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


Ontology type: schema:ScholarlyArticle     


Article Info

DATE

1997-07

AUTHORS

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

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. More... »

PAGES

333-339

Identifiers

URI

http://scigraph.springernature.com/pub.10.1023/a:1027382916924

DOI

http://dx.doi.org/10.1023/a:1027382916924

DIMENSIONS

https://app.dimensions.ai/details/publication/pub.1023082909

PUBMED

https://www.ncbi.nlm.nih.gov/pubmed/9258772


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