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Outcomes of Kidney Transplantation in Children with Nephronophthisis: An Analysis of the North American Pediatric Renal Trials and Collaborative Studies (NAPRTCS) Registry


Journal Article

Hamiwka, L.A.; Midgley, J.P.; Wade, A.W.; Martz, K.; Grisaru, S.

Pediatr Transplant




Adolescent; Child; Chlorides; Chromium Compounds; Denmark; Female; Genes; Graft Rejection; Graft Survival; Kidney Diseases; kidney transplantation; Male; Pediatric transplantation; Preschool; Recessive; Registries; Thrombosis; Treatment Outcome

NPHP is an autosomal recessive chronic tubulointerstitial nephropathy that progresses to ESRD. In the 2006 NAPRTCS report, NPHP was the primary diagnosis in 2.8% of all renal transplant patients. At our pediatric center, that covers a population in which the NPHP1 gene is prevalent, 24% of transplant recipients had a primary diagnosis of NPHP. Since no previous literature reports have documented kidney transplant outcomes in patients with NPHP, a review of the 2006 NAPRTCS database was performed. The results of this review illustrate that patients with NPHP as their underlying kidney disease have a significantly better overall graft survival when compared with all other patients registered in the NAPRTCS database. Sub-analysis demonstrated that this benefit is statistically significant only for LD kidney transplant recipients. CrCl was better in NPHP at all time points from transplant up to five-yr follow-up. Moreover, in NPHP LD transplant recipients the decline of CrCl over five yr was slower compared with non-NPHP LD transplant recipients. Rates of thrombosis, acute, and chronic rejection as well as causes of graft failure were similar in NPHP patients and all other patients. This review demonstrates that NPHP transplant recipients have excellent outcomes that are shown to be better compared with the general pediatric transplant population.

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