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Background Autosomal dominant polycystic kidney disease (ADPKD) is the leading genetic cause of end-stage renal failure (ESRF). The epidemiology of the incident ADPKD patient cohort requiring renal replacement therapy (RRT) in England and Wales has not been described. Methods We used a retrospective cohort design. Incident adult patients commencing RRT between 1 January 2000 and 31 December 2011 in England and Wales were identified from the UK Renal Registry. Patients were stratified into three groups based on primary renal diagnosis (PRD): (i) ADPKD, (ii) diabetes as PRD, (iii) individuals with another PRD (‘other’). Baseline demographics, comorbidity, care-related measures and outcomes including patient survival are described. Results A total of 52 608 individuals started RRT during the study period, 3598 (6.8%) had ADPKD, 12 137 (23.1%) diabetes as PRD and 36 873 had another PRD diagnosis. The median age of commencing RRT was 55 years in the ADPKD group compared with 62 and 66 years in those with diabetes or ‘other’ PRD, respectively. The median age of starting RRT did not change within the ADPKD group over the 10-year period. Median age at death was similar across all groups. The ADPKD group had a lower hazard for all-cause mortality compared with the ‘other’ PRD group (adjusted hazard ratio 0.45, 95% CI 0.38–0.53). In all PRD groups, crude mortality rates had improved between 2000–06 and 2007–11. Conclusion Although engaged in renal services earlier than some other patient groups, individuals with ADPKD start RRT at a younger age and this has remained unchanged over the last decade. Developing a nationwide cohort and an enhanced disease-specific dataset would facilitate a wide range of research and quality improvement initiatives to try to modify progression to ESRF and the course of RRT. [ABSTRACT FROM AUTHOR]