEDLINE: ] - PubMed
    1. Arneil GC. International trial of azathioprine in nephrotic syndrome in childhood [abstract]. Nephron 1971;8:95. [CENTRAL: CN-00602053]
ISKDC 1974 {published data only}
    1. Anonymous. Prospective, controlled trial of cyclophosphamide therapy in children with nephrotic syndrome. Report of the International Study of Kidney Disease in Children. Lancet 1974;304(7878):423-7. [MEDLINE: ] - PubMed
Mathew 2020 {published data only}
    1. Mathew G, Sinha A, Ahmed A, Grewal N, Khandelwal P, Hari P, et al. Efficacy of rituximab versus tacrolimus in difficult-to-treat steroid-sensitive nephrotic syndrome: an open-label pilot randomized controlled trial. Pediatric Nephrology 2022;37(12):3117‐26. [MEDLINE: ] - PMC - PubMed
    1. Mathew G, Sinha A, Grewal N, Ahmad A, Hari P, Bagga A. Efficacy and safety of rituximab versus tacrolimus in frequently relapsing nephrotic syndrome: an open label randomized controlled trial [abstract no: POS-457]. Kidney International Reports 2021;6(4 Suppl):S198-9. [EMBASE: 2011685164]
    1. Mathew G, Sinha A, Grewal N, Ahmed A, Hari P, Bagga A. Efficacy and safety of rituximab versus tacrolimus in frequently relapsing nephrotic syndrome: an open label randomized controlled trial with non-inferiority design [abstract no: ACPN210330P03]. Pediatric Nephrology 2021;36:2922.
    1. Mathew G, Sinha A, Grewal N, Ahmed A, Hari P, Bagga A. Efficacy and safety of rituximab versus tacrolimus in frequently relapsing nephrotic syndrome: an open-label randomized controlled trial [abstract no:S14]. Asian Journal of Pediatric Nephrology 2020;3(2):97.
    1. Mathew G, Sinha A, Grewal N, Ahmed A, NHari P, Bagga A. Efficacy and safety of rituximab versus tacrolimus in frequently relapsing nephrotic syndrome: An open label randomized controlled trial with non-inferiority design [abstract no: ACPN210330P]. Asian Pediatric Journal of Nephrology 2021;4(Abstract Suppl):28.
McCrory 1973 {published data only}
    1. McCrory WW, Shibuya M, Lu WH, Lewy JE. Therapeutic and toxic effects observed with different dosage programs of cyclophosphamide in treatment of steroid-responsive but frequently relapsing nephrotic syndrome. Journal of Pediatrics 1973;82(4):614-8. [MEDLINE: ] - PubMed
NEPHROVIR‐3 2022 {published and unpublished data}
    1. Dossier C, Kwon T, Chapelon E, Parmentier C, Galerne A, Chace A, et al. Efficacy of levamisole for maintaining remission after the first flare of steroid sensitive nephrotic syndrome in children: the NEPHROVIR-3 randomized controlled trial [abstract no: O-26]. Pediatric Nephrology 2023;38(7):2268. [DOI: 10.1007/s00467-022-05865-y] [EMBASE: 641737172] - DOI
    1. Dossier C, Kwon T, Chapelon E, Parmentier C, Galerne A, Chace A, et al. Efficacy of levamisole for maintaining remission after the first flare of steroid sensitive nephrotic syndrome in children: the NEPHROVIR-3 randomized controlled trial [abstract no: OP-39]. Pediatric Nephrology 2022;37(11):2818. [EMBASE: 639293210]
    1. Dossier C, Kwon T, Nekrouf C, Bensaid P, Parmentier C, Galerne A, et al. Efficacy of levamisole for maintaining remission after the first flare of steroid sensitive nephrotic syndrome in children: the NEPHROVIR-3 randomized controlled trial [abstract no: FC038]. Nephrology Dialysis Transplantation 2022;37(Suppl 3):i803. [EMBASE: 638263266]
NEPHRUTIX 2018 {published data only}
    1. Boumediene A, Vachin P, Sendeyo K, Oniszczuk J, Zhang SY, Henique C, et al. NEPHRUTIX: A randomized, double-blind, placebo vs rituximab-controlled trial assessing T-cell subset changes in minimal change nephrotic syndrome. Journal of Autoimmunity 2018;88:91-102. [MEDLINE: ] - PubMed
Niaudet 1992 {published data only}
    1. Niaudet P. Comparison of cyclosporin and chlorambucil in the treatment of steroid-dependent idiopathic nephrotic syndrome: a multicentre randomized controlled trial. The French Society of Paediatric Nephrology. Pediatric Nephrology 1992;6(1):1-3. [MEDLINE: ] - PubMed
Ponticelli 1989 {published and unpublished data}
    1. Edefonti A, Ghio L, Bettinelli A, Paterlini G, Giani M, Nebbia G, et al. Unconjugated hyperbilirubinemia due to ciclosporin administration in children with nephrotic syndrome. Contributions to Nephrology 1988;67:121-4. [MEDLINE: ] - PubMed
    1. Edefonti A, Ghio L, Bettinelli A, Paterlini G. Unconjugated hyperbilirubinemia due to cyclosporin A (CyA) administration in children with nephrotic syndrome (NS) [abstract no: F1.12]. Pediatric Nephrology 1987;1(4):C8. [CENTRAL: CN-00465774] - PubMed
    1. Edefonti A, Ghio L, Rizzoni G, Rinaldi S, Gusmano R, Lama G, et al. Cyclosporine (CSA) vs cyclophosphamide (CYC) for children with frequently relapsing/steroid dependent nephrotic syndrome (FR/SDNS): long term study [abstract]. Journal of the American Society of Nephrology 1992;3(3):310. [CENTRAL: CN-00460680]
    1. Edefonti A, Ghio L, Rizzoni G, Rinaldi S, Gusmano R, Lama G, et al. Cyclosporine (CSA) vs cyclophosphamide (CYC) for children with frequently relapsing/steroid dependent nephrotic syndrome: long term study [abstract]. In: 9th Congress of the International Pediatric Nephrology Association; 1992 Aug 30-Sep 4; Jerusalem, Israel. 1992:C70. [CENTRAL: CN-00483820]
    1. Ponticelli C, Edefonti A, Ghio L, Rizzoni G, Rinaldi S, Gusmano R, et al. Cyclosporin versus cyclophosphamide for patients with steroid-dependent and frequently relapsing idiopathic nephrotic syndrome: a multicentre randomized controlled trial. Nephrology Dialysis Transplantation 1993;8(12):1326-32. [MEDLINE: ] - PubMed
Prasad 2004 {published data only}
    1. Prasad N, Gulati S, Sharma RK, Singh U, Ahmed M. Pulse cyclophosphamide therapy in steroid-dependent nephrotic syndrome. Pediatric Nephrology 2004;19(5):494-8. [MEDLINE: ] - PubMed
Rashid 1996 {published data only}
    1. Rashid HU, Ahmed S, Fatima N, Khanam A. Levamisole in the treatment of steroid dependent or frequent relapsing nephrotic syndrome in children. Bangladesh Renal Journal 1996;15(1):6-8. [EMBASE: 26288912]
Ravani 2011 {published data only}
    1. Ravani P, Magnasco A, Edefonti A, Murer L, Rossi R, Ghio L, et al. Short-term effects of rituximab in children with steroid- and calcineurin-dependent nephrotic syndrome: a randomized controlled trial. Clinical Journal of the American Society of Nephrology: CJASN 2011;6(6):1308-15. [MEDLINE: ] - PMC - PubMed
Ravani 2015 {published data only}2008‐004486‐26
    1. Ravani P, Rossi R, Bonanni A, Quinn RR, Sica F, Bodria M, et al. Rituximab in children with steroid-dependent nephrotic syndrome: a multicenter, open-label, noninferiority, randomized controlled trial. Journal of the American Society of Nephrology 2015;26(9):2259-66. [MEDLINE: ] - PMC - PubMed
Ravani 2017 {published data only}2015‐000624‐28
    1. Angeletti A, Bruschi M, Lugani F, Colucci M, Vivarelli M, Emma F, et al. Antibodies anti-rituximab do not affect response to rituximab in idiopathic nephrotic syndrome [abstract no: SA-OR33]. Journal of the American Society of Nephrology 2021;32(Abstract Suppl):49. [EMBASE: 636329928]
    1. Bruschi M, Garbarino A, Kajana X, Spinelli S, Angeletti A, Lugani F, et al. Multi-omic serum profiling in children and young adults affected by steroid-dependent nephrotic syndrome [abstract no: FR-PO662]. Journal of the American Society of Nephrology 2023;34:584‐5. [DOI: 10.1681/ASN.20233411S1584d] [EMBASE: 642703044] - DOI
    1. Fribourg M, Cioni M, Ghiggeri G, Cantarelli C, Leventhal JS, Budge K, et al. CyTOF-enabled analysis identifies class-switched B cells as the main lymphocyte subset associated with disease relapse in children with idiopathic nephrotic syndrome. Frontiers in Immunology 2021;12:726428. [MEDLINE: ] - PMC - PubMed
    1. Ravani P, Bonanni A, Ghiggeri GM. Randomised controlled trial comparing ofatumumab to rituximab in children with steroid-dependent and calcineurin inhibitor-dependent idiopathic nephrotic syndrome: study protocol. BMJ Open 2017;7(3):e013319. [MEDLINE: ] - PMC - PubMed
    1. Ravani P, Colucci M, Bruschi M, Vivarelli M, Cioni M, Di Donato A, et al. Ofatumumab or rituximab for children with steroid-dependent nephrotic syndrome. A randomized controlled trial [abstract no: FC 131]. Nephrology Dialysis Transplantation 2021;36(Suppl 1):i89-90. [EMBASE: 635917754]
Ravani 2020 {published data only}2008‐004486‐26
    1. Ravani P, Lugani F, Pisani I, Bodria M, Piaggio G, Bartolomeo D, et al. Rituximab for very low dose steroid-dependent nephrotic syndrome in children: a randomized controlled study. Pediatric Nephrology 2020;35(8):1437-44. [MEDLINE: ] - PubMed
Ravani 2021 {published data only}
    1. Angeletti A, Ravani P, Ghiggeri GM. Inadequate dosage of mycophenolate mofetil is likely to yield substandard trial results and substandard patient care. JAMA Pediatrics 2021;175(8):870-1. [PMID: ] - PubMed
    1. Ravani P, Lugani F, Drovandi S, Caridi G, Angeletti A, Ghiggeri GM. Rituximab vs low-dose mycophenolate mofetil in recurrence of steroid-dependent nephrotic syndrome in children and young adults: a randomized clinical trial. JAMA Pediatrics 2021;175(6):631-2. [MEDLINE: ] - PMC - PubMed
RITURNS 2018 {published data only}2014/01/004355
    1. Basu A, Sander A, Preussler S, Sinha Mahapatra T, Schaefer F. Long-term efficacy of rituximab & MMF maintenance - therapy in childhood SDNS [abstract no: IPN11036-82]. Pediatric Nephrology 2019;34(10):2003.
    1. Basu B, Erdmann S, Sander A, Mahapatra TK, Meis J, Schaefer F. Long-term efficacy and safety of rituximab versus tacrolimus in children with steroid dependent nephrotic syndrome. KI Reports 2023;8(8):1575‐84. [DOI: 10.1016/j.ekir.2023.05.022] [PMID: ] - DOI - PMC - PubMed
    1. Basu B, Sander A, Roy B, Preussler S, Barua S, Mahapatra TK, et al. Efficacy of rituximab vs tacrolimus in pediatric corticosteroid-dependent nephrotic syndrome: a randomized clinical trial [Erratum in: JAMA Pediatr. 2018 Dec 1;172(12):1205]. JAMA Pediatrics 2018;172(8):757-64. [MEDLINE: ] - PMC - PubMed
    1. Schaefer F. Superior efficacy of rituximab vs tacrolimus in pediatric steroid dependent nephrotic syndrome [abstract]. In: 54th ERA-EDTA Congress; 2017 June 3-6; Madrid, Spain. 2017. [CENTRAL: CN-01657775]
Sawires 2019 {published data only}
    1. Sawires H, Abdelaziz H, Ahmed HM, Botrous O, Agban M. Randomized controlled trial on immunomodulatory effects of azithromycin in children with steroid-dependent nephrotic syndrome. Pediatric Nephrology 2019;34(9):1591-7. [MEDLINE: ] - PubMed
    1. Sawires H, Abdelaziz H, Mostafa O, Botrous O, Agban M. Randomized controlled trial on immunomodulatory effects of azithromycin in children with steroid dependent nephrotic syndrome [abstract no: IPN10189-90]. Pediatric Nephrology 2019;34(10):1933. - PubMed
Sinha 2019 {published data only}2012/02/002394
    1. Bagga A, Bhatia D, Puraswani M, Hari P, Sinha A. Randomized trial comparing efficacy & safety of mycophenolate mofetil and levamisole in frequently relapsing & steroid dependent nephrotic syndrome [abstract no: P-SUN199]. Pediatric Nephrology 2013;28(8):1599-600. [EMBASE: 71127678]
    1. Sinha A, Peruswami M, Rawat M, Hari P, Bagga A. Randomized controlled trial to compare the efficacy and safety of mycophenolate mofetil versus levamisole in children with frequently relapsing and steroid dependent nephrotic syndrome [abstract]. Pediatric Nephrology 2015;29(12):2434. [CENTRAL: CN-01912558]
    1. Sinha A, Puraswani M, Kalaivani M, Goyal P, Hari P, Bagga A. Efficacy and safety of mycophenolate mofetil versus levamisole in frequently relapsing nephrotic syndrome: an open-label randomized controlled trial. Kidney International 2019;95(1):2010-8. [MEDLINE: ] - PubMed
Sinha 2024 {published data only}2015/07/006002
    1. Sinha A, Devi KG, Kalra S, Mani K, Hari P, Bagga A. An open label non-inferiority randomized controlled trial evaluated alternate day prednisolone given daily during infections vs. levamisole in frequently relapsing nephrotic syndrome. Kidney International 2024;105(5):1113‐23. [DOI: 10.1016/j.kint.2024.01.028] [PMID: ] - DOI - PubMed
    1. Sinha A, Ghanapriya K, Hari P, Bagga A. Efficacy and safety of prednisolone on alternate days made daily with infections versus levamisole in frequently relapsing nephrotic syndrome: an open label randomized controlled trial [abstract no: Pos-462]. Kidney International Reports 2021;6(4 Suppl):S200-1. [EMBASE: 2011684297]
    1. Sinha A, Raut S, Ghanapriya K, Bhardwaj M, Kalra S, Hari P, et al. Alternate-day prednisolone made daily during infections versus levamisole for frequently-relapsing nephrotic syndrome [abstract no: IPN12086-88]. Pediatric Nephrology 2019;34(10):2171-2.
Su 2022 {published data only}
    1. Su Q, Qi X, Shen Y, Dou Z, Rong Z, Zhao X, et al. The efficacy and safety of rituximab in the treatment of steroid-dependent or frequently relapsing nephrotic syndrome in children. Hong Kong Journal of Paediatrics 2022;27(4):241-8. [EMBASE: 2018199526]
Sural 2001 {published data only}
    1. Sural S, Pahari DK, Mitra K, Bhattacharya S, Mondal S, Taraphder A. Efficacy of levamisole compared to cyclophosphamide and steroid in frequently relapsing (FR) minimal change nephrotic syndrome (MCNS) [abstract no: A0660]. Journal of the American Society of Nephrology 2001;12(Program & Abstracts):126A.
Tohjoh 1994 {published data only}
    1. Tohjoh S, Narita M, Koyama T, Miyahara M, Nagasawa T, Kitagawa T, et al. Clinical evaluation of cyclosporin in the treatment of nephrotic syndrome: multicentre double-blind study. Jin Tohseki 1994;37:565-608. [CENTRAL: CN-01912572]
Uddin 2016 {published data only}
    1. Uddin GM, Rahman MA, Rahman MH, Roy RR, Begum A, Huque SS. Comparative efficacy of mycophenolate mofetil and cyclosporine in children with frequent relapse nephrotic syndrome [abstract no: PO-276]. Pediatric Nephrology 2016;31(10):1852-3. [EMBASE: 612479647]
Ueda 1990 {published data only}
    1. Ueda N, Kuno K, Ito S. Eight and 12 week courses of cyclophosphamide in nephrotic syndrome. Archives of Disease in Childhood 1990;65(10):1147-59. [MEDLINE: ] - PMC - PubMed
Wang 2022 {published data only}
    1. Wang L, Zhu J, Xia M, Hua R, Deng F. Comparison of rituximab, cyclophosphamide, and tacrolimus as first steroid-sparing agents for complicated relapsing/steroid-dependent nephrotic syndrome in children: an evaluation of the health-related quality of life. Archives of Medical Science 2022;18(1):275-8. [PMID: ] - PMC - PubMed
Weiss 1993 {published and unpublished data}
    1. Weiss R. Results of a randomized, double-blind, placebo controlled, multi-center clinical trial of levamisole for the treatment of children with frequently relapsing or steroid dependent nephrotic syndrome. Personal communication 2005.
    1. Weiss R, NY, NJ, Phila Pediatric Nephrology Study Group. Randomized, double-blind, placebo (P) controlled trial of levamisole (L) for children (CH) with frequently relapsing/steroid dependant (FR/SD) nephrotic syndrome (NS) [abstract no: 98P]. Journal of the American Society of Nephrology 1993;4(Program & Abstracts):289. [CENTRAL: CN-00520404]
Yoshioka 2000 {published data only}
    1. Yoshioka K, Ohashi Y, Sakai T, Ito H, Yoshikawa N, Nakamura H, et al. A multicenter trial of mizoribine compared with placebo in children with frequently relapsing nephrotic syndrome. Kidney International 2000;58(1):317-24. [MEDLINE: ] - PubMed
    1. Yoshioka K, Ohashi Y, Sakai T, Ito H, Yoshikawa N, Nakamura H, et al. Placebo-controlled trial of mizoribine (MZR) in children with frequently relapsing nephrotic syndrome (FRNS). The Pediatric Mizoribine Study Group [abstract]. Journal of the American Society of Nephrology 1998;9(Program & Abstracts):163A. [CENTRAL: CN-00448484]
Zhang 2014 {published data only}
    1. Zhang B, Liu T, Wang W, Zhang X, Fan S, Liu Z, et al. A prospective randomly controlled clinical trial on azithromycin therapy for induction treatment of children with nephrotic syndrome. European Journal of Pediatrics 2014;173(4):509-15. [MEDLINE: ] - PubMed
    1. Zhang BL, Liu T. A prospective clinical randomized controlled trial on azithromycin therapy in induction treatment in children with nephrotic syndrome [abstract no: O-44]. Pediatric Nephrology 2013;28(8):1364. [EMBASE: 71126985]

References to studies excluded from this review

Arun 2009 {published data only}030600044
    1. Arun S, Bagga A, Bhatnagar S, Hari P, Menon S, Saini S. Efficacy of zinc (Zn) in reducing relapses in steroid sensitive nephrotic syndrome (SSNS): double blind, randomized controlled trial (RCT) (CRG030600044) [abstract no: 184]. Pediatric Nephrology 2007;22(9):1481. [CENTRAL: CN-00724915] - PubMed
    1. Arun S, Bhatnagar S, Menon S, Saini S, Hari P, Bagga A. Efficacy of zinc supplements in reducing relapses in steroid-sensitive nephrotic syndrome. Pediatric Nephrology 2009;24(8):1583-6. [MEDLINE: ] - PubMed
Basu 2015 {published data only}2013/10/004107
    1. Basu B, Pandey R, Mahapatra TK, Mondal N, Schaefer F. WITHDRAWN: Efficacy and safety of mycophenolate mofetil versus levamisole in children and adolescents with idiopathic nephrotic syndrome: results of a randomized clinical trial. American Journal of Kidney Diseases 2015 June 09;-:-. [MEDLINE: ] - PubMed
    1. Basu B, Pandey R, Mishra OP. WITHDRAWN: Randomized controlled trial to compare the efficacy & safety of mycophenolate mofetil vs levamisole in children with frequent relapsing & steroid dependent nephrotic syndrome [abstract no: O-18]. Pediatric Nephrology 2013;28(8):1353. [CENTRAL: CN-01658385]
    1. Basu B, Pandey R, Mondal N, Schaefer F. WITHDRAWN: Efficacy and safety of mycophenolate-mofetil vs levamisole in children with idiopathic nephrotic syndrome: results of a randomized clinical trial [abstract]. Nephrology Dialysis Transplantation 2014;29(Suppl 3):iii7. [EMBASE: 71491485]
Beige 2003 {published data only}
    1. Beige J, Moosmayer I, Liefeldt L, Neumayer HH, Zidek W, Peters H. Effective and safe treatment of primary nephrotic syndrome with tacrolimus (FK 506) [abstract no: M207]. Nephrology Dialysis Transplantation 2003;18(Suppl 4):65. [CENTRAL: CN-00444378]
Cerkauskiene 2004 {published data only}
    1. Cerkauskiene R, Jankauskiene A, Kaltenis P. Effect of omega-3 fatty acids on serum homocysteine in childhood steroid-sensitive nephrotic syndrome [abstract no: P011]. Pediatric Nephrology 2004;19(9):C95.
Dharmaraj 2009 {published data only}
    1. Dharmaraj R, Hari P, Bagga A. Randomized cross-over trial comparing albumin and frusemide infusions in nephrotic syndrome. Pediatric Nephrology 2009;24(4):775-82. [MEDLINE: ] - PubMed
    1. Hari P, Bagga A. Does albumin infusion augment frusemide induced diuresis in edematous children with nephrotic syndrome? [abstract no: M-PO-0834]. In: 4th World Congress of Nephrology.19th International Congress of the International Society of Nephrology (ISN); 2007 Apr 21-25; Rio de Janeiro, Brazil. 2007:285.
Fong 1997 {published data only}
    1. Fong GZ. Observation on refractory nephrotic syndrome with integrated traditional Chinese medicine (TCM) - Western medicine (WM) [abstract]. Nephrology 1997;3(Suppl 1):S125. [CENTRAL: CN-01912560]
Hu 2008a {published data only}
    1. Hu GH, Yi ZW, Wang JH, Yao JC. Effect of Triptergium wilfordii polyglycosidium on content of Th1 and Th2 in child recurrent nephrotic syndrome. Zhongguo Zhongyao Zazhi [China Journal of Chinese Materia Medica] 2008;33(4):441-3. [MEDLINE: ] - PubMed
Khemani 2016 {published data only}
    1. Khemani S, Moorani KN. Cyclosporine versus cyclophosphamide in childhood nephrotic syndrome. Journal of the Liaquat University of Medical & Health Sciences 2016;15(2):57-62. [EMBASE: 611436094]
Kirubakaran 1984 {published data only}
    1. Kirubakaran MG, Jacob GK, Date A, Shastry JC. A controlled trial of levamisole in frequently relapsing minimal change disease [abstract]. Kidney International 1984;26(2):240. [CENTRAL: CN-01912563]
Li 1994 {published data only}
    1. Li X, Li Z, Cheng Z. Treatment of children with simple nephrotic syndrom using prednison once per day. Acta Academiae Medicinae Hubei 1994;15(4):386-8. [EMBASE: 25005617]
Li 2005a {published data only}
    1. Li X, Tian J, Lin W, He X, Jiang H, Chen J. Mycophenolate mofetil therapy in primary nephrotic syndrome with hepatitis B virus infection [abstract no: F-PO1010]. Journal of the American Society of Nephrology 2005;16:557A. [CENTRAL: CN-01912562]
Liu 2016c {published data only}
    1. Liu Y, Qu X, Chen W, Zhang Y, Liu L. Efficacy of leflunomide combined with prednisone in the treatment of refractory nephrotic syndrome. Renal Failure 2016;38(10):1616-21. [MEDLINE: ] - PubMed
Lou 2004 {published data only}
    1. Lou T, Wang C, Chen Z, Tang H, Liu X, Yu X. Randomized controlled trial of leflunomide in the treatment of refractory nephrotic syndrome [abstract]. Journal of the American Society of Nephrology 2004;15:353A. [CENTRAL: CN-01912561]
Mazo 2022 {published data only}
    1. Mazo A, Pereira TE, Solomon S, Samsonov DV. Induction therapy with mycophenolate mofetil for steroid-sensitive nephrotic syndrome in children: a prospective single center pilot study [abstract no: SA-PO588]. Journal of the American Society of Nephrology 2022;33(Abstracts Suppl):766. [EMBASE: 639544789]
Naigui 1997 {published data only}
    1. Naigui X, Jian X, Jing H. A clinical study on low dose cyclosporin A with diltiazem in the treatment of primary nephrotic syndrome [abstract no: P203]. Nephrology 1997;3 Suppl(1):S125. [CENTRAL: CN-00461383]
NCT02390362 {published data only}
    1. NCT02390362. Randomized trial comparing rituximab against mycophenolate mofetil in children wtih refractory nephrotic syndrome (RAMP). www.clinicaltrials.gov/ct2/show/NCT02390362 (first received 17 March 2015).
NCT05599815 {published data only}
    1. NCT05599815. Part 1 - A clinical trial in patients with frequently relapsing and steroid-dependent nephrotic syndrome. clinicaltrials.gov/show/NCT05599815 (first received 21 October 2022).
NCT05627557 {published data only}
    1. NCT05627557. A study to evaluate the efficacy and safety of obinutuzumab versus MMF in participants with childhood onset idiopathic nephrotic syndrome (INShore) [A phase III, international, multicenter, randomised open label study to evaluate the efficacy and safety of obinutuzumab versus MMF in patients with childhood onset idiopathic nephrotic syndrome]. clinicaltrials.gov/show/NCT05627557 (first received 25 November 2022).
Ni 2003 {published data only}
    1. Ni ZH, Qian JQ, Lin AW, Mu S, Zhu ML, Fang W. A controlled, prospective study of efficacy of leflunomide in patients with nephrotic syndrome [abstract no: SA-PO1025]. Journal of the American Society of Nephrology 2003;14(Nov):524A. [CENTRAL: CN-00520368]
Nishiyama 1997 {published data only}
    1. Nishiyama J, Nakabayashi I, Moriya H, Kikuchi Y, Yoshizawa N. Effect of combined treatment with methylprednisolone pulse and nafamostat mesilate for nephrotic syndrome [abstract no: P221]. Nephrology 1997;3(Suppl 1):S130. [CENTRAL: CN-01912557]
Pecoraro 2003 {published data only}
    1. Pecoraro C, Caropreso MR, Malgieri G, Ferretti A, Nuzzi F. Therapy of first episode steroid responsive nephrotic syndrome (FESRNS): a randomised controlled trial [abstract no: MP087]. Nephrology Dialysis Transplantation 2005;20(Suppl 5):v230.
    1. Pecoraro C, Caropreso MR, Malgieri G, Ferretti AV, Raddi G, Piscitelli A, et al. Therapy of first episode of steroid responsive nephrotic syndrome: a randomised controlled trial [abstract no: OFC41]. Pediatric Nephrology 2004;19(9):C72. [CENTRAL: CN-00644160]
    1. Pecoraro C, Caropreso MR, Passaro G, Ferretti AV, Malgieri G. Therapy of first episode of steroid responsive nephrotic syndrome: a randomised controlled trial [abstract no: M199]. Nephrology Dialysis Transplantation 2003;18(Suppl 4):63. [CENTRAL: CN-00447140]
RITUXIVIG 2020 {published data only}2017‐000826‐36
    1. Hogan J, Perez A, Sellier-Leclerc AL, Vrillon I, Broux F, Nobili F, et al. Efficacy and safety of intravenous immunoglobulin with rituximab versus rituximab alone in childhood-onset steroid-dependent and frequently relapsing nephrotic syndrome: protocol for a multicentre randomised controlled trial. BMJ Open 2020;10(9):e037306. [MEDLINE: ] - PMC - PubMed
Rowe 1990 {published data only}
    1. Rowe PC, McLean RH, Ruley EJ, Salcedo JR, Baumgardner RA, Zaugg B, et al. Intravenous immunoglobulin in minimal change nephrotic syndrome: a crossover trial. Pediatric Nephrology 1990;4(1):32-5. [MEDLINE: ] - PubMed
Samuels 2002 {published data only}
    1. Samuels R, Mani UV, Iyer UM, Nayak US. Hypocholesterolemic effect of spirulina in patients with hyperlipidemic nephrotic syndrome. Journal of Medicinal Food 2002;5(2):91-6. [MEDLINE: ] - PubMed
Sancewicz‐Pach 1995 {published data only}
    1. Sancewicz-Pach K, Slowiaczek E, Kwinta-Rybicka J, Wilkosz K, Nowak J. Results for 8-week and 12-month cyclosporin A treatment in children with primary nephrotic syndrome [abstract]. In: XIIIth International Congress of Nephrology; 1995 Jul 2-6; Madrid, Spain. 1995:270.
Sasinka 1976 {published data only}
    1. Sasinka M, Plenta I, Kaiserova E, Izakovic V, Pavlovic M, CYTEMBENA/*THER USE, et al. Comparison of the effectiveness of cyclophosphamide and cytembena in controlled clinical trial in nephrotic syndrome and chronic glomerulonephritis [Porovnanie ucinnosti Cyclophosphamidu a Cytembeny v kontrolovanom klinickom pokuse pri nefrotickom syndrome a chronickej glomerulonefritide]. Ceskoslovenska Pediatrie 1976;31(3):127-30. [MEDLINE: ] - PubMed
Shendurnikar 2004 {published data only}
    1. Shendurnikar N, Agrawal N, Nayak US, Samuels R, Mani UV. Spirulina supplementation in pediatric nephrotic syndrome [abstract no: P051]. Pediatric Nephrology 2004;19(9):C105. [CENTRAL: CN-01912556]
Stavrovskaya 2001 {published data only}
    1. Stavrovskaya E, Neverov N, Shvetsov M, Tareeva I. Angiotensin-converting enzyme inhibitors (ACEI) and angiotensin 2 receptor blockers (ARB) attenuate hypercholesterolemia in patients with chronic nephrotic glomerulonephritis [abstract]. In: 38th Congress of the European Renal Association European Dialysis & Transplant Association; 2001 Jun 24-27; Vienna, Austria. 2001:96. [CENTRAL: CN-00461795]
Tejani 1988 {published data only}
    1. Tejani A, Gonzalez R, Rajpoot D, Sharma R, Pomrantz A. A randomized trial of cyclosporine with low-dose prednisone compared with high-dose prednisone in nephrotic syndrome. Transplantation Proceedings 1988;20(3 Suppl 4):262-4. [MEDLINE: ] - PubMed
    1. Tejani A, Suthanthiran M, Pomrantz A. A randomized controlled trial of low-dose prednisone and ciclosporin versus high-dose prednisone in nephrotic syndrome of children. Nephron 1991;59(1):96-9. [MEDLINE: ] - PubMed
    1. Tejani A, Suthanthiran M, Rajpoot D, Gonzalez R, Pomrantz A. Randomized trial of cylosporine and low dose prednisone (P&CS) vs conventional prednisone (P) therapy in recent onset (< 1 yr) nephrotic syndrome (NS) [abstract]. Kidney International 1989;35(1):213. [CENTRAL: CN-01912553]
Trompeter 1978 {published data only}
    1. Trompeter RS, Thomson PD, Barratt TM, Soothill JF. Controlled trial of disodium cromoglycate in prevention of relapse of steroid-responsive nephrotic syndrome of childhood. Archives of Disease in Childhood 1978;53(5):430-2. [MEDLINE: ] - PMC - PubMed
Urdaneta 1983 {published data only}
    1. Urdaneta Carruyo E, Zuniga Armendariz V, Gordillo Paniagua G. Therapeutic trial with levamisole in children with idiopathic minimal change nephrotic syndrome [Ensayo terapeutico con levamisol en ninos con sindrome nefrotico idiopatico de lesiones glomerulares minimas]. Boletin Medico del Hospital Infantil de Mexico 1983;40(2):82-8. [MEDLINE: ] - PubMed
Wang 2005 {published data only}
    1. Wang YP, Liu AM, Dai YW, Yang C, Tang HF. The treatment of relapsing primary nephrotic syndrome in children. Journal of Zhejiang University 2005;Science. B. 6(7):682-5. [MEDLINE: ] - PMC - PubMed
Wu 2015 {published data only}
    1. Wu B, Mao J, Shen H, Fu H, Wang J, Liu A, et al. Triple immunosuppressive therapy in steroid-resistant nephrotic syndrome children with tacrolimus resistance or tacrolimus sensitivity but frequently relapsing. Nephrology 2015;20(1):18-24. [MEDLINE: ] - PubMed
Yamashita 1971 {published data only}
    1. Yamashita F, Funatsu T, Nagayama K, Arihiro H, Anan S. Evaluation of alternate-day steroid therapy for nephrotic syndrome in childhood by cross-over study. Kurume Medical Journal 1971;18(3):153-60. [MEDLINE: ] - PubMed
Yi 2008 {published data only}
    1. Yi ZW, Wu XC, Xu H, Zhou LJ, Wu YB, Feng SP, et al. A prospective multicenter clinical control trial on treatment of refractory nephrotic syndrome with mycophenolate mofetil in children. Zhongguo Dangdai Erke Zazhi 2008;10(5):575-8. [MEDLINE: ] - PubMed
Zedan 2016 {published data only}
    1. Zedan MM, El-Refaey A, Zaghloul H, Abdelrahim ME, Osman A, Zedan MM, et al. Montelukast as an add-on treatment in steroid dependant nephrotic syndrome, randomised-controlled trial. Journal of Nephrology 2016;29(4):585-92. [MEDLINE: ] - PubMed
Zhang 2007d {published data only}
    1. Zhang Y, Huang JP, Xiao HJ, Ding J, Yao Y, Yang JY. Prospective clinical randomized controlled trial of pulse methylprednisolone therapy in children with steroid sensitive nephrotic syndrome [abstract no: 797]. Pediatric Nephrology 2007;22(9):1609. [CENTRAL: CN-01912552]
Zhi‐Hong 2004 {published data only}
    1. Zhi-Hong H, Ying D, Li Y. Effect of the astragalus injection on urine protein in children nephrotic syndrome [abstract]. Pediatric Nephrology 2004;19(9):C97.
Zhong 2007 {published data only}
    1. Zhong ZM, Yu L, Weng ZY, Hao ZH, Zhang L, Zhang YX, et al. Therapeutic effect of Ginkgo biloba leaf extract on hypercholestrolemia in children with nephrotic syndrome. Nanfang Yeie Daxue Xuebao [Journal of Southern Medical University] 2007;27(5):682-4. [MEDLINE: ] - PubMed
Zhu 2013 {published data only}
    1. Zhu S, Wu X. A study of leflunomide in treatment of juvenile refractory nephropathy syndrome [abstract no: P-SUN179]. Pediatric Nephrology 2013;28(8):1419. [EMBASE: 71127146]
Zou 1997 {published data only}
    1. Zou HQ, Wang YQ. Mechanism that Chinese traditional medicine reduces relapses of idiopathic nephrotic syndrome (INS) in children [abstract no: P198]. Nephrology 1997;3(Suppl 1):S124. [CENTRAL: CN-01912341]

References to studies awaiting assessment

Gaude 2022 {published data only}
    1. Gaude R, Devi A, Vacchani V, Mohapatra A. To study the efficacy of rituximab in frequently relapsing/steroid dependent nephrotic syndrome [abstract no: 87]. Indian Journal of Nephrology 2022;32(7):S64‐5. [EMBASE: 639938062]
Yang 2019 {published data only}
    1. Yang EM, Park E, Choi HJ, Hyun H, Park YH, Han KH, et al. Mycophenolate mofetil for maintenance of remission in children with steroid‑ and calcineurin inhibitor‑ dependent nephrotic syndrome: a prospective, randomized multicenter trial. Asian Journal of Pediatric Neprology 2019;2(2):82-8. [DOI: 10.4103/AJPN.AJPN_10_19] - DOI
Zhu 2023 {published data only}
    1. Zhu Y, Wu L, Wang Y, Zhu YF, Peng Y, Fang SH, et al. Efficacy and safety of low-dose rituximab in treatment of pediatric nephrotic syndrome: a prospective randomized controlled trial. Zhongguo Dangdai Erke Zazhi [Chinese Journal of Contemporary Pediatrics] 2023;25(6):606‐11. [DOI: 10.7499/j.issn.1008-8830.2301026] [PMID: ] - DOI - PMC - PubMed

References to ongoing studies

CTIS2023‐505140‐19‐00 {published data only}2023‐505140‐19‐00
    1. CTIS2023-505140-19-00. A study to evaluate the efficacy and safety of obinutuzumab versus MMF in participants with childhood onset idiopathic nephrotic syndrome. https://trialsearch.who.int/Trial2.aspx?TrialID=CTIS2023-505140-19-00 2024.
CTRI/2022/01/039125 {published data only}2022/01/039125
    1. CTRI/2022/01/039125. A clinical trial to study the efficacy of daily versus every-other day levamisole therapy in children with frequently relapsing nephrotic syndrome [Efficacy, safety and pharmacokinetics of daily versus every other day levamisole therapy in children aged 2-18 years with frequently relapsing or steroid dependent nephrotic syndrome: A prospective open label randomized trial with blinded end point assessment]. trialsearch.who.int/Trial2.aspx?TrialID=CTRI/2022/01/039125 (first received 21 November 2022).
CTRI/2022/01/039753 {published data only}2022/01/039753
    1. CTRI/2022/01/039753. Azithromycin in paediatric nephrotic syndrome [Effect of addition of azithromycin to routine treatment regime on time to remission in paediatric primary nephrotic syndrome]. trialsearch.who.int/Trial2.aspx?TrialID=CTRI/2022/01/039753 (first received 27 Jan 2022).
CTRI/2022/02/040186 {published data only}2022/02/040186
    1. CTRI/2022/02/040186. Rituximab maintenance therapy in nephrotic syndrome [Role of rituximab in maintaining long term remission of idiopathic steroid dependent and steroid resistant nephrotic syndrpme - RITUXIMAINS]. trialsearch.who.int/Trial2.aspx?TrialID=CTRI/2022/02/040186 (first received 10 February 2022).
CTRI/2022/10/046891 {published data only}2022/10/046891
    1. CTRI/2022/10/046891. Study comparing the ability of two medications, namely oral tablets of mycophenolate mofetil and cyclophosphamide, in keeping disease well controlled in patients with frequently relapsing or steroid dependent nephrotic syndrome [Efficacy and safety of mycophenolate mofetil versus oral cyclophosphamide in children with frequently relapsing or steroid dependent nephrotic syndrome: an open label randomized controlled trial]. trialsearch.who.int/Trial2.aspx?TrialID=CTRI/2022/10/046891 (first received 28 October 2022).
CTRI/2022/12/048452 {published data only}2022/12/048452
    1. CTRI/2022/12/048452. Comparison of a levamisole drug use in two different durations and dose in treatment of frequently relapsing nephrotic syndrome [Comparison of alternate day levamisole vs daily levamisole therapy in children with frequently relapsing nephrotic syndrome: an open labelled randomized controlled trial]. trialsearch.who.int/Trial2.aspx?TrialID=CTRI/2022/12/048452 (first received 26 December 2022).
CTRI/2023/09/058171 {published data only}2023/09/058171
    1. CTRI/2023/09/058171. A study to compare two medicines, mycophenolate mofetil and tacrolimus, to see which one works better in helping children with kidney problems called nephrotic syndrome stay healthy and not get sick again too often. trialsearch.who.int/Trial2.aspx?TrialID=CTRI/2023/09/058171 2023.
CTRI/2024/04/065552 {published data only}2024/04/065552
    1. CTRI/2024/04/065552. Study to determine whether two drugs are better than one in treatment of difficult to treat nephrotic syndrome. trialsearch.who.int/Trial2.aspx?TrialID=CTRI/2024/04/065552 2024.
ESOLAC 2022 {published data only}202201750782433
    1. PACTR202201750782433. Efficacy and safety of levamisole in African children with nephrotic syndrome: a protocol for a double-blinded, standard of care-controlled randomized trial (ESOLAC Trial) [Efficacy and safety of levamisole in frican children with idiopathic frequent relapsing and steroid dependent nephrotic syndrome: a protocol for a double-blinded, standard of care-controlled randomized trial (ESOLAC Trial)]. trialsearch.who.int/Trial2.aspx?TrialID=PACTR202201750782433 (first received 18 January 2022).
EUCTR2020‐004982‐37‐PL {published data only}2020‐004982‐37‐PL
    1. EUCTR2020-004982-37-PL. Early rituximab treatment in children with idiopathic nephrotic syndrome Eng. ERICONS - Early RITUXIMAB in Childhood Onset Nephrotic Syndrome [Early rituximab treatment in children with idiopathic nephrotic syndrome Eng. ERICONS - Early RITUXIMAB in Childhood Onset Nephrotic Syndrome - ERICONS]. trialsearch.who.int/Trial2.aspx?TrialID=EUCTR2020-004982-37-PL (first received 30 December 2020).
Hama 2018 {published data only}000005103
    1. Hama T, Nakanishi K, Ishikura K, Ito S, Nakamura H, Sako M, et al. Study protocol: high-dose mizoribine with prednisolone therapy in short-term relapsing steroid-sensitive nephrotic syndrome to prevent frequent relapse (JSKDC05 trial). BMC Nephrology 2018;19(1):223. [MEDLINE: ] - PMC - PubMed
INTENT 2018 {published data only}000065472014‐001991‐76
    1. Benz MR, Doetsch J, Ehren R, Gellermann J, Haffner D, Hoyer PF, et al. Protocol of an open, randomized, controlled, multicenter trial on the initial treatment of idiopathic steroid-sensitive nephrotic syndrome in children with mycophenolate mofetil vs. prednisone: the Intent Study [abstract no: P176]. Pediatric Nephrology 2014;29(9):1740. [EMBASE: 71662573]
    1. Ehren R, Benz MR, Doetsch J, Fichtner A, Gellermann J, Haffner D, et al. Initial treatment of steroid-sensitive idiopathic nephrotic syndrome in children with mycophenolate mofetil versus prednisone: protocol for a randomised, controlled, multicentre trial (INTENT study). BMJ Open 2018;8(10):e024882. [MEDLINE: ] - PMC - PubMed
    1. Tonshoff B. Initial treatment of idiopathic nephrotic syndrome in children with mycophenolate mofetil vs. prednisone: a randomized, controlled, multicenter study (INTENT Study): Clinical study protocol: Version 4.0/20150325. www.intent-study.de/.cm4all/iproc.php/Studienprotokoll_INTENT_V4_2015_03... (accessed 7 October 2024).
JSKDC10 2019 {published data only}IIA00380
    1. Nagano C, Sako M, Kamei K, Ishikura K, Nakamura H, Nakanishi K, et al. Study protocol: multicenter double-blind, randomized, placebo-controlled trial of rituximab for the treatment of childhood-onset early-stage uncomplicated frequently relapsing or steroid-dependent nephrotic syndrome (JSKDC10 trial). BMC Nephrology 2019;20(1):293. [MEDLINE: ] - PMC - PubMed
LEARNS 2019 {published data only}2017‐001025‐416826
    1. Veltkamp F, Khan DH, Reefman C, Veissi S, Oers HA, Levtchenko E, et al. Prevention of relapses with levamisole as adjuvant therapy in children with a first episode of idiopathic nephrotic syndrome: study protocol for a double blind, randomised placebo-controlled trial (the LEARNS study). BMJ Open 2019;9(8):e027011. [MEDLINE: ] - PMC - PubMed
    1. Veltkamp F, Reefman C, Khan DH, Veissi S, Haverman L, Mathot RA, et al. Prevention of relapses with levamisole as adjuvant therapy to corticosteroids in children with a first episode of idiopathic nephrotic syndrome – an international, double blind, placebo-controlled randomized trial [abstract no: O-327]. Pediatric Nephrology 2018;33(10):1938.
Lugani 2021 {published data only}
    1. Lugani F, Angeletti A, Ravani P, Vivarelli M, Colucci M, Caridi G, et al. Randomised controlled trial comparing rituximab to mycophenolate mofetil in children and young adults with steroid dependent idiopathic nephrotic syndrome: study protocol. BMJ Open 2021;11(11):e052450. [PMID: ] - PMC - PubMed
NCT02972346 {published data only}
    1. NCT02972346. Availability study of ACTH to treat children SRNS/SDNS. clinicaltrials.gov/show/NCT02972346 (first received 23 November 2016).
NCT04048161 {published data only}
    1. NCT04048161. Study of tacrolimus vs mycophenolate mofetil in pediatric patients with nephrotic syndrome (STAMP) [Study of tacrolimus vs mycophenolate mofetil in pediatric patients with frequently relapsing or steroid dependent nephrotic syndrome: a randomized, multicenter, open-label, parallel-arm study]. clinicaltrials.gov/show/NCT04048161 (first received 7 Aug 2019).
NCT05786768 {published data only}2022‐003336‐59
    1. NCT05786768. Efficacy and safety of obinutuzumab versus rituximab in childhood steroid dependant and frequent relapsing nephrotic syndrome. clinicaltrials.gov/show/NCT05786768 2023.
NCT05843968 {published data only}
    1. NCT05843968. Efficacy and safety of rituximab versus mycophenolate mofetil in children with steroid-dependent nephrotic syndrome. clinicaltrials.gov/ct2/show/NCT05843968 2023.
NCT05914155 {published data only}2041230037
    1. NCT05914155. Clinical study of rituximab for the treatment for idiopathic membranous nephropathy with nephrotic syndrome (PRIME) [The multi-center, randomized, double-blind, placebo-controlled study to evaluate the efficacy and safety of rituximab (genetical recombination) for the treatment for idiopathic membranous nephropathy with nephrotic syndrome (PRIME Study)]. clinicaltrials.gov/study/NCT05914155 2023.
NCT06079788 {published data only}
    1. NCT06079788. Study of adrenocorticotropic hormone on children with frequent relapse or steroid-dependent nephrotic syndrome: a prospective, multicenter, randomized, open-label clinical trial. clinicaltrials.gov/ct2/show/NCT06079788 2023.
RITURNS II 2020 {published data only}2019/04/018517
    1. Basu B, Preussler S, Sander A, Mahapatra TK, Schaefer F. Randomized clinical trial to compare efficacy and safety of repeated courses of rituximab to single-course rituximab followed by maintenance mycophenolate-mofetil in children with steroid dependent nephrotic syndrome. BMC Nephrology 2020;21(1):520. [MEDLINE: ] - PMC - PubMed
UMIN000004204 {published data only}031180132000004204
    1. UMIN000004204. A multi-center randomized controlled trial of tacrolimus versus cyclosporine for frequently relapsing nephrotic syndrome in children (JSKDC06). trialsearch.who.int/Trial2.aspx?TrialID=JPRN-UMIN000004204 (first received 10 January 2010).

Additional references

Arneil 1961
    1. Arneil GC. 164 children with nephrosis. Lancet 1961;2(7212):1103-10. [MEDLINE: ] - PubMed
Chan 2020
    1. Chan, EY, Webb H, Yu E, Ghiggeri GM, Kemper MJ, Ma AL, et al. Both the rituximab dose and maintenance immunosuppression in steroid-dependent/frequently-relapsing nephrotic syndrome have important effects on outcomes. Kidney International 2020;97(2):393-401. [PMID: ] - PubMed
Coutinho 2001
    1. Coutinho HM, Groothoff JW, Offringa M, Gruppen MP, Heymans HS. De novo malignancy after paediatric renal replacement therapy. Archives of Disease in Childhood 2001;85(6):478-83. [MEDLINE: ] - PMC - PubMed
Crowther 2010
    1. Crowther CA, Crosby DD. Phenobarbital prior to preterm birth for preventing neonatal periventricular haemorrhage. Cochrane Database of Systematic Reviews 2010, Issue 1. Art. No: CD000164. [DOI: 10.1002/14651858.CD000164.pub2] - DOI - PMC - PubMed
El Bakkali 2011
    1. El Bakkali L, Rodrigues Pereira R, Kuik DJ, Ket JC, Wijk JA. Nephrotic syndrome in The Netherlands: a population-based cohort study and a review of the literature. Pediatric Nephrology 2011;26(8):1241-6. [MEDLINE: ] - PMC - PubMed
Fairley 1972
    1. Fairley KF, Barrie JU, Johnson W. Sterility and testicular atrophy related to cyclophosphamide therapy. Lancet 1972;1(7750):568-9. [MEDLINE: ] - PubMed
French NS Guideline 2008
    1. Haute Autorité de Santé. Idiopathic nephrotic syndrome in children. National protocol for diagnosis and treatment for a rare disease [Syndrome néphrotique idiopathique de l’enfant. Protocole national de diagnostic et de soins pour une maladie rare]. www.has-sante.fr/portail/upload/docs/application/pdf/2008-06/pnds_sni_en... (accessed prior to 4 March 2020).
Gipson 2009
    1. Gipson DS, Massengill SF, Yao L, Nagaraj S, Smoyer WE, Mahan JD, et al. Management of childhood onset nephrotic syndrome. Pediatrics 2009;124(2):747-57. [MEDLINE: ] - PubMed
GRADE 2008
    1. Guyatt GH, Oxman AD, Vist GE, Kunz R, Falck-Ytter Y, Alonso-Coello P, et al. GRADE: an emerging consensus on rating quality of evidence and strength of recommendations. BMJ 2008;336(7650):924-6. [MEDLINE: ] - PMC - PubMed
GRADE 2011
    1. Guyatt G, Oxman AD, Akl EA, Kunz R, Vist G, Brozek J, et al. GRADE guidelines: 1. Introduction-GRADE evidence profiles and summary of findings tables. Journal of Clinical Epidemiology 2011;64(4):383-94. [MEDLINE: ] - PubMed
Hahn 2024
    1. Hahn D, Samuel SM, Willis NS, Craig JC, Hodson EM. Corticosteroid therapy for nephrotic syndrome in children. Cochrane Database of Systematic Reviews 2024, Issue 8. Art. No: CD001533. [DOI: 10.1002/14651858.CD001533.pub7] - DOI - PMC - PubMed
Higgins 2003
    1. Higgins JP, Thompson SG, Deeks JJ, Altman DG. Measuring inconsistency in meta-analyses. BMJ 2003;327(7414):557-60. [MEDLINE: ] - PMC - PubMed
Higgins 2022
    1. Higgins JP, Thomas J, Chandler J, Cumpston M, Li T, Page MJ, et al. Cochrane Handbook for Systematic Reviews of Interventions version 6.3 (updated February 2022). Cochrane, 2022. Available from www.training.cochrane.org/handbook.
Hogg 2003
    1. Hogg R, Fitzgibbons L, Bruick J, Bunke M, Ault B, Baqi N, et al. Multicenter trial of mycophenolate mofetil (MMF) in children with steroid dependent (SD) or frequent relapsing (FR) nephrotic syndrome (NS). Report of the Southwest Pediatric Nephrology Study Group [abstract]. Nephrology Dialysis Transplantation 2003;18(Suppl 4):261.
IPNA 2023
    1. Trautmann A, Boyer O, Hodson E, Bagga A, Gipson DS, Samuel S, et al. IPNA clinical practice recommendations for the diagnosis and management of children with steroid-sensitive nephrotic syndrome. Pediatric Nephrology 2023;38(3):877-919. [PMID: ] - PMC - PubMed
IPNS‐IAP 2021
    1. Sinha A, Bagga A, Banerjee S, Mishra K, Mehta A, Agarwal I, et al. Steroid sensitive nephrotic syndrome: revised guidelines. Indian Pediatrics 2021;58(5):461-81. [PMID: ] - PMC - PubMed
ISKDC 1978
    1. Anonymous. Nephrotic syndrome in children: prediction of histopathology from clinical and laboratory characteristics at time of diagnosis. A report of the International Study of Kidney Disease in Children. Kidney International 1978;13(2):159-65. [MEDLINE: ] - PubMed
Japanese Guideline 2014
    1. Nishi S, Ubara Y, Utsunomiya Y, Okada K, Obata Y, Kai H, et al. Evidence-based clinical practice guidelines for nephrotic syndrome 2014. Clinical & Experimental Nephrology 2014;20(3):342-70. [MEDLINE: ] - PMC - PubMed
KDIGO 2021
    1. Improving Global Outcomes (KDIGO) Glomerular Diseases Work Group. KDIGO 2021 clinical practice guideline for the management of glomerular diseases. Kidney International 2021;100(4S):S1–276. [PMID: ] - PubMed
Kemper 2001
    1. Kemper M, Wolf G, Müller-Wiefel D. Transmission of glomerular permeability factor from a mother to her child. New England Journal of Medicine 2001;344(5):386-7. [MEDLINE: ] - PubMed
Koskimies 1982
    1. Koskimies O, Vilska J, Rapola J, Hallman N. Long-term outcome of primary nephrotic syndrome. Archives of Disease in Childhood 1982;57(7):544-8. [MEDLINE: ] - PMC - PubMed
McKinney 2001
    1. McKinney PA, Feltbower RG, Brocklebank JT, Fitzpatrick MM. Time trends and ethnic patterns of childhood nephrotic syndrome in Yorkshire, UK. Pediatric Nephrology 2001;16(12):1040-4. [MEDLINE: ] - PubMed
Moher 1998
    1. Moher D, Pham B, Jones A, Cook DJ, Jadad AR, Moher M, et al. Does quality of reports of randomised trials affect estimates of intervention efficacy reported in meta-analyses? Lancet 1998;352(9128):609-13. [MEDLINE: ] - PubMed
Noone 2018
    1. Noone DG, Iijima K, Parekh R. Idiopathic nephrotic syndrome in children. Lancet 2018;392(10143):61-74. [MEDLINE: ] - PubMed
Queshi 1972
    1. Queshi MS, Pennington JH, Goldsmith HJ, Cox PE. Cyclophosphamide therapy and sterility. Lancet 972;2(7790):1290-1. [MEDLINE: ] - PubMed
Rapola 1973
    1. Rapola J, Koskimies O, Huttunen NP, Floman P, Vilska J, Hallman N. Cyclophosphamide and the pubertal testis. Lancet 1973;1(7794):98-9. [MEDLINE: ] - PubMed
Savin 1996
    1. Savin VJ, Sharma J, Sharma M, McCarthy ET, Swan SK, Ellis E, et al. Circulating factor associated with increased glomerular permeability to albumin in recurrent focal segmental glomerulosclerosis. New England Journal of Medicine 1996;354(14):878-83. [MEDLINE: ] - PubMed
Schlesinger 1968
    1. Schlesinger ER, Sultz HA, Mosher WE, Feldman JG. The nephrotic syndrome: its incidence and implications for the community. American Journal of Diseases of Children 1968;116(6):623-32. [MEDLINE: ] - PubMed
Schulz 1995
    1. Schulz KF, Chalmers I, Hayes RJ, Altman DG. Empirical evidence of bias. Dimensions of methodological quality associated with estimates of treatment effects in controlled trials. JAMA 1995;273(5):408-12. [MEDLINE: ] - PubMed
Schünemann 2022a
    1. Schünemann HJ, Higgins JP, Vist GE, Glasziou P, Akl EA, Skoetz N, et al. Chapter 14: Completing ‘Summary of findings’ tables and grading the certainty of the evidence. In: Higgins JP, Thomas J, Chandler J, Cumpston M, Li T, Page MJ, Welch VA (editors). Cochrane Handbook for Systematic Reviews of Interventions version 6.3 (updated February 2022). Cochrane, 2022. Available from www.training.cochrane.org/handbook.
Schünemann 2022b
    1. Schünemann HJ, Vist GE, Higgins JP, Santesso N, Deeks JJ, Glasziou P, et al. Chapter 15: Interpreting results and drawing conclusions. In: Higgins JP, Thomas J, Chandler J, Cumpston M, Li T, Page MJ, Welch VA (editors). Cochrane Handbook for Systematic Reviews of Interventions version 6.3 (updated February 2022). Cochrane, 2022. Available from www.training.cochrane.org/handbook.
SINePe Consensus 2017
    1. Pasini A, Benetti E, Conti G, Ghio L, Lepore M, Massella L, et al. The Italian Society for Pediatric Nephrology (SINePe) consensus document on the management of nephrotic syndrome in children: Part I - Diagnosis and treatment of the first episode and the first relapse. Italian Journal of Pediatrics 2017;43(1):41. [MEDLINE: ] - PMC - PubMed
Sinha 2006
    1. Sinha MD, MacLeod R, Rigby E, Clark AG. Treatment of severe steroid-dependent nephrotic syndrome (SDNS) in children with tacrolimus. Nephrology Dialysis Transplantation 2006;21(7):1848-54. [MEDLINE: ] - PubMed
Sinha 2023
    1. Sinha A, Mathew G, Arushi A, Govindarajan S, Ghanapriya K, Grewal N, et al. Sequential rituximab therapy sustains remission of nephrotic syndrome but carries high risk of adverse effects. Nephrology Dialysis Transplantation 2023;38(4):939-49. [PMID: ] - PubMed
Sureshkumar 2014
    1. Sureshkumar P, Hodson EM, Willis NS, Barzi F, Craig JC. Predictors of remission and relapse in idiopathic nephrotic syndrome: a prospective cohort study. Pediatric Nephrology 2014;29(6):1039-46. [MEDLINE: ] - PubMed
Tarshish 1997
    1. Tarshish P, Tobin JN, Bernstein J, Edelmann CM Jr. Prognostic significance of the early course of minimal change nephrotic syndrome: report of the International Study of Kidney Disease in Children. Journal of the American Society of Nephrology 1997;8(5):769-76. [MEDLINE: ] - PubMed
Wang 2012
    1. Wang W, Xia Y, Mao J, Chen Y, Wang D, Shen H, et al. Treatment of tacrolimus or cyclosporine A in children with idiopathic nephrotic syndrome. Pediatric Nephrology 2012;27(11):2073-9. [MEDLINE: ] - PubMed
Wong 2007
    1. Wong W. Idiopathic nephrotic syndrome in New Zealand children, demographic, clinical features, initial management and outcome after twelve-month follow-up: results of a three-year national surveillance study. Journal of Paediatrics & Child Health 2007;43(5):337-41. [MEDLINE: ] - PubMed

References to other published versions of this review

Durkan 2000
    1. Durkan A, Hodson EM, Willis NS, Craig JC. Non-corticosteroid treatment for nephrotic syndrome in children. Cochrane Database of Systematic Reviews 2000, Issue 1. Art. No: CD002290. [DOI: 10.1002/14651858.CD002290] - DOI - PubMed
Durkan 2001a
    1. Durkan A, Hodson EM, Willis NS, Craig JC. Non-corticosteroid treatment for nephrotic syndrome in children. Cochrane Database of Systematic Reviews 2001, Issue 4. Art. No: CD002290. [DOI: 10.1002/14651858.CD002290] - DOI - PubMed
Durkan 2001b
    1. Durkan AM, Hodson EM, Willis NS, Craig JC. Immunosuppressive agents in childhood nephrotic syndrome: a meta-analysis of randomized controlled trials. Kidney International 2001;59(5):1919-27. [MEDLINE: ] - PubMed
Durkan 2005
    1. Durkan A, Hodson EM, Willis NS, Craig JC. Non-corticosteroid treatment for nephrotic syndrome in children. Cochrane Database of Systematic Reviews 2005, Issue 2. Art. No: CD002290. [DOI: 10.1002/14651858.CD002290.pub2] - DOI - PubMed
Hodson 2008
    1. Hodson EM, Willis NS, Craig JC. Non-corticosteroid treatment for nephrotic syndrome in children [Non-corticosteroid treatment for nephrotic syndrome in children]. Cochrane Database of Systematic Reviews 2008, Issue 1. Art. No: CD002290. [DOI: 10.1002/14651858.CD002290.pub3] - DOI - PubMed
Larkins 2020
    1. Larkins NG, Liu ID, Willis NS, Craig JC, Hodson EM. Non-corticosteroid immunosuppressive medications for steroid-sensitive nephrotic syndrome in children. Cochrane Database of Systematic Reviews 2020, Issue 4. Art. No: CD002290. [DOI: 10.1002/14651858.CD002290.pub5] - DOI - PMC - PubMed
Pravitsitthikul 2013
    1. Pravitsitthikul N, Willis NS, Hodson EM, Craig JC. Non-corticosteroid immunosuppressive medications for steroid-sensitive nephrotic syndrome in children. Cochrane Database of Systematic Reviews 2013, Issue 10. Art. No: CD002290. [DOI: 10.1002/14651858.CD002290.pub4] - DOI - PubMed

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