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.
Publication types
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Research Support, Non-U.S. Gov't
MeSH terms
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Abnormalities, Multiple / epidemiology
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Abnormalities, Multiple / immunology
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Adolescent
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Agammaglobulinemia / complications
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Agammaglobulinemia / epidemiology
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Ataxia Telangiectasia / complications
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Ataxia Telangiectasia / epidemiology
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B-Lymphocytes / immunology
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Bacterial Infections / complications
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Bone and Bones / abnormalities
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Candidiasis, Chronic Mucocutaneous / complications
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Candidiasis, Chronic Mucocutaneous / epidemiology
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Child
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Child, Preschool
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Dysgammaglobulinemia / epidemiology
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Female
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Humans
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IgA Deficiency
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Immunologic Deficiency Syndromes / epidemiology*
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Immunologic Deficiency Syndromes / immunology
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Infant
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Infant, Newborn
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Male
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Neutropenia / complications
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Neutropenia / epidemiology
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Sweden
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Syndrome
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T-Lymphocytes / immunology
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Wiskott-Aldrich Syndrome / complications
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Wiskott-Aldrich Syndrome / epidemiology
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Wiskott-Aldrich Syndrome / genetics