Ashlene M McKay1,2, Siah Kim1,2,3, Sean E Kennedy4,5,6. 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.
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.
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