title: Changing referral characteristics of patients with autosomal dominant polycystic kidney disease.
abstract: OBJECTIVE
  Autosomal dominant polycystic kidney disease is the most frequent life-threatening hereditary disease. The study objective was to assess whether the clinical characteristics of patients with autosomal dominant polycystic kidney disease who are referred to a major autosomal dominant polycystic kidney disease center have changed over time.
  
  
  METHODS
  The clinical characteristics of patients with autosomal dominant polycystic kidney disease were compared between period A (1961-1990) and period B (1991-2011). The study took place at the Autosomal Dominant Polycystic Kidney Disease Center at the University of Colorado. A total of 837 patients referred with autosomal dominant polycystic kidney disease were included. Blood pressure control and renin-angiotensin-aldosterone system inhibition were instituted. Renal function, blood pressure, end-stage renal disease, and mortality were analyzed.
  
  
  RESULTS
  The results in period B compared with period A demonstrated an earlier age of autosomal dominant polycystic kidney disease diagnosis (29 vs 35 years, P < .001), lower mean blood pressure (129/82 vs 142/91 mm Hg, P < .001), better estimated glomerular filtration rate (63.6 vs 44.6 mL/min, P < .001), and more therapy with angiotensin-converting enzyme inhibition (42.5% vs 13.6%, P < .001). Time from birth to end-stage renal disease (52.8 ± 0.6 vs 49.1 ± 0.6 years, P < .001) and birth to death (63.5 ± 1.5 years vs 57.2 ± 1.0 years, P < .001) was longer in period B compared with period A when adjusted for age at diagnosis, sex, and estimated glomerular filtration rate. The study was retrospective, which is a limitation.
  
  
  CONCLUSIONS
  In period B, there was significantly better blood pressure control, more renin-angiotensin-aldosterone system inhibition, better preservation of renal function, and a longer period from birth to end-stage renal disease and death.
authors.affiliation: Division of Renal Diseases and Hypertension, University of Colorado Denver, Aurora, CO 80045, USA.
authors.forename: Imed
authors.initials: I
authors.lastname: Helal
authors.forename: Kim
authors.initials: K
authors.lastname: McFann
authors.forename: Berenice
authors.initials: B
authors.lastname: Reed
authors.forename: Xiang-Dong
authors.initials: XD
authors.lastname: Yan
authors.forename: Robert W
authors.initials: RW
authors.lastname: Schrier
mesh_terms: D000328:Adult; D016009:Chi-Square Distribution; D003120:Colorado / Q000453:epidemiology; D003710:Demography; D005260:Female; D006801:Humans; D008297:Male; D016891:Polycystic Kidney, Autosomal Dominant / Q000401:mortality / Q000628:therapy*; D012017:Referral and Consultation; D012042:Registries; D018709:Statistics, Nonparametric; D016896:Treatment Outcome
keywords: Angiotensin-converting enzyme inhibitor; Blood pressure; Chronic kidney disease; Renal progression