Abstract
A nationwide survey of symptomatic primary immunodeficiency disorders in children in Sweden during the 6-year period 1974–1979 resulted in 201 reported cases. The reported data for 174 children were analyzed. Antibody deficiencies were the most frequent (45.0%), followed by phagocytic disorders (22.0%) and combined T- and B-cell disorders (20.8%). Thirty-two children (18.4%) died, with the highest mortality for combined T- and B-cell defects. The sex ratio for all disorders was 2:1 for boys:girls. Neutropenia was significantly more common in the northern part of Sweden.
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References
World Health Organization: Immunodeficiency. Report of a WHO scientific group. Clin Immunol Immunopathol 13:296–359, 1979
National Central Bureau of Statistics: Statistical Abstract of Sweden 1980. Official Statistics of Sweden. Stockholm, National Central Bureau of Statistics, 67, 1980
Hanson LÅ, Lindholm L, Carlsson B, Fasth A, Fällström SP, Wadsworth C, Värendh G: T and B lymphocyte immunodeficiency in a male infant with increased suppressor cell activity. Scand J Immunol 5:1227–1231, 1976
Kostmann R: Infantile genetic agranulocytosis. A review with presentation of ten new cases. Acta Paediatr Scand 64:362–368, 1975
Hayakawa H, Iwata T, Yato J, Kobayashi N: Primary immunodeficiency syndrome in Japan. I. Overview of a nationwide survey on primary immunodeficiency syndrome. J Clin Immunol 1:31–39, 1981
Oxelius VA: Chronic infections in a family with hereditary deficiency of IgG 2 and IgG 4. Clin Exp Immunol 17:19–27, 1974
Conley ME, Beckwith JB, Mancer JFK, Tenkhoff L: The spectrum of DiGeorge syndrome. J Pediatr 94:883–890, 1979
Stiehm ER, Fulginiti VA: Immunologic disorders in infants and children. Philadelphia, Saunders, 1973
Hayward AR: Immunodeficiency. Curr Topics Immunol 6, 1977
Spector BD, Perry GS III, Kersey JH: Genetically determined immunodeficiency diseases (GDID) and malignancy: Report from the immunodeficiency-cancer registry. Clin Immunol Immunopathol 11:12–29, 1978
Ammann AJ, Hong R: Selective IgA deficiency: Presentation of 30 cases and a review of the literature. Medicine 50:223–236, 1971
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Fasth, A. Primary immunodeficiency disorders in Sweden: Cases among children, 1974–1979. J Clin Immunol 2, 86–92 (1982). https://doi.org/10.1007/BF00916891
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DOI: https://doi.org/10.1007/BF00916891