title: Outcomes of kidney transplantation in Alport syndrome compared with other forms of renal disease.
abstract: BACKGROUND
  Alport syndrome is an inherited renal disease characterized by hematuria, renal failure, hearing loss and a lamellated glomerular basement membrane. Patients with Alport syndrome who undergo renal transplantation have been shown to have patient and graft survival rates similar to or better than those of patients with other renal diseases.
  
  
  METHODS
  In this national case series, based in Beaumont Hospital Dublin, we studied the cohort of patients who underwent renal transplantation over the past 33 years, recorded prospectively in the Irish Renal Transplant Registry, and categorized them according to the presence or absence of Alport syndrome. The main outcomes assessed were patient and renal allograft survival.
  
  
  RESULTS
  Fifty-one patients diagnosed with Alport syndrome in Beaumont Hospital received 62 transplants between 1982 and 2014. The comparison group of non-Alport patients comprised 3430 patients for 3865 transplants. Twenty-year Alport patient survival rate was 70.2%, compared to 44.8% for patients with other renal diseases (p = .01). Factors associated with patient survival included younger age at transplantation as well as differences in recipient sex, donor age, cold ischemia time, and episodes of acute rejection. Twenty-year graft survival was 46.8% for patients with Alport syndrome compared to 30.2% for those with non-Alport disease (p = .11).
  
  
  CONCLUSIONS
  Adjusting for baseline differences between the groups, patients with end-stage kidney disease (ESKD) due to Alport syndrome have similar patient and graft survival to those with other causes of ESKD. This indicates that early diagnosis and management can lead to favorable outcomes for this patient cohort.
authors.affiliation: a Department of Nephrology , Beaumont Hospital , Dublin , Ireland.
authors.forename: Yvelynne P
authors.initials: YP
authors.lastname: Kelly
authors.affiliation: b Royal College of Surgeons in Ireland , Dublin , Ireland.
authors.forename: Anish
authors.initials: A
authors.lastname: Patil
authors.affiliation: b Royal College of Surgeons in Ireland , Dublin , Ireland.
authors.forename: Luke
authors.initials: L
authors.lastname: Wallis
authors.affiliation: c Department of Nephrology , University Hospital Galway , Galway , Ireland.
authors.forename: Susan
authors.initials: S
authors.lastname: Murray
authors.affiliation: d Department of Nephrology , Cork University Hospital , Cork , Ireland.
authors.forename: Saumitra
authors.initials: S
authors.lastname: Kant
authors.affiliation: e Department of Nephrology , University Hospital Limerick , Dooradoyle , Limerick , Ireland.
authors.forename: Mohammed A
authors.initials: MA
authors.lastname: Kaballo
authors.affiliation: e Department of Nephrology , University Hospital Limerick , Dooradoyle , Limerick , Ireland.
authors.forename: Liam
authors.initials: L
authors.lastname: Casserly
authors.affiliation: f Department of Pathology , Beaumont Hospital , Dublin , Ireland.
authors.forename: Brendan
authors.initials: B
authors.lastname: Doyle
authors.affiliation: f Department of Pathology , Beaumont Hospital , Dublin , Ireland.
authors.forename: Anthony
authors.initials: A
authors.lastname: Dorman
authors.affiliation: a Department of Nephrology , Beaumont Hospital , Dublin , Ireland.
authors.forename: Patrick
authors.initials: P
authors.lastname: O'Kelly
authors.affiliation: a Department of Nephrology , Beaumont Hospital , Dublin , Ireland.|b Royal College of Surgeons in Ireland , Dublin , Ireland.
authors.forename: Peter J
authors.initials: PJ
authors.lastname: Conlon
mesh_terms: D000328:Adult; D005260:Female; D006084:Graft Rejection / Q000453:epidemiology*; D006085:Graft Survival; D006801:Humans; D007494:Ireland; D053208:Kaplan-Meier Estimate; D007676:Kidney Failure, Chronic / Q000601:surgery*; D016030:Kidney Transplantation; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D009394:Nephritis, Hereditary / Q000150:complications* / Q000401:mortality; D016016:Proportional Hazards Models; D012042:Registries; D015996:Survival Rate; D014184:Transplantation, Homologous; D055815:Young Adult
keywords: Kidney transplantation; hematuria; hereditary nephritis; transplant complications; transplant outcomes; transplant rejection