Cystinosis in pediatric renal transplant recipients: Case control study from Kuwait
Prasad Nair 1, Mohamed Adel M1, Osama Gheith1, Torki Al-Otaibi1, Tarek Said1, Medhat A Halim1, Mohamed Balha 1, Zakaria Zakaria 1.
1Nephrology, OTC, Kuwait, Kuwait
Introduction and Aim: Cystinosis is an autosomal recessive lipsosomal storage multisystem disease characterized by deficient cystenosin that result in cystine accumulation in the lysosomes. It can lead to end stage kidney disease in most of cases before 20 years age.
We aimed to evaluate the outcome of renal transplantation in pediatric renal transplant with cystinosis.
Methods: Data of renal transplant recipients with Cystinosis (n=15) in Hamed Al-Essa organ transplant center were retrospectively evaluated against matched control cohort (n=128). Demographic data in both groups were compared and post-transplant complications and both graft and patients outcomes were assessed.
Results: Most of cystinosis patients (53.3%) were Kuwaiti males in their second decade of life with their mean age 13.3±3.9 vs. 68% and mean age of 14 ±3.1 years in the control group. The two groups were comparable regarding type of donor, pre transplant comorbidities (p>0.05). The percentage of cystinosis cases with immediate graft function was significantly higher than the control (p=0.024), and this was reflected by relatively lower basal creatinine but did not rank to significance (>0.05), and they received significantly less induction therapy (p=0.002). The two groups were maintained on comparable immunosuppressive regimen and we did not find any significant difference between the two groups regarding post-transplant complications like post-transplant diabetes ,viral infections ,graft function at 1 ,3,5,10 years and the both graft and patient outcomes were comparable (p>0.05).
Conclusion: Under standard immunosuppression therapy with steroid calcinurine inhibitors, mycofenolate mofetil , renal transplant is safe with good long term outcome in patient with cystinosis.
There are no comments yet...