Pediatric Nephrology

, Volume 34, Issue 11, pp 2409–2415 | Cite as

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

  • Ashlene M. McKay
  • Siah Kim
  • Sean E. KennedyEmail author
Original Article
Part of the following topical collections:
  1. What’s New in Renal Transplantation



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.


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.


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).


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.


CAKUT Kidney transplantation Posterior urethral valves Reflux nephropathy Graft survival Chronic kidney failure child 



The data reported here have been supplied by the Australia and New Zealand Dialysis and Transplant Registry (ANZDATA). The interpretation and reporting of these data are the responsibility of the Editors and in no way should be seen as an official policy or interpretation of the Australia and New Zealand Dialysis and Transplant Registry.

Authors’ contributions

S.E.K. and S.K. were responsible for study conception and design. A.M. and S.K. were responsible for data analysis and all authors were responsible for interpretation of the data. A.M. drafted the manuscript and all authors were responsible for revising the manuscript. All authors approved the final version of the manuscript.

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

Informed consent

This was a registry study of deidentified data from consenting patients and guardians, collected for the purpose of such analyses. The registry is conducted in accordance with the Australian Commonwealth Privacy Act and associated state legislation governing health data collection.


  1. 1.
    Orr NI, McDonald SP, McTaggart S, Henning P, Craig JC (2009) Frequency, etiology and treatment of childhood end-stage kidney disease in Australia and New Zealand. Pediatr Nephrol 24:1719–1726CrossRefGoogle Scholar
  2. 2.
    Harambat J, van Stralen KJ, Kim JJ, Tizard EJ (2012) Epidemiology of chronic kidney disease in children. Pediatr Nephrol 27:363–373CrossRefGoogle Scholar
  3. 3.
    Wuhl E, van Stralen KJ, Verrina E, Bjerre A, Wanner C, Heaf JG, Zurriaga O, Hoitsma A, Niaudet P, Palsson R, Ravani P, Jager KJ, Schaefer F (2013) Timing and outcome of renal replacement therapy in patients with congenital malformations of the kidney and urinary tract. Clin J Am Soc Nephrol 8:67–74CrossRefGoogle Scholar
  4. 4.
    Kamal MM, El-Hefnawy AS, Soliman S, Shokeir AA, Ghoneim MA (2011) Impact of posterior urethral valves on pediatric renal transplantation: a single-center comparative study of 297 cases. Pediatr Transplant 15:482–487CrossRefGoogle Scholar
  5. 5.
    Otukesh H, Basiri A, Simfroosh N, Hoseini R, Fereshtehnejad SM, Chalian M (2008) Kidney transplantation in children with posterior urethral valves. Pediatr Transplant 12:516–519CrossRefGoogle Scholar
  6. 6.
    Mendizabal S, Zamora I, Serrano A, Sanahuja MJ, Roman E, Dominguez C, Ortega P, Garcia Ibarra F (2006) Renal transplantation in children with posterior urethral valves. Pediatr Nephrol 21:566–571CrossRefGoogle Scholar
  7. 7.
    Connolly JA, Miller B, Bretan PN (1995) Renal transplantation in patients with posterior urethral valves: favorable long-term outcome. J Urol 154:1153–1155CrossRefGoogle Scholar
  8. 8.
    Ross JH, Kay R, Novick AC, Hayes JM, Hodge EE, Streem SB (1994) Long-term results of renal transplantation into the valve bladder. J Urol 151:1500–1504CrossRefGoogle Scholar
  9. 9.
    Salomon L, Fontaine E, Gagnadoux MF, Broyer M, Beurton D (1997) Posterior urethral valves: long-term renal function consequences after transplantation. J Urol 157:992–995CrossRefGoogle Scholar
  10. 10.
    Mochon M, Kaiser BA, Dunn S, Palmer J, Polinsky MS, Schulman SL, Flynn JT, Baluarte HJ (1992) Urinary tract infections in children with posterior urethral valves after kidney transplantation. J Urol 148:1874–1876CrossRefGoogle Scholar
  11. 11.
    Indudhara R, Joseph DB, Perez LM, Diethelm AG (1998) Renal transplantation in children with posterior urethral valves revisited: a 10-year followup. J Urol 160:1201–1203 discussion 1216CrossRefGoogle Scholar
  12. 12.
    Kennedy SE, Mackie FE, Rosenberg AR, McDonald SP (2006) Waiting time and outcome of kidney transplantation in adolescents. Transplantation 82:1046–1050CrossRefGoogle Scholar
  13. 13.
    Van Arendonk KJ, Boyarsky BJ, Orandi BJ, James NT, Smith JM, Colombani PM, Segev DL (2014) National trends over 25 years in pediatric kidney transplant outcomes. Pediatrics 133:594–601CrossRefGoogle Scholar
  14. 14.
    Rees L (2009) Long-term outcome after renal transplantation in childhood. Pediatr Nephrol 24:475–484CrossRefGoogle Scholar
  15. 15.
    Bartsch L, Sarwal M, Orlandi P, Yorgin PD, Salvatierra O Jr (2002) Limited surgical interventions in children with posterior urethral valves can lead to better outcomes following renal transplantation. Pediatr Transplant 6:400–405CrossRefGoogle Scholar
  16. 16.
    ANZDATA Registry (2017) 39th report, chapter 11 Paediatrics: Australia and New Zealand Dialysis and Transplant Registry. Adelaide, AustraliaGoogle Scholar
  17. 17.
    Fine MS, Smith KM, Shrivastava D, Cook ME, Shukla AR (2011) Posterior urethral valve treatments and outcomes in children receiving kidney transplants. J Urol 185:2507–2511CrossRefGoogle Scholar
  18. 18.
    ANZDATA Registry (2018) 41st report, chapter 10: End stage kidney disease in Indigenous peoples of Australia and Aotearoa/New Zealand. Australia and New Zealand Dialysis and Transplant Registry, Adelaide, AustraliaGoogle Scholar
  19. 19.
    Craig JC, Irwig LM, Knight JF, Roy LP (2000) Does treatment of vesicoureteric reflux in childhood prevent end-stage renal disease attributable to reflux nephropathy? Pediatrics 105:1236–1241CrossRefGoogle Scholar
  20. 20.
    Adams J, Mehls O, Wiesel M (2004) Pediatric renal transplantation and the dysfunctional bladder. Transpl Int 17:596–602CrossRefGoogle Scholar
  21. 21.
    Kara E, Sakaci T, Ahbap E, Sahutoglu T, Koc Y, Basturk T, Sevinc M, Akgol C, Kayalar AO, Ucar ZA, Unsal A, Seyahi N (2016) Posttransplant urinary tract infection rates and graft outcome in kidney transplantation for end-stage renal disease due to reflux nephropathy versus chronic glomerulonephritis. Transplant Proc 48:2065–2071CrossRefGoogle Scholar
  22. 22.
    Yazici H, Caliskan Y, Ozturk S, Ozkan O, Turkmen A, Sever MS (2011) Outcome of kidney transplantation following end-stage renal disease due to reflux nephropathy. Transplant Proc 43:1566–1569CrossRefGoogle Scholar
  23. 23.
    Neild GH, Dakmish A, Wood S, Nauth-Misir R, Woodhouse CR (2004) Renal transplantation in adults with abnormal bladders. Transplantation 77:1123–1127CrossRefGoogle Scholar
  24. 24.
    van Stralen KJ, Borzych-Duzalka D, Hataya H, Kennedy SE, Jager KJ, Verrina E, Inward C, Ronnholm K, Vondrak K, Warady BA, Zurowska AM, Schaefer F, Cochat P, registry EE-E, registry I, registry A, Japanese RRTr (2014) Survival and clinical outcomes of children starting renal replacement therapy in the neonatal period. Kidney Int 86:168–174CrossRefGoogle Scholar
  25. 25.
    Dharnidharka VR, Lamb KE, Zheng J, Schechtman KB, Meier-Kriesche HU (2015) Across all solid organs, adolescent age recipients have worse transplant organ survival than younger age children: a US national registry analysis. Pediatr Transplant 19:471–476CrossRefGoogle Scholar
  26. 26.
    Ritchie AG, Clayton PA, McDonald SP, Kennedy SE (2018) Age-specific risk of renal graft loss from late acute rejection or non-compliance in the adolescent and young adult period. Nephrology (Carlton, Vic) 23:585–591CrossRefGoogle Scholar
  27. 27.
    Kabore R, Couchoud C, Macher MA, Salomon R, Ranchin B, Lahoche A, Roussey-Kesler G, Garaix F, Decramer S, Pietrement C, Lassalle M, Baudouin V, Cochat P, Niaudet P, Joly P, Leffondre K, Harambat J (2016) Age dependent risk of graft failure in young kidney transplant recipients. TransplantationGoogle Scholar
  28. 28.
    Van Arendonk KJ, James NT, Boyarsky BJ, Garonzik-Wang JM, Orandi BJ, Magee JC, Smith JM, Colombani PM, Segev DL (2013) Age at graft loss after pediatric kidney transplantation: exploring the high-risk age window. Clin J Am Soc Nephrol 8:1019–1026CrossRefGoogle Scholar

Copyright information

© IPNA 2019

Authors and Affiliations

  1. 1.Department of NephrologySydney Children’s HospitalRandwickAustralia
  2. 2.School of Women’s and Children’s Health, Faculty of MedicineUniversity of New South WalesSydneyAustralia
  3. 3.Australia and New Zealand Dialysis and Transplant (ANZDATA) RegistryRoyal Adelaide HospitalAdelaideAustralia

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