title: Follow-up of patients with juvenile nephronophthisis after renal transplantation: a single center experience.
abstract: BACKGROUND
  Nephronophthisis (NPHP) is the most common genetic cause of end-stage renal disease (ESRD) in the first 3 decades of life. Treatment of patients with NPHP is symptomatic; kidney transplantation is the treatment of choice when ESRD is established. We report herein our center's experience with kidney transplantation for children with juvenile NPHP.
  
  
  METHODS
  We retrospectively analyzed medical records of 9 renal transplant recipients with a primary diagnosis of juvenile NPHP confirmed by genetic analysis and/or renal biopsy findings in a single center from 1996 to 2010.
  
  
  RESULTS
  Of the 9 patients, 6 received a living related and 3 a cadaveric donor transplantation. Preemptive renal transplantation was performed in 7 patients. The median age of the patients was 13.38 ± 4.6 years; the median follow-up period was 17 months. Posttransplantation immusuppression comprised corticosteroids, a calcineurin inhibitor, and mycophenolate mofetil or azathioprine. One patient lost his renal graft owing to renal graft thrombosis, and grade II chronic allograft nephropathy was diagnosed by renal biopsy on the 62th day after renal transplantation in another patient. The median glomerular filtration rates after transplantation at 1, 3, and 5 years were 85, 75.2, and 83.2 mL/min/1.73 m(2), respectively.
  
  
  CONCLUSIONS
  We observed preserved graft functions for long periods among renal transplant recipients with juvenile NPHP. Chronic allograft nephropathy developed rarely on long follow-up.
authors.affiliation: Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey.
authors.forename: A C
authors.initials: AC
authors.lastname: Tayfur
authors.forename: N
authors.initials: N
authors.lastname: Besbas
authors.forename: Y
authors.initials: Y
authors.lastname: Bilginer
authors.forename: F
authors.initials: F
authors.lastname: Ozaltin
authors.forename: A
authors.initials: A
authors.lastname: Duzova
authors.forename: M
authors.initials: M
authors.lastname: Bakkaloglu
authors.forename: F T
authors.initials: FT
authors.lastname: Aki
authors.forename: S
authors.initials: S
authors.lastname: Ozen
authors.forename: R
authors.initials: R
authors.lastname: Topaloglu
authors.forename: A
authors.initials: A
authors.lastname: Bakkaloglu
mesh_terms: D000293:Adolescent; D002648:Child; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D052177:Kidney Diseases, Cystic / Q000151:congenital / Q000601:surgery; D016030:Kidney Transplantation; D008297:Male