renal transplantation outcome in children with cystinosis

background and objective: 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).  the study aimed to assess the outcome of renal transplantation in iranian children with cystinosis. methods: this retrospective study is a follow up of 21 cystinosis children transplanted in labafinejad hospital, tehran, iran. results: 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. conclusion: we showed that cystinosis patients had good graft function in long term after transplantation.