Journal of Clinical Immunology

, Volume 17, Issue 4, pp 333–339 | Cite as

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


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 


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. 1.
    Conley ME, Stiehm ER: Immunodeficiency disorders: General considerations. In Immunologic Disorders in Infants and Children, ER Stiehm (ed). Philadelphia, WB Saunders, 1996, pp 202–222Google Scholar
  2. 2.
    Hayakawa Y, Iwata T, Yata J, Kobayashi N: Primary immunodeficiency syndrome in Japan. I. Overview of a nationwide survey on primary immunodeficiency syndrome. J Clin Immunol 1:31–39, 1981Google Scholar
  3. 3.
    Luzi G, Businco L, Aiuti F: Primary immunodeficiency syndromes in Italy: A report of the national register in children and adults. J Clin Immunol 3:316–320, 1983Google Scholar
  4. 4.
    Fasth A: Primary immunodeficiency disorders in Sweden: Cases among children 1974–1979. J Clin Immunol 2:86–92, 1982Google Scholar
  5. 5.
    Ryser O, Morell A, Hitzig WH: Primary immunodeficiencies in Switzerland: First report of the national registry in adults and children. J Clin Immunol 8:479–485, 1988Google Scholar
  6. 6.
    Hertel T, Koch Ch: Updating of the Danish registry of primary immunodeficiencies. In Progress in Immunodeficiency Research and Therapy II, J Vossen, C Griscelli (eds). Amsterdam, Elsevier, 1986, pp 329–332Google Scholar
  7. 7.
    Español T, Garcia Arumí MR, Garcia Sanz JA, Muñoz Lopez F, Martín MA, Sierra JI, Plaza AM, Bosque M, Marco MT, Catalá M, Sáez A: Inmunodeficiencias primarias en Cataluña. Immunología 4:50–52, 1985Google Scholar
  8. 8.
    Matamoros N, Milá J, Raga S, Español T, Fontán G: Registro Español de Inmunodeficiencias primarias. Informe REDIP-1995. Inmunología 14:76, 1995Google Scholar
  9. 9.
    Primary immunodeficiency diseases: Report of a WHO Scientific Group. Clin Exp Immunol 99(Suppl 1):1–24, 1995Google Scholar
  10. 10.
    Abedi MR, Morgan G, Paganelli R, Hammarström L: Report from the ESID registry of primary immunodeficiencies. ESID-European concerted action (Biomed I). Newsletter 3:5–9, 1995Google Scholar
  11. 11.
    Armitage P, Berry G: Estadística para la investigación biomédica. Barcelona, Doyma SA, 1992, pp 470–473Google Scholar
  12. 12.
    INE: Censo de Población de 1981. Tomo I. Resultados Nacionales. Madrid, INE, 1989Google Scholar
  13. 13.
    INE: Padrón Municipal de habitantes (1-04-86). Tomo I. Resultados Nacionales. Características de la Población total. Madríd, INE, 1985Google Scholar
  14. 14.
    INE: Censo de Población de 1991. Tomo II. Resultados Autonómicos y Provinciales. Madrid, INE, 1994Google Scholar
  15. 15.
    INE: Censo de Población 1991. Tomo I. Resultados Nacionales. Características generales de la población. Madrid, INE, 1994Google Scholar
  16. 16.
    Toolan J, Mattin CA, Gooi HC: Primary immunodeficiency register. United Kingdom 1996. Abstracts. VIII Meeting of the European Society for Immunodeficiencies. Göteborg, ESID, 1996Google Scholar

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

Personalised recommendations