Evaluation of Children with Steroid-resistant Nephrotic Syndrome Showing Pathologic Findings of Focal Segmental Glomerulosclerosis (FSGS) after Renal Transplantation in Iranian Educational Medical Centers from 1998 to 2018
Journal of Pediatric Nephrology,
Vol. 6 No. 2 (2018),
4 August 2018
,
Page 1-4
https://doi.org/10.22037/jpn.v6i2.21691
Abstract
Introduction: Steroid resistant nephrotic syndrome due to idiopathic focal segmental glomerulosclerosis (FSGS) or genetic mutations is one of the most common causes of end-stage renal disease (ESRD) that leads to renal transplantation. The relapse of the disease in the transplanted kidney, despite proper therapeutic management pre and post transplantation, may result in graft loss. Lack of accurate data about the status of transplanted patients due to FSGS encouraged us to obtain data from all pediatric nephrologists in Iran to achieve better pre and post transplantation therapeutic management.
Material and Methods: The personal data of the pediatric nephrologist as well as the data of surgical and medical management prior to transplantation, relapse in the allograft kidney, and the therapeutic response rate after relapse were collected via a questionnaire.
Results: Of 82 cases of FSGS from 1998 to 2018, 23 had relapse, mostly within 1 year after transplantation. When relapse occurred, nearly all centers used plasmapheresis and rituximab and some used angiotensin converting enzyme inhibitor (ACEI) or angiotensin receptor blocker (ARB) in addition to immunosuppressive medications and methyl prednisolone pulse therapy. Genetic studies were done in only two centers and there was no difference in immunosuppressive medications between these two centers and the idiopathic group. Pre-transplant plasmapheresis and rituximab were administered in four centers, while two centers used IVIG and one center only used plasmapheresis. Delayed graft function (DGF) was negative in 7 and positive in 9 centers. In most centers, immunosuppressive therapy consisted of a corticosteroid, mycophenolate mofetil, and tacrolimus. Relapse and recovery rates varied from less than 10% to more than 50% in all centers. Seven centers had no response to any medication. The lowest relapse rates were seen in two centers that had deceased donors and used rituximab and plasmapheresis prior to transplantation.
Conclusion: It can be concluded that with regard to the possibility of relapse after transplantation and variable therapeutic management modalities before and after transplantation, it is reasonable that genetic analysis of mutations, identification of idiopathic and high-risk cases, and use of appropriate therapeutic protocols should be considered to decrease the relapse rate.
Keywords: FSGS; Renal transplantation; Nephrotic syndrome; Child.
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References
Sharma M, Sharma R, Ellen T,et al. The focal segmental sclerosis permeability factor: biochemical characteristics and biological effects. Exp Biol 2004 Jan; 229(1):8598.
Gbadegesin R, Lavin P, Foreman J, Winn. Pathogenesis and therapy for FSGS: an update. Pediatr Nephrol 2011; 10011015.
Bose B, Cattran D for the Toronto Glomerulonephritis Registery. Glomerular Disease: FSGS, Clin J Am Soc Nephrol 2014; 9(3):62632.
Fogo AB. Causes and pathogenesis of FSGS. Nat Rev Nephrol 2015;11(2): 7687.
Gyung Kang H, Ha S, Cheong H. Recurrence and treatment after renal transplantation in children with FSGS. Biomed Res int 2016; 2016:6832971.
Ponticelli C. Recurrence of focal segmental glomerular sclerosis(FSGS) after renal transplantation. Nephrology Dialysis. Transplantation, 2010; 25: 2531.
Gohh RY, Shaffi SK. Longterm. results of early plasmaphresis to prevent recurrence of FSGS in high risk renal transplant recipients. Takakura M, Shimizu M, Mizuta M, et al. Successful treatment of Rituximab and SteroidResistant Nephrotic syndrome with Leukocytopenia. J Clin Apher 2018 Jan 16, doi: 10.1002, Jca.21613.
Francis A, Trnka P, McTaggart SJ. Longterm outcome of kidney transplantation in recipients with FSGS. Clinical J am Soc Nephrol 2016;7:20412046.
Baum MA, Stablein DM, Panzario VM,et al. Loss of living donor allograft survival advantage in children with FSGS. Kidney int. 2001;59(1)328333.
Baum MA. Outcomes after renal transplantation for FSGS in children. Pediatric transplantation 2004;8.
Sharbaf F, Martin Bitzan, Szymanski KM,et al. Native nephrectomy prior to pediatric kidney transplantation: biological and clinical aspect. Pediatr Nephrol 2012;27(7): 11791188.
Cravedi P, Koppand J.B, Remuzzi G. Recent progress in the pathophysiology and treatment of FSGS recurrence. American Journal of Transplantation 2013; 13: 266274.
Jordan SC, Ashley V, Pharm D, et al. Desensitization therapy with (IVIG) application in solid organ transplantation. American clinical and climatologial association 2006; 117: 199211.
Trachtman R, Sran SS, Trachtman H. Recurrent FSGS after kidney transplantation. Pediatr Nephrol 2015; 30: 17931802.
Bose B, Cattran D for the Toronto Glomerulonephritis Registery. Glomerular Disease: FSGS, Clin J Am Soc Nephrol 2014; 9(3):62632.
Sreepada TK Rao, chief editor: Vecihi Batuman .Focal segmental glomerulosclerosis treatment and management. 2017.
Tran M. H., Chanc. Pasch W., Carpenter P., et al. Treatment of focal segmental glomerulosclerosis recurrence in renal allograft: a report of two cases. Nephrology Dialysis March 2016.
Savin VJ, Sharma M, Zhou J, et al. Multiple targets for novel therapy of FSGS associated with circulating permeability factor. Biomed Res Int. 2017;2017:6232616.
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