en پژوهشي اصیل Original Research <p><strong>Background and Objective</strong><strong>:</strong> Cystinosis is a rare inherited disease that leads to renal failure. Fanconi syndrome is the major renal involvement in cystinosis patients. Renal transplantation is the treatment of choice in cystinosis children with end-stage renal disease (ESRD).&nbsp; The study aimed to assess the outcome of renal transplantation in Iranian children with cystinosis.</p> <p><strong>Methods</strong>: This retrospective study is a follow up of 21 cystinosis children transplanted in Labafinejad Hospital, Tehran, Iran.</p> <p><strong>Results</strong>: Three cystinosis patients involved by primary non-function because of graft vein thrombosis and/or severe acute tubular necrosis. The remaining cystinosis patients had excellent graft survival rate and only one patient lost the graft 3 years post-transplant due to noncompliance. The graft survival rate after excluding the patients with primary non-function was 100%, 94%, 94% and 94% at 1,3,5 and 10 years after transplant. The mean serum creatinine in patients with functioning graft 10 years after transplant was 1.6 mg/dl.</p> <p><strong>Conclusion</strong>: We showed that cystinosis patients had good graft function in long term after transplantation.</p> <p></p> Renal Transplantation, Cystinosis, Failure 10 12 http://ijca.iums.ac.ir/browse.php?a_code=A-10-1-10&slc_lang=en&sid=1 Rozita Hoseini rozitahoseini@yahoo.com 1003194753284600142 1003194753284600142 No Pediatric nephrologist, Pediatric Transplantation Dialysis Research Center, Iran University of Medical Sciences, Tehran, Iran hasanotoukesh@yahoo.com 1003194753284600143 1003194753284600143 Yes Pediatric nephrologist, Pediatric Transplantation Dialysis Research Center, Iran University of Medical Sciences, Tehran, Iran Nasim Mirzaiee 1003194753284600144 1003194753284600144 No Tehran University of Medical Sciences, Tehran, Iran Nahid Rahimzadeh cpidir@gmail.com 1003194753284600145 1003194753284600145 No Pediatric Nephrologist, Pediatric Transplantation Dialysis Research Center, Iran University of Medical Sciences, Tehran, Iran