caa a22 4500 475748182 CHVBK 20180406123519.0 cr unu---uuuuu 170329e20001101xx s 000 0 eng 10.1023/A:1026416017763 doi (NATIONALLICENCE)springer-10.1023/A:1026416017763 Primary Immunodeficiency Diseases in Norway [Elektronische Daten] [Asbjørg Stray-Pedersen, Tore Abrahamsen, Stig Frøland] This study represents the first national epidemiological survey of primary immunodeficiency diseases in Norway. Uniform questionnaires were sent out in April 1998 to all hospital departments considered relevant. As of February 1999, a total of 372 patients have been registered, of whom 69 patients are deceased. With a population of 4.45 million people, the total prevalence of primary immunodeficiency diseases in Norway February 1, 1999 is 6.82 per 100.000 inhabitants. Distribution between the main immunodeficiency diagnoses is (a) antibody deficiencies 50.8%, (b) combined deficiencies included other immunodeficiency syndromes 12.4%, (c) complement deficiencies 21.0%, (d) phagocytic disorders 6.7%, (e) and immunodeficiency associated with other congenital diseases 9.1%. Compared to previous reports from other European countries, there is a smaller proportion of antibody deficiencies due to few IgA deficiencies registered and a large proportion of complement deficiencies due to many patients with hereditary angioedema. Plenum Publishing Corporation, 2000 Primary immunodeficiency nationallicence registry nationallicence Norway nationallicence Stray-Pedersen Asbjørg The Centre for Rare Disorders, Department of Medicine, Rikshospitalet, University of Oslo, Oslo, Norway aut Abrahamsen Tore Department of Pediatrics, Department of Medicine, Rikshospitalet, University of Oslo, Oslo, Norway aut Frøland Stig Section of Clinical Immunology and Infectious Diseases, Department of Medicine, Rikshospitalet, University of Oslo, Oslo, Norway aut Journal of Clinical Immunology Kluwer Academic Publishers-Plenum Publishers 20/6(2000-11-01), 477-485 0271-9142 20:6<477 2000 20 10875 https://doi.org/10.1023/A:1026416017763 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1023/A:1026416017763 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Stray-Pedersen Asbjørg The Centre for Rare Disorders, Department of Medicine, Rikshospitalet, University of Oslo, Oslo, Norway aut NATIONALLICENCE 700 1- Abrahamsen Tore Department of Pediatrics, Department of Medicine, Rikshospitalet, University of Oslo, Oslo, Norway aut NATIONALLICENCE 700 1- Frøland Stig Section of Clinical Immunology and Infectious Diseases, Department of Medicine, Rikshospitalet, University of Oslo, Oslo, Norway aut NATIONALLICENCE 773 0- Journal of Clinical Immunology Kluwer Academic Publishers-Plenum Publishers 20/6(2000-11-01), 477-485 0271-9142 20:6<477 2000 20 10875 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer