The German National Registry for Primary Immunodeficiencies (PI).

Clin Exp Immunol. 2013 Mar 15. doi: 10.1111/cei.12105. [Epub ahead of print]
The German National Registry for Primary Immunodeficiencies (PI).
Gathmann B, Grimbacher B, Kindle G, Goldacker S, Klima M, Belohradsky BH, Notheis G, Ehl S, Ritterbusch H, Baumann U, Meyer-Bahlburg A, Witte T, Schmidt R, Borte M, Borte S, Linde R, Schubert R, Bienemann K, Laws HJ, Dueckers G, Roesler J, Rothoeft T, Krüger R, Scharbatke EC, Masjosthusmann K, Wasmuth JC, Moser O, Kaiser P, Groß-Wieltsch U, Classen CF, Horneff G, Reiser V, Binder N, El-Helou SM, Klein C.
Source

Centre of Chronic Immunodeficiency (CCI), University Medical Center Freiburg and University of Freiburg, Germany.
Abstract

In 2009, a federally funded clinical and research consortium (pi-NET, www.pi-net.org) established the first national registry for primary immunodeficiencies (PI) in Germany. The registry contains clinical and genetic information on PI patients and is set up within the framework of the existing European Database for Primary Immunodeficiencies, run by the European Society for Primary Immunodeficiencies. Following the example of other national registries, a central data entry clerk has been employed to support data entry at the participating centres. Regulations for ethics approvals have presented a major challenge for participation of individual centres and have led to a delay of data entry in some cases. Data on 630 patients, entered in the European registry between 2004 and 2009, were incorporated into the national registry. From April 2009 to March 2012, the number of contributing centres increased from seven to 21, and 738 additional patients were reported, leading to a total number of 1,368 patients, of which 1,232 were alive. The age distribution of living patients differs significantly by gender, with twice as many males than females among children, but 15% more women than men in the age group 30 and older. The diagnostic delay between onset of symptoms and diagnosis has decreased for some PI over the past 20 years, but remains particularly high at a median of four years in common variable immunodeficiency (CVID), the most prevalent PI.

© 2013 British Society for Immunology, Clinical and Experimental Immunology.

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