End-stage kidney disease due to alport syndrome: Outcomes in 296 consecutive Australia and New Zealand dialysis and transplant registry cases

Andrew Mallett, Wen Tang, Philip A. Clayton, Sarah Stevenson, Stephen P. McDonald, Carmel M. Hawley, Sunil V. Badve, Neil Boudville, Fiona G. Brown, Scott B. Campbell, David W. Johnson

Research output: Contribution to journalArticle

13 Citations (Scopus)

Abstract

Background. Alport syndrome is a rare inheritable renal disease. Clinical outcomes for patients progressing to endstage kidney disease (ESKD) are not well described. Methods. This study aimed to investigate the characteristics and clinical outcomes of patients from Australia and New Zealand commencing renal replacement therapy (RRT) for ESKD due to Alport syndrome between 1965 and 1995 (early cohort) and between 1996 and 2010 (contemporary cohort) compared with propensity score-matched, RRT-treated, non-Alport ESKD controls. Results. A total of 58 422 patients started RRT during this period of which 296 (0.5%) patients had Alport ESKD. In the Conclusion. Alport syndrome patients experienced comparable dialysis and renal transplant outcomes to matched non-Alport ESKD controls in the contemporary cohort due to relatively greater improvements in outcomes for non-Alport ESKD patients over time. Post-transplant anti-GBM disease was rare.

LanguageEnglish
Pages2277-2286
Number of pages10
JournalNephrology Dialysis Transplantation
Volume29
Issue number12
DOIs
Publication statusPublished - 1 Dec 2014
Externally publishedYes

Keywords

  • Alport syndrome
  • Dialysis
  • Hereditary nephritis
  • Outcomes
  • Renal transplantation

ASJC Scopus subject areas

  • Nephrology
  • Transplantation

Cite this

Mallett, Andrew ; Tang, Wen ; Clayton, Philip A. ; Stevenson, Sarah ; McDonald, Stephen P. ; Hawley, Carmel M. ; Badve, Sunil V. ; Boudville, Neil ; Brown, Fiona G. ; Campbell, Scott B. ; Johnson, David W. / End-stage kidney disease due to alport syndrome : Outcomes in 296 consecutive Australia and New Zealand dialysis and transplant registry cases. In: Nephrology Dialysis Transplantation. 2014 ; Vol. 29, No. 12. pp. 2277-2286.
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abstract = "Background. Alport syndrome is a rare inheritable renal disease. Clinical outcomes for patients progressing to endstage kidney disease (ESKD) are not well described. Methods. This study aimed to investigate the characteristics and clinical outcomes of patients from Australia and New Zealand commencing renal replacement therapy (RRT) for ESKD due to Alport syndrome between 1965 and 1995 (early cohort) and between 1996 and 2010 (contemporary cohort) compared with propensity score-matched, RRT-treated, non-Alport ESKD controls. Results. A total of 58 422 patients started RRT during this period of which 296 (0.5{\%}) patients had Alport ESKD. In the Conclusion. Alport syndrome patients experienced comparable dialysis and renal transplant outcomes to matched non-Alport ESKD controls in the contemporary cohort due to relatively greater improvements in outcomes for non-Alport ESKD patients over time. Post-transplant anti-GBM disease was rare.",
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Mallett, A, Tang, W, Clayton, PA, Stevenson, S, McDonald, SP, Hawley, CM, Badve, SV, Boudville, N, Brown, FG, Campbell, SB & Johnson, DW 2014, 'End-stage kidney disease due to alport syndrome: Outcomes in 296 consecutive Australia and New Zealand dialysis and transplant registry cases', Nephrology Dialysis Transplantation, vol. 29, no. 12, pp. 2277-2286. https://doi.org/10.1093/ndt/gfu254

End-stage kidney disease due to alport syndrome : Outcomes in 296 consecutive Australia and New Zealand dialysis and transplant registry cases. / Mallett, Andrew; Tang, Wen; Clayton, Philip A.; Stevenson, Sarah; McDonald, Stephen P.; Hawley, Carmel M.; Badve, Sunil V.; Boudville, Neil; Brown, Fiona G.; Campbell, Scott B.; Johnson, David W.

In: Nephrology Dialysis Transplantation, Vol. 29, No. 12, 01.12.2014, p. 2277-2286.

Research output: Contribution to journalArticle

TY - JOUR

T1 - End-stage kidney disease due to alport syndrome

T2 - Nephrology Dialysis Transplantation

AU - Mallett, Andrew

AU - Tang, Wen

AU - Clayton, Philip A.

AU - Stevenson, Sarah

AU - McDonald, Stephen P.

AU - Hawley, Carmel M.

AU - Badve, Sunil V.

AU - Boudville, Neil

AU - Brown, Fiona G.

AU - Campbell, Scott B.

AU - Johnson, David W.

PY - 2014/12/1

Y1 - 2014/12/1

N2 - Background. Alport syndrome is a rare inheritable renal disease. Clinical outcomes for patients progressing to endstage kidney disease (ESKD) are not well described. Methods. This study aimed to investigate the characteristics and clinical outcomes of patients from Australia and New Zealand commencing renal replacement therapy (RRT) for ESKD due to Alport syndrome between 1965 and 1995 (early cohort) and between 1996 and 2010 (contemporary cohort) compared with propensity score-matched, RRT-treated, non-Alport ESKD controls. Results. A total of 58 422 patients started RRT during this period of which 296 (0.5%) patients had Alport ESKD. In the Conclusion. Alport syndrome patients experienced comparable dialysis and renal transplant outcomes to matched non-Alport ESKD controls in the contemporary cohort due to relatively greater improvements in outcomes for non-Alport ESKD patients over time. Post-transplant anti-GBM disease was rare.

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