Research Article
BibTex RIS Cite

Mycophenolate Mofetil Treatment in Childhood Steroid-sensitive Nephrotic Syndrome: Single Center Experience

Year 2022, Volume: 16 Issue: 1, 32 - 36, 28.01.2022
https://doi.org/10.12956/tchd.817363

Abstract

Objective: We aimed to investigate the efficacy of mycophenolate mofetil (MMF) for maintaining remission and reduce the number of relapses in childhood steroid-sensitive nephrotic syndrome. The effects of MMF on growth and blood pressure parameters were also evaluated.

Material and Methods: This retrospective, single-center observational study included patients with steroid-sensitive nephrotic syndrome who were treated using MMF between 2009 and 2019 in the Department of Pediatric Nephrology in our hospital.


Results:
Ten patients had steroid-dependent nephrotic syndrome; six patients frequently had relapsing nephrotic syndrome in this study. The mean duration of the disease was 93.3 ± 25.0 months and the mean duration of the MMF onset was 33.9 ± 16.7 months after diagnosis. Ten patients showed a 50% or greater reduction in the relapse rate and the prednisolone treatment was discontinued in eight patients for six months or more. Compared to the previous year, before the start of the MMF treatment, there was a 52.7% reduction in the relapse rate and a 36.6% reduction in the cumulative annual dose of steroid after 12 months of MMF treatment. The height z score and the median office systolic blood pressure standard deviation scores of the patients improved after MMF treatment (respectively p = 0.003, p = 0.01).


Conclusion:
The findings suggest that MMF may lead to decreased relapse rates and cumulative steroid dose, which has a positive effect on growth and blood pressure parameters in steroid-sensitive nephrotic syndrome.

References

  • 1. MacHardy N, Miles PV, Massengill SF, et al. Management patterns of childhood-onset nephrotic syndrome. Pediatr Nephrol 2009;24:2193-201.
  • 2. Hodson EM, Knight JF, Willis NS, Craig JC. Corticosteroid therapy for nephrotic syndrome in children. Cochrane Database Syst Rev 2005;25:CD001533.
  • 3. Hoyer PF, Brodehl J. Initial treatment of idiopathic nephrotic syndrome in children: prednisone versus prednisone plus cyclosporine A—a prospective, randomized trial. J Am Soc Nephrol 2006;17:1151–7.
  • 4. van Husen M, Kemper MJ. New therapies in steroid sensitive and steroid-resistant idiopathic nephrotic syndrome. Pediatr Nephrol 2011;26:881–92.
  • 5. Hogg RJ, Fitzgibbons L, Bruick J, et al. Mycophenolate mofetil in children with frequently relapsing nephrotic syndrome: a report from the Southwest Pediatric Nephrology Study Group. Clin J Am Soc Nephrol 2006;1:1173-8.
  • 6. Dorresteijn EM, Kist-van Holthe JE, Levtchenko EN, Nauta J, Hop WC, van der Heijden AJ. Mycophenolate mofetil versus cyclosporine for remission maintenance in nephrotic syndrome. Pediatr Nephrol 2008;23:2013–20.
  • 7. Habashy D, Hodson E, Craig J. Interventions for idiopathic steroid-resistant nephrotic syndrome in children. Cochrane Database Syst Rev 2004;(2):CD003594.
  • 8. Baudouin V, Alberti C, Lapeyraque A-L, et al. Mycophenolate mofetil for steroiddependent nephrotic syndrome: a phase II Bayesian trial. Pediatr Nephrol 2012;27:389–96.
  • 9. Barletta GM, Smoyer WE, Bunchman TE, Flynn JT, Kershaw DB. Use of mycophenolate mofetil in steroid-dependent and -resistant nephrotic syndrome. Pediatr Nephrol 2003;18:833–7.
  • 10. Bansal SB, Saxena V, Pokhariyal S, et al. Comparison of azathioprine with mycophenolate mofetil in a living donor kidney transplant programme. Indian J Nephrol 2011;21:258–63.
  • 11. Abeyagunawardena AS, Dillon MJ, Rees L, van’t Hoff W, Trompeter RS. The use of steroid-sparing agents in steroid sensitive nephrotic syndrome. Pediatr Nephrol 2003; 18:919–24.
  • 12. Fujinaga S, Ohtomo Y, Umino D, et al. A prospective study on the use of MMF in children with cyclosporine dependent nephrotic syndrome. Pediatr Nephrol 2007;22:71–6.
  • 13. Gellermann J, Weber L, Pape L, Tönshoff B, Hoyer P, Querfeld U. Mycophenolate mofetil versus cyclosporin A in children with frequently relapsing nephrotic syndrome. J Am Soc Nephrol 2003;24:1689–97.
  • 14. Afzal K, Bagga A, Menon S, Hari P, Jordan SC. Treatment with mycophenolate mofetil and prednisolone for steroid-dependent nephrotic syndrome. Pediatr Nephrol 2007;22:2059–65.
  • 15. Al-Akash S, Al-Makdama A. Mycophenolate mofetil in children with steroid-dependent and/or frequently relapsing nephrotic syndrome. Ann Saudi Med 2005;25: 380–4.
  • 16. Novak I, Frank R, Vento S, Vergara M, Gauthier B, Trachtman H. Efficacy of mycophenolate mofetil in pediatric patients with steroid-dependent nephrotic syndrome. Pediatr Nephrol 2005;20:1265–8.
  • 17. Hassan AV, Sinha MD, Waller S. A single-centre retrospective study of the safety and efficacy of mycophenolate mofetil in children and adolescents with nephrotic syndrome. Clin Kidney J 2013;6:385–9.
  • 18. Banerjee S, Pahari A, Sengupta J, Patnaik SK. Outcome of severe steroid-dependent nephrotic syndrome treated with mycophenolate mofetil. Pediatr Nephrol 2013;28: 93–7.
  • 19. Bagga A, Hari P, Moudgil A, Jordan SC. Mycophenolate mofetil and prednisolone therapy in children with steroid dependent nephrotic syndrome. Am J Kidney Dis 2003;42:1114–20.
  • 20. Gellermann J, Querfeld U. Frequently relapsing nephrotic syndrome: treatment with mycophenolate mofetil. Pediatr Nephrol 2004;19:101–4.

Çocukluk Çağı Steroid Duyarlı Nefrotik Sendromda Mikofenolat Mofetil Tedavisi: Tek Merkez Deneyimi

Year 2022, Volume: 16 Issue: 1, 32 - 36, 28.01.2022
https://doi.org/10.12956/tchd.817363

Abstract

Amaç: Mikofenolat mofetilin çocukluk çağı steroide duyarlı nefrotik sendromda remisyonu sürdürme ve relaps sayısını azaltmadaki etkinliğinin belirlemesi amaçlandı. Ayrıca MMF’in büyüme ve kan basıncı üzerindeki etkileri değerlendirildi.

Gereç ve Yöntemler: Bu retrospektif, tek merkezli gözlemsel çalışma, 2009-2019 yılları arasında hastanemiz çocuk nefroloji kliniğinde MMF ile tedavi edilen steroide duyarlı nefrotik sendromlu hastaları içermektedir.

Bulgular: Çalışmamızdaki 10 hasta steroide bağımlı nefrotik sendrom, 6 hasta sık relaps nefrotik sendromdu. Ortalama hastalık süresi 93.3 ± 25.0 ay ve tanıdan sonra ortalama MMF başlangıç süresi 33.9 ± 16.7 aydı. On hastanın nüks oranında %50 veya daha fazla azalma görüldü ve prednizolon tedavisi 8 hastada 6 ay veya daha uzun süre kesildi. Bir önceki yıla kıyasla, 12 aylık MMF tedavisinden sonra nüks oranında %52,7 ve yıllık kümülatif steroid dozunda %36.6 azalma oldu. Mikofenolat mofetil tedavisi sonrası hastaların boy z skoru ve ortanca ofis sistolik kan basıncı standart sapma skorları iyileşti (sırasıyla p = 0.003, p = 0.01).

Sonuç: Mikofenolat mofetil, steroid duyarlı nefrotik sendromda nüks oranlarını, kümülatif steroid dozunu azaltarak büyüme ve kan basıncı ölçümleri üzerine olumlu etkilere neden olur.

References

  • 1. MacHardy N, Miles PV, Massengill SF, et al. Management patterns of childhood-onset nephrotic syndrome. Pediatr Nephrol 2009;24:2193-201.
  • 2. Hodson EM, Knight JF, Willis NS, Craig JC. Corticosteroid therapy for nephrotic syndrome in children. Cochrane Database Syst Rev 2005;25:CD001533.
  • 3. Hoyer PF, Brodehl J. Initial treatment of idiopathic nephrotic syndrome in children: prednisone versus prednisone plus cyclosporine A—a prospective, randomized trial. J Am Soc Nephrol 2006;17:1151–7.
  • 4. van Husen M, Kemper MJ. New therapies in steroid sensitive and steroid-resistant idiopathic nephrotic syndrome. Pediatr Nephrol 2011;26:881–92.
  • 5. Hogg RJ, Fitzgibbons L, Bruick J, et al. Mycophenolate mofetil in children with frequently relapsing nephrotic syndrome: a report from the Southwest Pediatric Nephrology Study Group. Clin J Am Soc Nephrol 2006;1:1173-8.
  • 6. Dorresteijn EM, Kist-van Holthe JE, Levtchenko EN, Nauta J, Hop WC, van der Heijden AJ. Mycophenolate mofetil versus cyclosporine for remission maintenance in nephrotic syndrome. Pediatr Nephrol 2008;23:2013–20.
  • 7. Habashy D, Hodson E, Craig J. Interventions for idiopathic steroid-resistant nephrotic syndrome in children. Cochrane Database Syst Rev 2004;(2):CD003594.
  • 8. Baudouin V, Alberti C, Lapeyraque A-L, et al. Mycophenolate mofetil for steroiddependent nephrotic syndrome: a phase II Bayesian trial. Pediatr Nephrol 2012;27:389–96.
  • 9. Barletta GM, Smoyer WE, Bunchman TE, Flynn JT, Kershaw DB. Use of mycophenolate mofetil in steroid-dependent and -resistant nephrotic syndrome. Pediatr Nephrol 2003;18:833–7.
  • 10. Bansal SB, Saxena V, Pokhariyal S, et al. Comparison of azathioprine with mycophenolate mofetil in a living donor kidney transplant programme. Indian J Nephrol 2011;21:258–63.
  • 11. Abeyagunawardena AS, Dillon MJ, Rees L, van’t Hoff W, Trompeter RS. The use of steroid-sparing agents in steroid sensitive nephrotic syndrome. Pediatr Nephrol 2003; 18:919–24.
  • 12. Fujinaga S, Ohtomo Y, Umino D, et al. A prospective study on the use of MMF in children with cyclosporine dependent nephrotic syndrome. Pediatr Nephrol 2007;22:71–6.
  • 13. Gellermann J, Weber L, Pape L, Tönshoff B, Hoyer P, Querfeld U. Mycophenolate mofetil versus cyclosporin A in children with frequently relapsing nephrotic syndrome. J Am Soc Nephrol 2003;24:1689–97.
  • 14. Afzal K, Bagga A, Menon S, Hari P, Jordan SC. Treatment with mycophenolate mofetil and prednisolone for steroid-dependent nephrotic syndrome. Pediatr Nephrol 2007;22:2059–65.
  • 15. Al-Akash S, Al-Makdama A. Mycophenolate mofetil in children with steroid-dependent and/or frequently relapsing nephrotic syndrome. Ann Saudi Med 2005;25: 380–4.
  • 16. Novak I, Frank R, Vento S, Vergara M, Gauthier B, Trachtman H. Efficacy of mycophenolate mofetil in pediatric patients with steroid-dependent nephrotic syndrome. Pediatr Nephrol 2005;20:1265–8.
  • 17. Hassan AV, Sinha MD, Waller S. A single-centre retrospective study of the safety and efficacy of mycophenolate mofetil in children and adolescents with nephrotic syndrome. Clin Kidney J 2013;6:385–9.
  • 18. Banerjee S, Pahari A, Sengupta J, Patnaik SK. Outcome of severe steroid-dependent nephrotic syndrome treated with mycophenolate mofetil. Pediatr Nephrol 2013;28: 93–7.
  • 19. Bagga A, Hari P, Moudgil A, Jordan SC. Mycophenolate mofetil and prednisolone therapy in children with steroid dependent nephrotic syndrome. Am J Kidney Dis 2003;42:1114–20.
  • 20. Gellermann J, Querfeld U. Frequently relapsing nephrotic syndrome: treatment with mycophenolate mofetil. Pediatr Nephrol 2004;19:101–4.
There are 20 citations in total.

Details

Primary Language English
Subjects ​Internal Diseases
Journal Section ORIGINAL ARTICLES
Authors

Tülin Güngör 0000-0002-5881-1565

Fehime Kara Eroğlu 0000-0003-2364-4282

Evrim Kargın Çakıcı 0000-0002-1697-6206

Fatma Yazılıtaş 0000-0001-6483-8978

Eda Didem Kurt-sukur This is me 0000-0003-1451-4443

Gökçe Can This is me 0000-0002-5881-1565

Semanur Özdel 0000-0003-2695-2045

Evra Çelikkaya This is me 0000-0003-2695-2045

Deniz Karakaya This is me 0000-0001-7720-4923

Mehmet Bülbül 0000-0001-9007-9653

Publication Date January 28, 2022
Submission Date October 28, 2020
Published in Issue Year 2022 Volume: 16 Issue: 1

Cite

Vancouver Güngör T, Kara Eroğlu F, Kargın Çakıcı E, Yazılıtaş F, Kurt-sukur ED, Can G, Özdel S, Çelikkaya E, Karakaya D, Bülbül M. Mycophenolate Mofetil Treatment in Childhood Steroid-sensitive Nephrotic Syndrome: Single Center Experience. Türkiye Çocuk Hast Derg. 2022;16(1):32-6.


The publication language of Turkish Journal of Pediatric Disease is English.


Manuscripts submitted to the Turkish Journal of Pediatric Disease will go through a double-blind peer-review process. Each submission will be reviewed by at least two external, independent peer reviewers who are experts in the field, in order to ensure an unbiased evaluation process. The editorial board will invite an external and independent editor to manage the evaluation processes of manuscripts submitted by editors or by the editorial board members of the journal. The Editor in Chief is the final authority in the decision-making process for all submissions. Articles accepted for publication in the Turkish Journal of Pediatrics are put in the order of publication, with at least 6 original articles in each issue, taking into account the acceptance dates. If the articles sent to the reviewers for evaluation are assessed as a senior for publication by the reviewers, the section editor and the editor considering all aspects (originality, high scientific quality and citation potential), it receives publication priority in addition to the articles assigned for the next issue.


The aim of the Turkish Journal of Pediatrics is to publish high-quality original research articles that will contribute to the international literature in the field of general pediatric health and diseases and its sub-branches. It also publishes editorial opinions, letters to the editor, reviews, case reports, book reviews, comments on previously published articles, meeting and conference proceedings, announcements, and biography. In addition to the field of child health and diseases, the journal also includes articles prepared in fields such as surgery, dentistry, public health, nutrition and dietetics, social services, human genetics, basic sciences, psychology, psychiatry, educational sciences, sociology and nursing, provided that they are related to this field. can be published.