Long-term outcome of kidney transplantation in patients with congenital anomalies of the kidney and urinary tract

Pediatr Nephrol. 2019 Nov;34(11):2409-2415. doi: 10.1007/s00467-019-04300-z. Epub 2019 Jul 15.


Background: Congenital anomalies of the kidney and urinary tract (CAKUT) are a leading cause of end-stage kidney failure in the young. However, there is limited information on long-term outcomes after kidney transplantation in this group. We explored the outcomes of kidney transplant in patients with the 3 most common severe forms of CAKUT: posterior urethral valves (PUV), reflux nephropathy and renal hypoplasia/dysplasia.

Methods: Data were extracted from the Australian & New Zealand Dialysis & Transplant Registry on first kidney transplants performed between 1985 and 2015 in recipients with a primary diagnosis of PUV, renal hypoplasia/dysplasia or reflux nephropathy (under the age of 30 years). Using multivariate Cox regression, we compared death-censored graft survival between the three groups.

Results: One hundred twenty-seven patients with PUV, 245 with hypoplasia/dysplasia and 727 with reflux nephropathy were included. A 10-year graft survival in PUV, hypoplasia/dysplasia and reflux nephropathy was 70%, 76% and 70%, respectively and a 20-year graft survival was 30%, 53% and 49%. After adjusting for age at transplant, graft source and HLA matching, there was evidence for poorer graft survival in PUV (HR, 1.65; 95% CI, 1.15 to 2.38).

Conclusions: Graft survival of the first transplant in CAKUT is favourable at 10 years; however, recipients with PUV have increased risk of graft loss beyond a 10-year post-transplant, which may be related to bladder dysfunction.

Keywords: CAKUT; Chronic kidney failure child; Graft survival; Kidney transplantation; Posterior urethral valves; Reflux nephropathy.

MeSH terms

  • Adolescent
  • Adult
  • Australia / epidemiology
  • Child
  • Child, Preschool
  • Female
  • Graft Survival*
  • Humans
  • Kidney Failure, Chronic / etiology
  • Kidney Failure, Chronic / surgery*
  • Kidney Transplantation / adverse effects
  • Kidney Transplantation / statistics & numerical data*
  • Male
  • New Zealand / epidemiology
  • Registries / statistics & numerical data
  • Time Factors
  • Treatment Outcome
  • Urogenital Abnormalities / complications
  • Urogenital Abnormalities / surgery*
  • Vesico-Ureteral Reflux / complications
  • Vesico-Ureteral Reflux / surgery*
  • Young Adult

Supplementary concepts

  • Cakut