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The United Kingdom Primary Immune Deficiency (UKPID) registry 2012 to 2017
Authors:B Shillitoe  C Bangs  D Guzman  A R Gennery  H J Longhurst  M Slatter  D M Edgar  M Thomas  A Worth  A Huissoon  P D Arkwright  S Jolles  H Bourne  H Alachkar  S Savic  D S Kumararatne  S Patel  H Baxendale  S Noorani  P F K Yong  C Waruiru  V Pavaladurai  P Kelleher  R Herriot  J Bernatonienne  M Bhole  C Steele  G Hayman  A Richter  M Gompels  C Chopra  T Garcez  M Buckland
Affiliation:1. On behalf of the UKPIN Registry Committee, UKPIN, London, UK;2. Great North Children's Hospital, Newcastle upon Tyne, UK;3. Institute of Cellular Medicine, Newcastle University, Newcastle upon Tyne, UK;4. Manchester University NHS Foundation Trust, Manchester, UK;5. UCL Centre for Immunodeficiency, Royal Free Hospital, London, UK;6. Addenbrooke's Hospital, Cambridge Universities NHS Foundation Trust, Cambridge, UK;7. The Royal Hospitals, Belfast, UK;8. NHS Greater Glasgow and Clyde, Glasgow, UK;9. Great Ormond Street Hospital and Institute of Child Health, London, UK;10. Heart of England NHS Foundation Trust, Birmingham, Birmingham, UK;11. University Hospital of Wales, Cardiff, UK;12. The Newcastle Upon Tyne Hospitals NHS Foundation Trust, Newcastle upon Tyne, UK;13. Salford Royal NHS Foundation Trust, Salford, UK;14. Leeds Teaching Hospitals NHS Trust, Leeds, UK;15. John Radcliffe Hospital, Headington, Oxford, UK;16. Papworth NHS Foundation Trust, Cambridge, UK;17. Sandwell and West Birmingham Hospitals NHS Trust, Birmingham, UK;18. Frimley Health NHS Foundation Trust, Frimley, UK;19. Sheffield Children's NHS Foundation Trust, Sheffield, UK;20. Lancashire Teaching Hospitals NHS Foundation Trust, Preston, UK;21. Royal Brompton and Harefield NHS Foundation Trust, London, UK;22. NHS Grampian, Aberdeen, UK;23. University Hospitals Bristol NHS Foundation Trust, Bristol, UK;24. The Dudley Group NHS Foundation Trust, Dudley, UK;25. NHS Tayside, Dundee, UK;26. Epsom and St Helier University Hospitals NHS Trust, St Helier, UK;27. University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK;28. North Bristol NHS Trust, Southmead Hospital, Bristol, UK;29. NHS Lothian, Edinburgh, UK
Abstract:This is the second report of the United Kingdom Primary Immunodeficiency (UKPID) registry. The registry will be a decade old in 2018 and, as of August 2017, had recruited 4758 patients encompassing 97% of immunology centres within the United Kingdom. This represents a doubling of recruitment into the registry since we reported on 2229 patients included in our first report of 2013. Minimum PID prevalence in the United Kingdom is currently 5·90/100 000 and an average incidence of PID between 1980 and 2000 of 7·6 cases per 100 000 UK live births. Data are presented on the frequency of diseases recorded, disease prevalence, diagnostic delay and treatment modality, including haematopoietic stem cell transplantation (HSCT) and gene therapy. The registry provides valuable information to clinicians, researchers, service commissioners and industry alike on PID within the United Kingdom, which may not otherwise be available without the existence of a well‐established registry.
Keywords:autoimmunity  autoinflammatory disease  human  immunodeficiency diseases  transplantation
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