Mycophenolate mofetil and prednisolone therapy in children with steroid-dependent nephrotic syndrome

2003 ◽  
Vol 42 (6) ◽  
pp. 1114-1120 ◽  
Author(s):  
Arvind Bagga ◽  
Pankaj Hari ◽  
Asha Moudgil ◽  
Stanley C Jordan
Keyword(s):  
Nephrotic Syndrome ◽  
Mycophenolate Mofetil ◽  
Prednisolone Therapy ◽  
Steroid Dependent ◽  
Steroid Dependent Nephrotic Syndrome

Mycophenolate Mofetil after Rituximab for Childhood-onset Complicated Frequently-relapsing or Steroid-dependent Nephrotic Syndrome

2021 ◽  
pp. ASN.2021050643
Author(s):  
Kazumoto Iijima ◽  
Mayumi Sako ◽  
Mari Oba ◽  
Seiji Tanaka ◽  
Riku Hamada ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Mycophenolate Mofetil ◽  
Treatment Failure ◽  
Treatment Period ◽  
Immunosuppressive Agents ◽  
Steroid Resistance ◽  
Rank Test ◽  
Childhood Onset ◽  
Steroid Dependent ◽  
Steroid Dependent Nephrotic Syndrome

Background. Rituximab is the standard therapy for childhood-onset complicated frequently-relapsing or steroid-dependent nephrotic syndrome (FRNS/SDNS). However, most patients redevelop FRNS/SDNS following peripheral B cell recovery. Methods. We conducted a multicenter, randomized, double-blind, placebo-controlled trial to examine whether mycophenolate mofetil (MMF) administration after rituximab can prevent treatment failure (FRNS, SDNS, steroid resistance, or use of immunosuppressive agents or rituximab). Thirty-nine patients (per group) were treated with rituximab, followed by either MMF or placebo until Day 505 (treatment period). The primary outcome was time to treatment failure (TTF) throughout the treatment and follow-up periods (until Day 505 for the last enrolled patient). Results. TTFs were clinically but not statistically significantly longer among patients given MMF after rituximab than among patients receiving rituximab monotherapy (median: 784.0 vs. 472.5 days, hazard ratio (HR): 0.593, 95% confidence interval (CI): 0.336-1.049, log-rank test: P=0.0694). Because most patients in the MMF group presented with treatment failure after MMF discontinuation, we performed a post-hoc analysis limited to the treatment period and found that MMF after rituximab prolonged the TTF and decreased the risk of treatment failure by 80% (HR: 0.202, 95% CI: 0.081-0.503). Moreover, MMF after rituximab reduced the relapse rate and daily steroid dose during the treatment period by 74% and 57%, respectively. The frequency and severity of adverse events were similar in both groups. Conclusions. Administration of MMF after rituximab may sufficiently prevent the development of treatment failure and is well tolerated, although the relapse-preventing effect disappears after MMF discontinuation.

scholarly journals The safety and efficacy of mycophenolate mofetil in children and adolescents with steroid-dependent nephrotic syndrome: a single-centre study

2019 ◽  
Vol 13 (2) ◽  
pp. 179-183
Author(s):  
Saravanakumar Karunamoorthy ◽  
Dineshkumar Thanigachalam ◽  
Dhanapriya Jeyachandran ◽  
Sakthirajan Ramanathan ◽  
Gopalakrishnan Natarajan ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Mycophenolate Mofetil ◽  
Median Duration ◽  
Significant Risk ◽  
Duration Of Treatment ◽  
Minimum Period ◽  
Steroid Dependent ◽  
Long Term Treatment ◽  
Steroid Dependent Nephrotic Syndrome

Abstract Background Steroid-dependent nephrotic syndrome (SDNS) patients experience frequent relapse or adverse effects on long-term treatment with steroids or cyclophosphamide. This study assessed the efficacy and side effect profile of mycophenolate mofetil (MMF) therapy in children with nephrotic syndrome in our population. Methods A retrospective study was performed on children with SDNS who were on MMF therapy for a minimum period of 1 year, and were on regular follow-up in the Department of Nephrology at the Institute of Child Health and hospital for children attached to Madras Medical College. Results The study included 87 patients, with a male:female ratio of 2:1. The median age at diagnosis of nephrotic syndrome was 3 years [95% confidence interval (CI): 1–8 years], which was found to be a statistically significant risk factor for MMF failure. The median duration of follow-up after initiation of MMF therapy was 3 years and 3 months (95% CI: 1 year and 3 months to 6 years and 6 months). At initial evaluation, 31 (36%) patients presented with SDNS while the remaining had frequently relapsing nephrotic syndrome progressing to SDNS. Intravenous cyclophosphamide was used as first-line therapy in 82 patients, of whom 24 patients had persistent proteinuria while the remaining 58 had attained remission for a median duration of 6 months. The median duration of treatment with MMF was 2 years and 6 months (95% CI: 1 year and 3 months to 4 years and 6 months). MMF was used at a mean dose of 28.5 mg/kg. Seventy-two (83%) patients were MMF-sensitive, and these patients had a reduction in mean prednisolone dose from 1.28 to 0.35 mg/kg (P < 0.05). Among the MMF-sensitive patients, 31 had stopped MMF after a minimum period of 2 years, following which they had a median remission period of 5 months (95% CI: 1–8 months). MMF failure occurred in 15 (17%) patients. Adverse events were documented in 19 (22%) patients. Conclusions Continuous MMF therapy achieved remission in 83% of patients. MMF was well tolerated in the study population and discontinuation of MMF resulted in 100% relapse.

Outcome of severe steroid-dependent nephrotic syndrome treated with mycophenolate mofetil

2012 ◽  
Vol 28 (1) ◽  
pp. 93-97 ◽  
Author(s):  
Sushmita Banerjee ◽  
Amitava Pahari ◽  
Jayati Sengupta ◽  
Saroj K. Patnaik
Keyword(s):  
Nephrotic Syndrome ◽  
Mycophenolate Mofetil ◽  
Steroid Dependent ◽  
Steroid Dependent Nephrotic Syndrome

scholarly journals Safety and efficacy of mycophenolate mofetil as first-line immunosuppressive therapy in children with steroid-dependent nephrotic syndrome

2020 ◽  
Vol 23 (6) ◽  
pp. 360-364
Author(s):  
Asmik G. Agaronyan ◽  
Tatyana V. Vashurina ◽  
Svetlana V. Dmitrienko ◽  
Tatyna S. Voznesenskaya ◽  
Olga V. Komarova ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Mycophenolate Mofetil ◽  
Cumulative Dose ◽  
Term Therapy ◽  
First Line ◽  
First Line Therapy ◽  
Line Therapy ◽  
Steroid Dependent ◽  
Steroid Dependent Nephrotic Syndrome

Introduction. More than half of children with idiopathic nephrotic syndrome have relapsed or become steroid-dependent after the disease’s onset. So far they, require long-term therapy with glucocorticosteroids and/or other immunosuppressive treatment to maintain remission. Despite the widespread use of immunosuppressive agents, there is no consensus for appropriate first-line therapy. Materials and methods. A retrospective study included children presenting with steroid-dependent nephrotic syndrome who received treatment with mycophenolate mofetil in the Nephrology department between 2003 and 2018 at the National Medical Research Center for Children’s Health (Moscow). Results. In total, withdrawal of corticosteroids was recorded in 24 (85.7%) patients, while the median remission period was 23 [12; 30] months, after glucocorticosteroids cessation - 19 [10; 24.5] months, the cumulative dose of steroids was reduced from 189 [125.9; 240] to 45.5 [19.5; 68.2] mg/kg per year and relapses rate per year from 1.9 [1.35; 2.35] to 0 during a year. Among children who did not overcome steroid dependence, the treatment failed in 3 patients, and in 1 child, the steroid dosage has been reduced to the lower dose. Conclusion. Mycophenolate mofetil has a favorable efficacy due to the significant reduction of the cumulative dose of steroids and maintenance of long-term remission of nephrotic syndrome after glucocorticosteroid cessation and may be recommended as the first-line therapy in children with steroid-dependent nephrotic syndrome.

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