MANAGEMENT OF CHILDHOOD STEROID DEPENDENT NEPHROTIC SYNDROME WITH CYCLOPHOSPHAMIDE – AN EXPERIENCE AT AYUB TEACHING HOSPITAL, ABBOTTABAD
AbstractBackground: This study has been done in children with Steroid dependent nephrotic syndrome (SDNS) to check for the response to cyclophosphamide and relapse on follow up for one year after completion of treatment. Methods: This study was conducted over two years and nine months. Patients were taken as steroid dependent when there were two consecutive relapses occur on steroids tapering or within two weeks of stopping treatment. Children of either sex between ages of 1–14 years, diagnosed case of SDNS were included in this study. Renal biopsy was not done in any patient. After achieving remission with oral steroids, cyclophosphamide was given after calculation of maximum cumulative dose 168 mg/kg for 8 – 12 weeks along with oral steroids. Follow up done every two weeks till completion of treatment for response and adverse effects and thereafter for one year. Results: There were 31 patients, 23 (74.2%) male and 8 (25.8%) females. Age ranged from 1.5 years to 11 years with mean age 5.44±2.39 years. There was full response to cyclophosphamide as none of patient had proteinuria on cyclophosphamide therapy. After completion of cyclophosphamide course, four patients (12.9%) relapsed on follow up while 87.9% remain in complete remission. Only one female patient (3.23%) had adverse effect in form of hair fall and she recovered after completion of treatment. None of patient showed any other adverse effect including haematuria. Conclusion: Cyclophosphamide is an effective therapy in management of childhood SDNS with minimum adverse effects in medium term
Gruppen MP, Bouts AH, Weide MCJ, Merkus MP, Zurowska A, Maternik M, et al. A randomized clinical trial indicates that levamisole increases the time to relapse in children with steroid-sensitive idiopathic nephrotic syndrome. Kidney Int 2018;93:510–18.
Davin JC. The glomerular permeability factors in idiopathic nephrotic syndrome. Pediatr Nephrol 2016;31:207–15.
Karunamoorthy S, Thanigachalam D, Jeyachandran D, Ramanathan S, Natarajan G, Thoppalan B. The safety and efficacy of mycophenolate mofetil in children and adolescents with steroid-dependent nephrotic syndrome: a single-centre study. Clin Kidney J 2020;13(2):179–83.
Park Shin J. Complications of nephrotic syndrome. Korean J Pediatr 2011;54(8):322.
A study of efficacy of levamisole in children with frequently relapsing and steroid-dependent nephrotic syndrome. Indian J Res 2019;8(3):26–7.
Hettiarachchi HNS, Raja M, Karunadasa UI, Thalgahagoda RS. Efficacy and side effects of mycophenolate mofetil therapy in children with steroid dependent nephrotic syndrome in a tertiary paediatric nephrology centre in Sri Lanka. Sri Lanka J Child Health 2018;48(1):53–8.
Vivarelli M, Massella L, Ruggiero B, Emma F. Minimal change disease. Clin J Am Soc Nephrol 2017;12:332–45.
Pravitsitthikul N, Willis N, Hodson E, Craig J. Non-corticosteroid immunosuppressive medications for steroid-sensitive nephrotic syndrome in children. Cochrane Database Syst Rev 2013;(10):CD002290.
Khemani S, Moorani KN. Cyclosporine versus Cyclophosphamide in Childhood Nephrotic Syndrome. J Liaquat Univ Med Health Sci 2016;15(2):57–62.
Wang C, Travers C, McCracken C, Leong T, Gbadegesin R, Quiroga A, et al. Adrenocorticotropic Hormone for Childhood Nephrotic Syndrome. The ATLANTIS Randomized Trial. Clin J Am Soc Nephrol 2018;13:1859–65.
Teeninga N, Kist-van Holthe JE, Nauta J. Extending prednisolone treatment does not reduce relapses in childhood nephrotic syndrome. J Am Soc Nephrol 2012;24:149–59.
Ali EMA, Elhaj NAO, Abdelraheem MB, Ellidir RA, Osman R. Cyclophosphamide versus cyclosporine in children with frequent-relapsing and steroid-dependent nephrotic syndrome in Khartoum State, Sudan. Khartoum Med J 2017;10(3):1402–10.
Abeyagunawardena S, Jayaweera AHHM, Thalgahagoda RS, Karunadasa UI, Abeyagunawardena AS. Intravenous pulsed vs oral cyclophosphamide therapy in steroid dependent nephrotic syndrome. Sri Lanka J Child Health 2017;46(4):317–21.
Bajeer IA, Khatri S, Tresa V, Hashmi S, Mubarak M, Lanewala AA. Histopathological Spectrum and short-term outcome of treatment with cyclophosphamide in relapsing steroid-sensitive nephrotic Syndrome. J Coll Physicians Surg Pak 2018;28(6):436–9.
Moorani KN, Hotchandani HM, Zubair AM, Lohana NC, Veerwani NR. Immunosuppressive therapy in children with primary nephrotic syndrome: single center experience, Karachi, Pakistan. BMC Nephrol 2019;20:239.
Azib S, Macher MA, Kwon T, Dechartres A, Alberti C, Loirat C. Cyclophosphamide in steroid-dependent nephrotic syndrome. Pediatr Nephrol 2011;26:927–32.
Thalgahagoda RS, Jayaweera AH, Karunadasa UI, Abeyagunawardena AS. Therapies for steroid‑sensitive nephrotic syndrome. Asian J Pediatr Nephrol 2018;1:56–61.
Deschênes G, Dossier C, Hogan J. Treating the idiopathic nephrotic syndrome: are steroids the answer? Pediatr Nephrol 2019;34:777–85.
Gajjar R, Miller SD, Meyers KE, Ginsberg JP. Fertility preservation in patients receiving cyclophosphamide therapy for renal disease. Pediatr Nephrol 2015;30:1099–1106.
Tan L, Li S, Yang H, Zou Q, Wan J, Li Q. Efficacy and acceptability of immunosuppressive agents for pediatric frequently-relapsing and steroid-dependent nephrotic syndrome. Medicine 2019;98(22):e15927.
Mehta S, Makkar V, Soha PM, Sethi S, Kaur S. Cyclophosphamide-Induced Melanonychia in a Patient with Steroid Dependent Nephrotic Syndrome: A Rare Presentation. Saudi J Kidney Dis Transpl 2019;30(4):978–81.
Korsgaard T, Andersen RF, Joshi S, Hagstrøm S, Rittig S. Childhood onset steroid-sensitive nephrotic syndrome continues into adulthood. Pediatr Nephrol 2019;34(4):641–8.