title: End-stage kidney disease due to Alport syndrome: outcomes in 296 consecutive Australia and New Zealand Dialysis and Transplant Registry cases.
abstract: BACKGROUND
  Alport syndrome is a rare inheritable renal disease. Clinical outcomes for patients progressing to end-stage 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 early cohort, Alport ESKD was associated with superior dialysis patient survival [adjusted hazard ratio (HR): 0.41, 95% confidence interval (CI): 0.20-0.83, P = 0.01], renal allograft survival (HR: 0.74, 95% CI: 0.54-1.01, P = 0.05) and renal transplant patient survival (HR: 0.43, 95% CI: 0.28-0.66, P < 0.001) compared with controls. In the contemporary cohort, no differences were observed between the two groups for dialysis patient survival (HR: 1.42, 95% CI: 0.65-3.11, P = 0.38), renal allograft survival (HR: 1.01, 95% CI: 0.57-1.79, P = 0.98) or renal transplant patient survival (HR: 0.67, 95% CI: 0.26-1.73, P = 0.41). One Alport patient (0.4%) had post-transplant anti-glomerular basement membrane (anti-GBM) disease. Four female and 41 male Alport patients became parents on RRT with generally good neonatal outcomes.
  
  
  CONCLUSIONS
  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.
authors: ANZDATA Registry, Adelaide, South Australia, Australia Department of Nephrology, Princess Alexandra Hospital, Brisbane, Queensland, Australia Department of Renal Medicine, Royal Brisbane and Women's Hospital, Brisbane, Queensland, Australia.
authors: Andrew
authors: A
authors: Mallett
authors: Division of Nephrology, Peking University Third Hospital, Beijing, China.
authors: Wen
authors: W
authors: Tang
authors: ANZDATA Registry, Adelaide, South Australia, Australia Department of Renal Medicine, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia School of Public Health, University of Sydney, Sydney, New South Wales, Australia.
authors: Philip A
authors: PA
authors: Clayton
authors: ANZDATA Registry, Adelaide, South Australia, Australia Department of Nephrology, Princess Alexandra Hospital, Brisbane, Queensland, Australia.
authors: Sarah
authors: S
authors: Stevenson
authors: ANZDATA Registry, Adelaide, South Australia, Australia Department of Nephrology and Transplantation Services, University of Adelaide at Central Northern Adelaide Renal and Transplantation Services, Adelaide, South Australia, Australia.
authors: Stephen P
authors: SP
authors: McDonald
authors: ANZDATA Registry, Adelaide, South Australia, Australia Department of Nephrology, Princess Alexandra Hospital, Brisbane, Queensland, Australia.
authors: Carmel M
authors: CM
authors: Hawley
authors: ANZDATA Registry, Adelaide, South Australia, Australia Department of Nephrology, Princess Alexandra Hospital, Brisbane, Queensland, Australia.
authors: Sunil V
authors: SV
authors: Badve
authors: ANZDATA Registry, Adelaide, South Australia, Australia School of Medicine and Pharmacology, Sir Charles Gairdner Hospital Unit, The University of Western Australia, Perth, Western Australia, Australia.
authors: Neil
authors: N
authors: Boudville
authors: ANZDATA Registry, Adelaide, South Australia, Australia Department of Nephrology, Monash Medical Centre, Melbourne, Victoria, Australia.
authors: Fiona G
authors: FG
authors: Brown
authors: ANZDATA Registry, Adelaide, South Australia, Australia Department of Nephrology, Princess Alexandra Hospital, Brisbane, Queensland, Australia.
authors: Scott B
authors: SB
authors: Campbell
authors: ANZDATA Registry, Adelaide, South Australia, Australia Department of Nephrology, Princess Alexandra Hospital, Brisbane, Queensland, Australia.
authors: David W
authors: DW
authors: Johnson
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001315:Australia / Q000453:epidemiology; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006085:Graft Survival; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D007676:Kidney Failure, Chronic / Q000453:epidemiology / Q000209:etiology / Q000601:surgery*; D016030:Kidney Transplantation; D008297:Male; D008875:Middle Aged; D009394:Nephritis, Hereditary / Q000150:complications* / Q000453:epidemiology; D009520:New Zealand / Q000453:epidemiology; D011379:Prognosis; D012042:Registries; D012189:Retrospective Studies; D015996:Survival Rate / Q000639:trends; D055815:Young Adult
keywords: Alport syndrome; dialysis; hereditary nephritis; outcomes; renal transplantation