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Published on: October 11, 2014
Renal Transplant Outcomes in Pediatric Cystinosis Patients: A Comparative Analysis with Non-Cystinosis Controls
Osama Gheith1, Ayman Nagib, Mohamed Adel
1From Nephrology Department, Hamed Al-Essa Organ Transplant Center, Ibn Sina Hospital, Sabah Area, Kuwait and Department of Dialysis and Transplantation, The Urology and Nephrology Center, Mansoura University, Egypt.
Objectives:
Cystinosis is a rare autosomal recessive lysosomal storage disorder causing progressive chronic kidney disease. Limited data exist on renal transplant outcomes in transplant recipients with cystinosis. In this study, we compared clinical outcomes of pediatric renal transplant recipients with cystinosis versus those without cystinosis.
Materials And Methods:
We conducted a retrospective comparative analysis of 143 pediatric renal transplant recipients (23 with cystinosis, 120 without cystinosis {control group } ) followed at a Kuwait kidney transplant center. Primary outcomes included graft function, patient survival, and posttransplant complications. Secondary outcomes assessed anthropometric parameters and serum creatinine trajectories.
Results:
Transplant recipients with cystinosis versus without cystinosis had significantly lower height at last follow -up (1.34 ± 0.18 vs 1.52 ± 0.15 cm; P = .001 )and better baseline renal function (creatinine level of 84.5 ± 36.4 vs 105.0 ± 89.0 μmol /L; P = .04 ). Graft survival at final follow -up was comparable between groups (78.3 % in cystinosis group vs 69.2 % in control group; P = .39 ). New -onset diabetes after transplant was not observed in the cystinosis group versus shown in 9.2 % of patients in the control group (P = .17 ). Viral complications, including BK viremia, BK nephropathy, and cytomegalovirus viremia, showed no significant difference between groups. Patient survival was excellent in both groups (100 % vs 98.3 % in the cystinosis vs control group; P = .55 ).
Conclusions:
Pediatric renal transplant recipients with cystinosis demonstrated comparable graft survival and favorable posttransplant outcomes versus recipients without cystinosis, with potential advantages, including lower incidence of new -onset diabetes after transplant and better early renal function. These findings support renal transplant as a viable treatment option for pediatric patients with cystinosis and end -stage renal disease.
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