Journal of Clinical Immunology

, Volume 17, Issue 4, pp 333–339

Primary Immunodeficiency Syndrome in Spain: First Report of the National Registry in Children and Adults

  • N. Matamoros Florí
  • J. Mila Llambi
  • T. Español Boren
  • S. Raga Borja
  • G. Fontan Casariego
Article

DOI: 10.1023/A:1027382916924

Cite this article as:
Florí, N.M., Llambi, J.M., Boren, T.E. et al. J Clin Immunol (1997) 17: 333. doi:10.1023/A:1027382916924

Abstract

The Spanish Registry for Primary Immunodeficiency Diseases (REDIP) was organized in 1993. One thousand sixty-nine cases of primary immunodeficiency diseases (PID) were registered in patients diagnosed between January 1980 and December 1995. PID diagnosis was made according to the World Health Organization criteria. The most frequent disorders were IgA deficiency (n = 394) and common variable immunodeficiency (n = 213), followed by severe combined immunodeficiency (n = 61), C1 inhibitor deficiency (n = 52), X-Iinked agammaglobulinemia (n = 49), IgG subclass deficiency (n = 48), and chronic granulomatous disease (n = 32). A comparative study between REDIP and data recently obtained from the European registry (ESID Report, 1995) revealed important differences between phagocytic disorders and complement deficiencies reported in both registries, 4.9 vs 8.7 and 6.0 vs 3.6, while percentages of predominantly antibody deficiencies and T cell and combined deficiencies concurred with those reported in the European registry, 69.3 vs 64.7 and 14.7 vs 20.2, respectively. The heterogeneous nature of the geographical distribution of cases submitted may indicate underdiagnosis of PID in some country areas; surprisingly, the interval between the onset of clinical symptoms and diagnosis was significant, even in immunodeficiency diseases, such as IgA deficiency, which are easy to diagnose.

Primary immunodeficiency diseases registry Spain 

Copyright information

© Plenum Publishing Corporation 1997

Authors and Affiliations

  • N. Matamoros Florí
    • 1
  • J. Mila Llambi
    • 1
  • T. Español Boren
    • 2
  • S. Raga Borja
    • 2
  • G. Fontan Casariego
    • 3
  1. 1.Department of ImmunologyHospital Son DuretaPalma de MallorcaSpain
  2. 2.Department of ImmunologyHospital Vall d'HebronBarcelonaSpain
  3. 3.Department of ImmunologyHospital La PazMadridSpain

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