Primary immunodeficiency disorders in Sweden: cases among children, 1974-1979.
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.
D000015:Abnormalities, Multiple / Q000453:epidemiology / Q000276:immunology; D000293:Adolescent; D000361:Agammaglobulinemia / Q000150:complications / Q000453:epidemiology; D001260:Ataxia Telangiectasia / Q000150:complications / Q000453:epidemiology; D001402:B-Lymphocytes / Q000276:immunology; D001424:Bacterial Infections / Q000150:complications; D001842:Bone and Bones / Q000002:abnormalities; D002178:Candidiasis, Chronic Mucocutaneous / Q000150:complications / Q000453:epidemiology; D002648:Child; D002675:Child, Preschool; D004406:Dysgammaglobulinemia / Q000453:epidemiology; D005260:Female; D006801:Humans; D017098:IgA Deficiency; D007153:Immunologic Deficiency Syndromes / Q000453:epidemiology* / Q000276:immunology; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D009503:Neutropenia / Q000150:complications / Q000453:epidemiology; D013548:Sweden; D013577:Syndrome; D013601:T-Lymphocytes / Q000276:immunology; D014923:Wiskott-Aldrich Syndrome / Q000150:complications / Q000453:epidemiology / Q000235:genetics