Mycophenolate Mofetil Therapy for Children with Steroid-resistant Nephrotic Syndrome

Overview

Journal Pediatr Nephrol

Specialties Nephrology
Pediatrics

Date 2009 Dec 3

PMID 19953275

Citations 17

Authors

Zhihui Li

Cuirong Duan

Jinhua He

Tianhui Wu

Mai Xun

Yi Zhang

Yan Yin

Affiliations

Soon will be listed here.

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.

Citing Articles

Combination of rituximab and low-dose glucocorticoids for idiopathic refractory nephrotic syndrome with MCD/FSGS: a single-center prospective cohort study.

Yimamuyushan A, Li Y, Jiao W, Yu J, Li J, Shi Y Ren Fail. 2024; 46(2):2428330.

PMID: 39544106 PMC: 11571727. DOI: 10.1080/0886022X.2024.2428330.

Clinical Efficacy of Persian Medicine Diet Combined with Western Medicine-Based Diet on Proteinuria in Pediatric Nephrotic Syndrome: A Randomized Controlled Clinical Trial.

Rahmani A, Naseri M, Mohkam M, Motaharifard M, Bakhtiary M, Shakeri N Evid Based Complement Alternat Med. 2022; 2022:2279209.

PMID: 35656469 PMC: 9155912. DOI: 10.1155/2022/2279209.

Current understandings in treating children with steroid-resistant nephrotic syndrome.

Lee J, Kronbichler A, Shin J, Oh J Pediatr Nephrol. 2020; 36(4):747-761.

PMID: 32086590 PMC: 7910243. DOI: 10.1007/s00467-020-04476-9.

Treatment of steroid-resistant nephrotic syndrome in the genomic era.

Bensimhon A, Williams A, Gbadegesin R Pediatr Nephrol. 2018; 34(11):2279-2293.

PMID: 30280213 PMC: 6445770. DOI: 10.1007/s00467-018-4093-1.

Exploring the Clinical and Genetic Spectrum of Steroid Resistant Nephrotic Syndrome: The PodoNet Registry.

Trautmann A, Lipska-Zietkiewicz B, Schaefer F Front Pediatr. 2018; 6:200.

PMID: 30065916 PMC: 6057105. DOI: 10.3389/fped.2018.00200.

References

Ejaz I, Khan H, Javaid B, Rasool G, Bhatti M . Histopathological diagnosis and outcome of paediatric nephrotic syndrome. J Coll Physicians Surg Pak. 2004; 14(4):229-33. DOI: 04.2004/JCPSP.229233. View

Appel A, Appel G . An update on the use of mycophenolate mofetil in lupus nephritis and other primary glomerular diseases. Nat Clin Pract Nephrol. 2009; 5(3):132-42. DOI: 10.1038/ncpneph1036. View

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(9):1265-8. DOI: 10.1007/s00467-005-1957-y. View

Afzal K, Bagga A, Menon S, Hari P, Jordan S . Treatment with mycophenolate mofetil and prednisolone for steroid-dependent nephrotic syndrome. Pediatr Nephrol. 2007; 22(12):2059-65. DOI: 10.1007/s00467-007-0617-9. View

Barletta G, Smoyer W, Bunchman T, Flynn J, Kershaw D . Use of mycophenolate mofetil in steroid-dependent and -resistant nephrotic syndrome. Pediatr Nephrol. 2003; 18(8):833-7. DOI: 10.1007/s00467-003-1175-4. View

Cattran D, Appel G, Hebert L, Hunsicker L, Pohl M, Hoy W . A randomized trial of cyclosporine in patients with steroid-resistant focal segmental glomerulosclerosis. North America Nephrotic Syndrome Study Group. Kidney Int. 1999; 56(6):2220-6. DOI: 10.1046/j.1523-1755.1999.00778.x. View

Chandra M, Susin M, Abitbol C . Remission of relapsing childhood nephrotic syndrome with mycophenolate mofetil. Pediatr Nephrol. 2001; 14(3):224-6. DOI: 10.1007/s004670050047. View

Ulinski T, Dubourg L, Said M, Parchoux B, Ranchin B, Cochat P . Switch from cyclosporine A to mycophenolate mofetil in nephrotic children. Pediatr Nephrol. 2005; 20(4):482-5. DOI: 10.1007/s00467-004-1778-4. View

Moudgil A, Bagga A, Jordan S . Mycophenolate mofetil therapy in frequently relapsing steroid-dependent and steroid-resistant nephrotic syndrome of childhood: current status and future directions. Pediatr Nephrol. 2005; 20(10):1376-81. DOI: 10.1007/s00467-005-1964-z. View

10.

Okada M, Sugimoto K, Yagi K, Yanagida H, Tabata N, Takemura T . Mycophenolate mofetil therapy for children with intractable nephrotic syndrome. Pediatr Int. 2007; 49(6):933-7. DOI: 10.1111/j.1442-200X.2007.02487.x. View

11.

. The primary nephrotic syndrome in children. Identification of patients with minimal change nephrotic syndrome from initial response to prednisone. A report of the International Study of Kidney Disease in Children. J Pediatr. 1981; 98(4):561-4. DOI: 10.1016/s0022-3476(81)80760-3. View

12.

Montane B, Abitbol C, Chandar J, Strauss J, Zilleruelo G . Novel therapy of focal glomerulosclerosis with mycophenolate and angiotensin blockade. Pediatr Nephrol. 2003; 18(8):772-7. DOI: 10.1007/s00467-003-1174-5. View

13.

Sorof J, Hawkins E, Brewer E, Boydstun I, Kale A, Powell D . Age and ethnicity affect the risk and outcome of focal segmental glomerulosclerosis. Pediatr Nephrol. 1999; 12(9):764-8. DOI: 10.1007/s004670050542. View

14.

Gellermann J, Querfeld U . Frequently relapsing nephrotic syndrome: treatment with mycophenolate mofetil. Pediatr Nephrol. 2004; 19(1):101-4. DOI: 10.1007/s00467-003-1300-4. View

15.

Goonasekera C, Koziell A, Hulton S, Dillon M . Vincristine and focal segmental sclerosis: do we need a multicentre trial?. Pediatr Nephrol. 1998; 12(4):284-9. DOI: 10.1007/s004670050454. View

16.

Algoet C, Proesmans W . Renal biopsy 2-9 years after Henoch Schönlein purpura. Pediatr Nephrol. 2003; 18(5):471-3. DOI: 10.1007/s00467-003-1132-2. View

17.

Griswold W, Tune B, Reznik V, Vazquez M, Prime D, Brock P . Treatment of childhood prednisone-resistant nephrotic syndrome and focal segmental glomerulosclerosis with intravenous methylprednisolone and oral alkylating agents. Nephron. 1987; 46(1):73-7. DOI: 10.1159/000184308. View

18.

Gregory M, Smoyer W, Sedman A, Kershaw D, Valentini R, Johnson K . Long-term cyclosporine therapy for pediatric nephrotic syndrome: a clinical and histologic analysis. J Am Soc Nephrol. 1996; 7(4):543-9. DOI: 10.1681/ASN.V74543. View

19.

Bagga A, Hari P, Moudgil A, Jordan S . Mycophenolate mofetil and prednisolone therapy in children with steroid-dependent nephrotic syndrome. Am J Kidney Dis. 2003; 42(6):1114-20. DOI: 10.1053/j.ajkd.2003.08.011. View

20.

Pena A, Bravo J, Melgosa M, Fernandez C, Meseguer C, Espinosa L . Steroid-resistant nephrotic syndrome: long-term evolution after sequential therapy. Pediatr Nephrol. 2007; 22(11):1875-80. DOI: 10.1007/s00467-007-0567-2. View