scholarly journals Efficacy and side effects of mycophenolate mofetil therapy in children with steroid dependent nephrotic syndrome in a tertiary paediatric nephrology centre in Sri Lanka

2019 ◽  
Vol 48 (1) ◽  
pp. 53 ◽  
Author(s):  
H N S Hettiarachchi ◽  
M Raja ◽  
U I Karunadasa ◽  
R S Thalgahagoda
Keyword(s):  
Nephrotic Syndrome ◽  
Mycophenolate Mofetil ◽  
Side Effects ◽  
Sri Lanka ◽  
Paediatric Nephrology ◽  
Steroid Dependent ◽  
Steroid Dependent Nephrotic Syndrome

scholarly journals Rituximab Use in Nephrotic Syndrome

2018 ◽  
Vol 3 (1) ◽  
Keyword(s):  
Nephrotic Syndrome ◽  
Mycophenolate Mofetil ◽  
Side Effects ◽  
Calcineurin Inhibitor ◽  
Minimal Change ◽  
Steroid Resistant ◽  
Steroid Resistant Nephrotic Syndrome ◽  
Steroid Dependent ◽  
Minimal Change Glomerulonephritis ◽  
Steroid Dependent Nephrotic Syndrome

Background: Nephrotic syndrome may be caused by primary (idiopathic) renal disease or by a variety of secondary causes. Patients present with marked edema, proteinuria, hypoalbuminemia, and often hyperlipidemia. Treatment of most patients should include fluid and sodium restriction, oral or intravenous diuretics, and angiotensin-converting enzyme inhibitors. Adults with nephrotic syndrome may benefit from corticosteroid treatment. The treatment of patients with the steroid-resistant nephrotic syndrome (SRNS) and steroid-dependent nephrotic syndrome (SDNS) is challenging. On the basis of suggestions that B lymphocytes are crucial in the pathogenesis of the nephrotic syndrome, rituximab (a monoclonal antibody against CD20 antigen) is used in treatment of these patients. Aim of study: To evaluate the role of rituximaband mycophenolic acid in treatment of patientswith steroid-resistant (SRNS) and steroid-dependent nephrotic syndrome (SDNS), whom not respond or relapse after calcineurin inhibitor (CNI) (tacrolimus or cyclosporine) had been used. Patients and methods: Case series study was done between 2012 - 2015 in AL-Sadder Teaching Hospital Nephrology Center and record 40 patients with different age groups, males and females with different histopathological types (Minimal Change Glomerulonephritis, Focal Segmental Glomerulosclerosis, Mesengeo Prolifrative Glomerulonephritis). These patients were taking prednisilone and\or calcineurin inhibitor (tacrolimus “prograf”) or (cyclosporine “sandimmune”), and they get either Steroid Dependent Nephrotic Syndrome or Steroid Resistant Nephrotic Syndrome with frequent admission more than four time per year. To these patients we start rituximab intravenous infusion monthly for at least six months with the use of steroid and mycophenolate mofetil during these six months. The patients followed up for 3-12 months after initiation of rituximab by different investigations and the patients were classified according to their response into complete, partial and no response. After one year stop rituximab treatment, follow the patients clinically and by investigations for (1-2) years to determine which patients get relapse. Results: Majority (80%) of patients with nephrotic syndrome who had good response to rituximab were younger age group < 15 years. Better response to rituximab associated with Minimal Change Glomerulonephritis. There was significant reduction in blood urea, serum creatinine, urine (protein/creatinine) ratio and serum cholesterol. Serum albumin was significant elevated. Response to rituximab was not significantly associated with gender or steroid response. Majority of patients with good response not relapse and need more time for follow up. Relapsing after stopping rituximab not significantly associated with age, gender, histopathological type and steroid response. Conclusion: Rituximab and mycophenolate mofetil used in steroid-resistant nephrotic syndrome to get ride from side effects of calcineurine inhibitor (tacrolimus or cyclosporine). Rituximab and mycophenolate mofetil used in steroid-dependent nephrotic syndrome after calcineurine inhibitorto get ride from side effects of steroid. Improvement in renal function is result from stopping of calcineurine inhibitor (nephrotoxic drugs) and/or from rituximab and mycophenolate mofetil. Cost of rituximab is less than the cost that needed if the patients had frequent admissions to the hospital or developed renal failure and ended with dialysis.

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 &lt; 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

Therapeutic Effect of Chai-Ling-Tang (Sairei-to) on the Steroid-dependent Nephrotic Syndrome in Children

1995 ◽  
Vol 23 (03n04) ◽  
pp. 255-260 ◽  
Author(s):  
Xiao-Yun Liu
Keyword(s):  
Nephrotic Syndrome ◽  
Side Effects ◽  
Toxic Effect ◽  
Cytotoxic Agents ◽  
Short Term ◽  
Prednisone Dosage ◽  
Steroid Dependent ◽  
Steroid Dependent Nephrotic Syndrome ◽  
Negative Conversion

37 children with steroid-dependent nephrotic syndrome (SDNS) were administered with Chai-Ling-Tang (Sairei-to) under corticosteroid. After treatment with Chai-Ling-Tang, relapse was markedly improved, time for negative conversion of proteinuria shortened, prednisone dosage significantly reduced, and side effects eased. 32 children with SDNS treated with prednisone and cyclophosphamide served as control. Results showed that short-term and long-term relapse and average prednisone dosage were similar between these two groups. It is considered that Chai-Ling-Tang may be a useful substitute for patients with SDNS who fail to respond to or manifest severe toxic effect from cytotoxic agents.

scholarly journals Adrenocorticotropic Hormone for Childhood Nephrotic Syndrome

2018 ◽  
Vol 13 (12) ◽  
pp. 1859-1865 ◽  
Author(s):  
Chia-shi Wang ◽  
Curtis Travers ◽  
Courtney McCracken ◽  
Traci Leong ◽  
Rasheed Gbadegesin ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Side Effects ◽  
Confidence Interval ◽  
Adrenocorticotropic Hormone ◽  
Control Group ◽  
Disease Relapse ◽  
Steroid Dependent ◽  
Acth Treatment ◽  
First Relapse ◽  
Steroid Dependent Nephrotic Syndrome

Background and objectivesThere is renewed interest in adrenocorticotropic hormone (ACTH) for the treatment of nephrotic syndrome. We evaluated the efficacy and safety of ACTH in children with frequently relapsing or steroid-dependent nephrotic syndrome in a randomized trial.Design, setting, participants, & measurementsParticipants aged 2–20 years old with frequently relapsing or steroid-dependent nephrotic syndrome were enrolled from 16 sites in the United States and randomized 1:1 to ACTH (repository corticotropin injection) or no relapse-preventing treatment. ACTH treatment regimen was 80 U/1.73 m2 administered twice weekly for 6 months, followed by 40 U/1.73 m2 administered twice weekly for 6 months. The primary outcome was disease relapse during the first 6 months. Participants in the control group were offered crossover to ACTH treatment if they relapsed within 6 months. Secondary outcomes were relapse after ACTH dose reduction and treatment side effects.ResultsThe trial was stopped at a preplanned interim analysis after enrollment of 31 participants because of a lack of discernible treatment efficacy. Fourteen out of 15 (93%) participants in the ACTH arm experienced disease relapse in the first 6 months, with a median time to first relapse of 23 days (interquartile range, 9–32), compared with 15 out of 16 (94%) participants and at a median of 21 days (interquartile range, 14–51) in the control group. There was no difference in the proportion of relapsed patients (odds ratio, 0.93; 95% confidence interval, 0.05 to 16.40; P>0.99) or time to first relapse (hazard ratio, 1.03; 95% confidence interval, 0.50 to 2.15; P=0.93). Thirteen out of 16 participants in the control group crossed over to ACTH treatment. Three out of 28 participants completed 12 months of ACTH treatment; the others exited the trial because of frequent relapses or side effects. There were no disease relapses after ACTH dose reduction among the three participants. Most side effects were mild and similar to side effects of corticosteroids.ConclusionsACTH at 80 U/1.73 m2 administered twice weekly was ineffective at preventing disease relapses in pediatric nephrotic syndrome.

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