Long-Term Outcomes of Kidney Transplant Recipients With Juvenile Nephronophthisis

Objectives: Nephronophthisis is the most common genetic cause of kidney failure in childhood. Treatment for nephronophthisis is symptomatic, and kidney transplant is a good treatment option when kidney failure has developed. We reported the outcomes of kidney transplant recipients with primary diagnosis of juvenile nephronophthisis who were followed-up in our center. Materials and Methods: We retrospectively examined medical records of 17 kidney transplant patients with a primary diagnosis of juvenile nephronophthisis. We compared this group of 17 patients with kidney transplant recipients who had other etiologies of kidney failure in terms of transplant age, donor type, immunosuppressive treatment, acute rejection, graft loss rates, and glomerular filtration rates at 1 and 5 years posttransplant (N = 180 total analyzed). Results: Among 180 kidney transplant recipients, the 17 patients (9.4%) with nephronophthisis had a mean age of 12.6 +/- 4.3 years and mean follow-up time posttransplant of 79.5 +/- 41.9 months. Five of 17 patients received a kidney transplant from a deceased donor (29.4%), and the remaining 12 patients (70.6%) received transplants from living related donors. Preemptive kidney transplant was performed in 4 patients (23.5%). There was a statistically significant difference (P < .05) in terms of acute rejection between patients with nephronophthisis (17.6%) versus patients with other primary diagnoses (34%). However, the patients with nephronophthisis versus those with other primary diagnoses were similar (P > .05) in terms of transplant age (12.6 +/- 4.3 vs 13.8 +/- 6.7 years, respectively) and follow-up time (79.5 +/- 41.9 vs 59.1 +/- 38.8 months, respectively). Donor type, immunosuppressive treatment, and 1-year (96.7 +/- 23.2 vs 97.6 +/- 28.4 mL/min/1.73 m(2)) and 5-year (84.7 +/- 31.1 vs 86.7 +/- 21.7 mL/min/1.73 m(2)) glomerular filtration rates were also similar (P > .05) between groups. Conclusions: Posttransplant prognosis was good among kidney transplant recipients with juvenile nephronophthisis.