Abstract
Treating children with steroid-resistant nephrotic syndrome (SRNS) has been a clinical challenge for pediatricians. We recruited 24 children (18 boys and six girls) with steroid-resistant idiopathic nephrotic syndrome (SRINS) who were <2 years. All patients were administered prednisone 2 mg/kg per day prior to mycophenolate mofetil (MMF). By the end of the eighth week, MMF was initiated at 25–30 mg/kg daily for 6− 12 months. Prednisone dose was reduced stepwise. Biochemical assays were performed every 2 months. Complete remission was achieved in 15 patients, partial remission in six, and no response to MMF was noted in three. With MMF treatment, the levels of urinary protein and serum cholesterol decreased and that of serum albumin increased in a time-dependant manner. We demonstrated the MMF could reduce proteinuria in SRINS children <2 years. Our study suggests that MMF therapy might be an effective strategy for treating SRINS in children <2 years.
Similar content being viewed by others
References
El-Husseini A, El-Basuony F, Mahmoud I, Donia A (2004) Co-administration of cyclosporine and ketoconazole in idiopathic childhood nephrosis. Pediatr Nephrol 19:976–981
Ejaz I, Khan HI, Javaid BK, Rasool G (2004) Histopathological diagnosis and outcome of paediatric nephrotic syndrome. J Coll Physicians Surg Pak 14:229–233
Sorof JM, Hawkins EP, Brewer ED, Boydstun II, Kale AS, Powell DR (1998) Age and ethnicity affect the risk and outcome of focal segmental glomeruloslcerosis. Pediatr Neprhol 12:764–768
Okada M, Hino S, Takemura T, Fukushima K (1999) Cyclosporin therapy in children with steroid-resistant nephrotic syndrome. Clin Exp Nephrol 3:S34–S39
Allison AC, Eugui EM (1993) Immunosuppressive and other effects of mycophenolic acid ester prodrug, Mycophenolate mofetil. Immunol Rev 136:5–28
Barletta G, Smoyer W, Bunchman T, Flynn J, Kershaw D (2003) Use of Mycophenolate Mofetil in steroid-dependent and –resistant nephrotic syndrome. Pediatr Nephrol 18:833–837
Okada M, Sugimoto K, Yagi K, Yanagida H, Tabata N, Takemura T (2007) Mycophenolate mofetil therapy for children with intractable nephrotic syndrome. Pediatr Int 49:933–937
Bagga A, Hari P, Moudgil A, Jordan SC (2003) Mycophenolate mofetil and prednisolone therapy in children with steroid-dependent nephrotic syndrome. Am J Kidney Dis 42:1114–1120
Afzal K, Bagga A, Menon S, Hari P, Jordan SC (2007) Treatment with mycophenolate mofetil and prednisolone for steroid-dependent nephrotic syndrome. Pediatr Nephrol 22:2059–2065
Moudgil A, Bagga A, Jordan SC (2005) Mycophenolate mofetil therapy in frequently relapsing steroid-dependent and steroid-resistant nephrotic syndrome of childhood: current status and future directions. Pediatr Nephrol 20:1376–1381
Dorresteijn EM, Kist-van Holthe JE, Levtchenko EN, Nauta J, Hop WC, van der Heijden AJ (2008) Mycophenolate mofetil versus cyclosporine for remission maintenance in nephrotic syndrome. Pediatr Nephrol 23:2013–2020
Fujinaga S, Ohtomo Y, Umino D, Takemoto M, Shimizu T, Yamashiro Y, Kaneko K (2007) A prospective study on the use of mycophenolate mofetil in children with cyclosporine-dependent nephrotic syndrome. Pediatr Nephrol 22:71–76
Novak I, Frank R, Vento S, Vergara M, Gauthier B, Trachtman H (2005) Efficacy of mycophenolate mofetil in pediatric patients with steroid-dependent nephrotic syndrome. Pediatr Nephrol 20:1265–1268
Mendizábal S, Zamora I, Berbel O, Sanahuja MJ, Fuentes J, Simon J (2005) Mycophenolate mofetil in steroid/cyclosporine-dependent/resistant nephrotic syndrome. Pediatr Nephrol 20:914–919
Ulinski T, Dubourg L, Saïd MH, Parchoux B, Ranchin B, Cochat P (2005) Switch from cyclosporine A to mycophenolate mofetil in nephrotic children. Pediatr Nephrol 20:482–485
Gellermann J, Querfeld U (2004) Frequently relapsing nephrotic syndrome: treatment with mycophenolate mofetil. Pediatr Nephrol 19:101–104
Chandra M, Susin M, Abitbol C (2000) Remission of relapsing childhood nephrotic syndrome with mycophenolate mofetil. Pediatr Nephrol 14:224–226
Peña A, Bravo J, Melgosa M, Fernandez C, Meseguer C, Espinosa L, Alonso A, Picazo ML, Navarro M (2007) Steroid-resistant nephrotic syndrome: long-term evolution after sequential therapy. Pediatr Nephrol 22:1875–1888
Montané B, Abitbol C, Chandar J, Strauss J, Zilleruelo G (2003) Novel therapy of focal glomerulosclerosis with mycophenolate and angiotensin blockade. Pediatr Nephrol 18:772–777
International Study of Kidney Disease in Children (1981) The primary nephrotic syndrome in children. Identification of patients with minimal change nephrotic syndrome from initial response to prednisone. J Pediatr 98:561–564
Srivastava T, Simon SD, Alon US (1999) High incidence of focal segmental glomerulosclerosis in nephrotic syndrome of childhood. Pediatr Nephrol 13:13–18
McBryde KD, Kershaw DB, Smoyer WE (2001) Pediatric steroid-resistant nephrotic syndrome. Curr Probl Pediatr Adolesc Health Care 31:280–307
Feng SP, Huang JP, Xu M, Li S, Luo W, Lai XJ (2007) Clinical presentations and pathologic features of 25 cases of infant nephrotic syndrome. Zhongguo Dang Dai Er Ke Za Zhi 9:151–152
Yu L, Wen ZY, Hua SD, Zhang YX, Huang YH, Meiying Z, Xiaoshu Y (2002) Analysis of clinical characteristics in infantile nephrotic syndrome. Chinese Journal of Practical Pediatrics 17:553–555
Wenyan H, Mingchang H, Xingyou J, Yi C (1995) Nephrotic syndrome in infants below 3-year-old. Acta Universitatis Medicinalis Nanjing 15:326–327
Fangyun S, Xingyu H, Yingqing G, Moyi G, Xiurong Z (1994) Long term follow-up of nephrotic syndrome in children under 2 years. Chin J Pediatr 32:276–278
Wang JG, Zhou ZL, Pan T, Li CC, Yang Q (2002) Integrated traditional Chinese and Western medicine for infantile nephrotic syndrome-150 cases report. Shanghai Journal of Traditional Chinese Medicine 2:27–29
Tune BM, Kirpekar R, Sibley RK, Reznik VM, Griswold WR, Mendoza SA (1995) Intravenous methylprednisolone and oral alkylating agent therapy of prednisone-resistant pediatric focal segmental glomerulosclerosis: a long-term follow-up. Clin Nephrol 43:84–88
Griswold WR, Tune BM, Reznik VM, Vazquez M, Prime DJ, Brock P, Mendoza SA (1987) Treatment of childhood prednisone-resistant nephrotic syndrome and focal segmental glomerulosclerosis with intravenous methylprednisolone and oral alkylating agents. Nephron 46:73–77
Goonasekera CD, Koziell AB, Hulton SA, Dillon MJ (1998) Vincristine and focal segmental sclerosis: do we need a multicentre trial? Pediatr Nephrol 12:284–289
Almeida MP, Almeida HA, Rosa FC (1994) Vincristine in steroid-resistant nephrotic syndrome. Pediatr Nephrol 8:79–80
Gregory MJ, Smoyer WE, Sedman A, Kershaw DB, Valentini RP, Johnson K, Bunchman TE (1996) Long-term cyclosporine therapy for pediatric nephrotic syndrome: a clinical and histologic analysis. J Am Soc Nephrol 7:543–549
Cattran DC, Appel GB, Hebert LA, Hunsicker LG, Pohl MA, Hoy WE, Maxwell DR, Kunis CL; North America Nephrotic Syndrome Study Group (2001) Cyclosporine in patients with steroid-resistant membranous nephropathy: a randomized trial. Kidney Int 59:1484–1490
Cattran DC, Appel GB, Hebert LA, Hunsicker LG, Pohl MA, Hoy WE, Maxwell DR, Kunis CL (1999) A randomized trial of cyclosporine in patients with steroid-resistant focal segmental glomerulosclerosis. North America Nephrotic Syndrome Study Group. Kidney Int 56:2220–2226
Zaffanello M, Brugnara M, Franchini M (2007) Therapy for children with henoch-schonlein purpura nephritis: a systematic review. ScientificWorldJournal 7:20–30
Algoet C, Proesmans W (2003) Renal biopsy 2–9 years after Henoch Schönlein purpura. Pediatr Nephrol 18:471–473
Liu HM, Xu H, Zhou LJ, Zhang J (2007) Efficacy of mycophenolate mofetil and methylprednisolone alternate-day therapy in childhood lupus nephritis. Chinese Journal of Practical Pediatrics 22:137–138
Appel AS, Appel GB (2009) An update on the use of mycophenolate mofetil in lupus nephritis and other primary glomerular diseases. Nat Clin Pract Nephrol 5:132–142
Xu G, Tu W, Jiang D, Xu C (2009) Mycophenolate mofetil treatment for IgA nephropathy: a meta-analysis. Am J Nephrol 29:362–367
Acknowledgement
This work was supported by the State of Health Science Foundation of Hunan province (B2005149), State of Science and Technology Science Foundation of Hunan province (06SK3198), State Administration of Traditional Chinese Medicine of Hunan province (6301) in China. The authors thank all colleagues who took medical records, managed patients, did renal biopsies, and assisted with follow-up.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Li, Z., Duan, C., He, J. et al. Mycophenolate mofetil therapy for children with steroid-resistant nephrotic syndrome. Pediatr Nephrol 25, 883–888 (2010). https://doi.org/10.1007/s00467-009-1375-7
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00467-009-1375-7