title: Timing and outcome of renal replacement therapy in patients with congenital malformations of the kidney and urinary tract.
abstract: OBJECTIVE
  Congenital anomalies of the kidney and urinary tract (CAKUT) are the leading cause of ESRD in children, but the proportion of patients with individual CAKUT entities progressing to ESRD during adulthood and their long-term clinical outcomes are unknown. This study assessed the age at onset of renal replacement therapy (RRT) and patient and renal graft survival in patients with CAKUT across the entire age range.
  
  
  METHODS
  Patients with CAKUT were compared with age-matched patients who were undergoing RRT for other renal disorders on the basis of data from the European Renal Association-European Dialysis and Transplant Association Registry. Competing risk and Cox regression analyses were conducted.
  
  
  RESULTS
  Of 212,930 patients commencing RRT from 1990 to 2009, 4765 (2.2%) had renal diagnoses consistent with CAKUT. The proportion of incident RRT patients with CAKUT decreased from infancy to childhood and then increased until age 15-19 years, followed by a gradual decline throughout adulthood. Median age at RRT start was 31 years in the CAKUT cohort and 61 years in the non-CAKUT cohort (P<0.001). RRT was started earlier (median, 16 years) in patients with isolated renal dysplasia than in those with renal hypoplasia and associated urinary tract disorders (median, 29.5-39.5 years). Patients with CAKUT survived longer than age- and sex-matched non-CAKUT controls because of lower cardiovascular mortality (10-year survival rate, 76.4% versus 70.7%; P<0.001).
  
  
  CONCLUSIONS
  CAKUT leads to ESRD more often at adult than pediatric age. Treatment outcomes differ from those of acquired kidney diseases and vary within CAKUT subcategories.
authors.affiliation: Division of Pediatric Nephrology, Center for Pediatrics and Adolescent Medicine, Heidelberg,Germany.
authors.forename: Elke
authors.initials: E
authors.lastname: Wühl
authors.forename: Karlijn J
authors.initials: KJ
authors.lastname: van Stralen
authors.forename: Enrico
authors.initials: E
authors.lastname: Verrina
authors.forename: Anna
authors.initials: A
authors.lastname: Bjerre
authors.forename: Christoph
authors.initials: C
authors.lastname: Wanner
authors.forename: James Goya
authors.initials: JG
authors.lastname: Heaf
authors.forename: Oscar
authors.initials: O
authors.lastname: Zurriaga
authors.forename: Andries
authors.initials: A
authors.lastname: Hoitsma
authors.forename: Patrick
authors.initials: P
authors.lastname: Niaudet
authors.forename: Runolfur
authors.initials: R
authors.lastname: Palsson
authors.forename: Pietro
authors.initials: P
authors.lastname: Ravani
authors.forename: Kitty J
authors.initials: KJ
authors.lastname: Jager
authors.forename: Franz
authors.initials: F
authors.lastname: Schaefer
mesh_terms: D000293:Adolescent; D000328:Adult; D017677:Age Distribution; D017668:Age of Onset; D000368:Aged; D002648:Child; D002675:Child, Preschool; D005260:Female; D006085:Graft Survival; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D007668:Kidney / Q000002:abnormalities*; D007676:Kidney Failure, Chronic / Q000151:congenital / Q000401:mortality* / Q000628:therapy*; D016030:Kidney Transplantation / Q000401:mortality*; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D016016:Proportional Hazards Models; D012042:Registries / Q000706:statistics & numerical data; D017582:Renal Replacement Therapy / Q000401:mortality*; D012307:Risk Factors; D014551:Urinary Tract / Q000002:abnormalities*; D044465:White People / Q000706:statistics & numerical data