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Pediatr Nephrol. 2019 Jul 15. doi: 10.1007/s00467-019-04300-z. [Epub ahead of print]

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

McKay AM1,2, Kim S1,2,3, Kennedy SE4,5,6.

Author information

1
Department of Nephrology, Sydney Children's Hospital, Randwick, NSW, 2031, Australia.
2
School of Women's and Children's Health, Faculty of Medicine, University of New South Wales, Sydney, NSW, 2000, Australia.
3
Australia and New Zealand Dialysis and Transplant (ANZDATA) Registry, Royal Adelaide Hospital, Adelaide, SA, Australia.
4
Department of Nephrology, Sydney Children's Hospital, Randwick, NSW, 2031, Australia. Sean.Kennedy@unsw.edu.au.
5
School of Women's and Children's Health, Faculty of Medicine, University of New South Wales, Sydney, NSW, 2000, Australia. Sean.Kennedy@unsw.edu.au.
6
Australia and New Zealand Dialysis and Transplant (ANZDATA) Registry, Royal Adelaide Hospital, Adelaide, SA, Australia. Sean.Kennedy@unsw.edu.au.

Abstract

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

PMID:
31309282
DOI:
10.1007/s00467-019-04300-z

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