title: Outcomes of male patients with Alport syndrome undergoing renal replacement therapy.
abstract: OBJECTIVE
  Patients with the hereditary disease Alport syndrome commonly require renal replacement therapy (RRT) in the second or third decade of life. This study compared age at onset of RRT, renal allograft, and patient survival in men with Alport syndrome receiving various forms of RRT (peritoneal dialysis, hemodialysis, or transplantation) with those of men with other renal diseases.
  
  
  METHODS
  Patients with Alport syndrome receiving RRT identified from 14 registries in Europe were matched to patients with other renal diseases. A linear spline model was used to detect changes in the age at start of RRT over time. Kaplan-Meier method and Cox regression analysis were used to examine patient and graft survival.
  
  
  RESULTS
  Age at start of RRT among patients with Alport syndrome remained stable during the 1990s but increased by 6 years between 2000-2004 and 2005-2009. Survival of patients with Alport syndrome requiring dialysis or transplantation did not change between 1990 and 2009. However, patients with Alport syndrome had better renal graft and patient survival than matched controls. Numbers of living-donor transplantations were lower in patients with Alport syndrome than in matched controls.
  
  
  CONCLUSIONS
  These data suggest that kidney failure in patients with Alport syndrome is now being delayed compared with previous decades. These patients appear to have superior patient survival while undergoing dialysis and superior patient and graft survival after deceased-donor kidney transplantation compared with patients receiving RRT because of other causes of kidney failure.
authors.affiliation: Dept Nephrology&Rheumatology, University Medical Center Göttingen, Göttingen, Germany.
authors.forename: Johanna
authors.initials: J
authors.lastname: Temme
authors.forename: Anneke
authors.initials: A
authors.lastname: Kramer
authors.forename: Kitty J
authors.initials: KJ
authors.lastname: Jager
authors.forename: Katharina
authors.initials: K
authors.lastname: Lange
authors.forename: Frederick
authors.initials: F
authors.lastname: Peters
authors.forename: Gerhard-Anton
authors.initials: GA
authors.lastname: Müller
authors.forename: Reinhard
authors.initials: R
authors.lastname: Kramar
authors.forename: James G
authors.initials: JG
authors.lastname: Heaf
authors.forename: Patrik
authors.initials: P
authors.lastname: Finne
authors.forename: Runolfur
authors.initials: R
authors.lastname: Palsson
authors.forename: Anna V
authors.initials: AV
authors.lastname: Reisæter
authors.forename: Andries J
authors.initials: AJ
authors.lastname: Hoitsma
authors.forename: Wendy
authors.initials: W
authors.lastname: Metcalfe
authors.forename: Maurizio
authors.initials: M
authors.lastname: Postorino
authors.forename: Oscar
authors.initials: O
authors.lastname: Zurriaga
authors.forename: Julio P
authors.initials: JP
authors.lastname: Santos
authors.forename: Pietro
authors.initials: P
authors.lastname: Ravani
authors.forename: Faical
authors.initials: F
authors.lastname: Jarraya
authors.forename: Enrico
authors.initials: E
authors.lastname: Verrina
authors.forename: Friedo W
authors.initials: FW
authors.lastname: Dekker
authors.forename: Oliver
authors.initials: O
authors.lastname: Gross
mesh_terms: D000328:Adult; D000367:Age Factors; D006085:Graft Survival; D006801:Humans; D053208:Kaplan-Meier Estimate; D007674:Kidney Diseases / Q000628:therapy; D007676:Kidney Failure, Chronic / Q000209:etiology / Q000601:surgery / Q000628:therapy*; D016030:Kidney Transplantation; D008297:Male; D009394:Nephritis, Hereditary / Q000150:complications / Q000601:surgery / Q000628:therapy*; D010530:Peritoneal Dialysis; D016016:Proportional Hazards Models; D006435:Renal Dialysis; D015996:Survival Rate; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult