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. 2018 Jun;192(3):284-291.
doi: 10.1111/cei.13125.

The United Kingdom Primary Immune Deficiency (UKPID) registry 2012 to 2017

B Shillitoe  1   2   3 , C Bangs  1   4 , D Guzman  1   5 , A R Gennery  1   2   3 , H J Longhurst  6 , M Slatter  2   3 , D M Edgar  7 , M Thomas  8 , A Worth  1   9 , A Huissoon  10 , P D Arkwright  4 , S Jolles  11 , H Bourne  12 , H Alachkar  13 , S Savic  14 , D S Kumararatne  6 , S Patel  15 , H Baxendale  16 , S Noorani  17 , P F K Yong  18 , C Waruiru  19 , V Pavaladurai  20 , P Kelleher  21 , R Herriot  22 , J Bernatonienne  23 , M Bhole  24 , C Steele  25 , G Hayman  26 , A Richter  27 , M Gompels  28 , C Chopra  29 , T Garcez  4 , M Buckland  1   5   9
Affiliations

The United Kingdom Primary Immune Deficiency (UKPID) registry 2012 to 2017

B Shillitoe et al. Clin Exp Immunol. 2018 Jun.

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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Figures

Figure 1
Figure 1
Geographical distribution of patients enrolled in the United Kingdom Primary Immune Deficiency (UKPID) Registry by city or town of documenting centre. The diameter of the circle is directly proportional to the number of patients enrolled in each centre.
Figure 2
Figure 2
Recruitment of total patient numbers into the United Kingdom Primary Immunodeficiency Registry.
Figure 3
Figure 3
UK incidence of registered primary immunodeficiency (PID) per 100 000 live births.
Figure 4
Figure 4
Number of primary immunodeficiency (PID) patients undergoing haematopoietic stem cell transplantation (HSCT) or gene therapy.
See this image and copyright information in PMC

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