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dc.contributor.authorLudviksson, Bjorn R.
dc.contributor.authorSigurdardottir, Sigurveig T.
dc.contributor.authorJohannsson, Johann Heidar
dc.contributor.authorHaraldsson, Asgeir
dc.contributor.authorHardarson, Thorgeir O.
dc.date.accessioned2015-08-18T14:47:12Zen
dc.date.available2015-08-18T14:47:12Zen
dc.date.issued2014-10-15en
dc.date.submitted2015en
dc.identifier.citationJournal of Clinical Immunology 2015, 35 (1):75-79en
dc.identifier.issn0271-9142en
dc.identifier.issn1573-2592en
dc.identifier.doi10.1007/s10875-014-0107-3en
dc.identifier.urihttp://hdl.handle.net/2336/575137en
dc.descriptionTo access publisher's full text version of this article click on the hyperlink at the bottom of the pageen
dc.description.abstractPrimary immunodeficiencies (PID) are rare heterogeneous diseases. Little is known about the prevalence of PID in Iceland and no national registry exists. The aim of the study was to describe the epidemiology of PID in Iceland. Using The European Society's for Immunodeficiencies (ESID) criteria for PID, information about individuals with a known PID between 1990 and 2010 in Iceland were collected from inpatient registries of the National University Hospital of Iceland, the Department of Immunology and from clinical immunologists. Selective IgA deficiency, mannan binding lectin deficiency and secondary immunodeficiencies were excluded Sixty six individuals met the study criteria, 35 of them (53 %) were females. Four patients died during the study period from PID- or treatment related complications and two moved abroad. In the beginning of 2011 there were 60 individuals living in Iceland with a known PID diagnosis meeting ESID's criteria. Estimated prevalence for PID in the Icelandic population of 318.452 habitants was 18.8 for 100.000 inhabitants. Predominantly antibody disorders comprised the largest category of PID in Iceland. The prevalence of PID is high in Iceland compared to reports from other nations. Our patient data are easily accessible and a central laboratory measures the immune parameters. This high prevalence may indicate that PID is more common than generally recognized.
dc.language.isoenen
dc.publisherSpringer /Plenum Publisheren
dc.relation.urlhttp://link.springer.com/10.1007/s10875-014-0107-3en
dc.relation.urlhttp://dx.doi.org/ 10.1007/s10875-014-0107-3en
dc.rightsArchived with thanks to Journal of Clinical Immunologyen
dc.subject.meshImmunologic Deficiency Syndromesen
dc.subject.meshIcelanden
dc.titleEpidemiology of Primary Immunodeficiency in Icelanden
dc.typeArticleen
dc.contributor.department[ 1 ] Landspitali Natl Univ Hosp Iceland, Dept Immunol, IS-101 Reykjavik, Iceland [ 2 ] Landspitali Natl Univ Hosp Iceland, Dept Genet & Mol Med, IS-101 Reykjavik, Iceland [ 3 ] Landspitali Natl Univ Hosp Iceland, Childrens Hosp Iceland, IS-101 Reykjavik, Icelanden
dc.identifier.journalJournal of Clinical Immunologyen
dc.rights.accessNational Consortium - Landsaðganguren
html.description.abstractPrimary immunodeficiencies (PID) are rare heterogeneous diseases. Little is known about the prevalence of PID in Iceland and no national registry exists. The aim of the study was to describe the epidemiology of PID in Iceland. Using The European Society's for Immunodeficiencies (ESID) criteria for PID, information about individuals with a known PID between 1990 and 2010 in Iceland were collected from inpatient registries of the National University Hospital of Iceland, the Department of Immunology and from clinical immunologists. Selective IgA deficiency, mannan binding lectin deficiency and secondary immunodeficiencies were excluded Sixty six individuals met the study criteria, 35 of them (53 %) were females. Four patients died during the study period from PID- or treatment related complications and two moved abroad. In the beginning of 2011 there were 60 individuals living in Iceland with a known PID diagnosis meeting ESID's criteria. Estimated prevalence for PID in the Icelandic population of 318.452 habitants was 18.8 for 100.000 inhabitants. Predominantly antibody disorders comprised the largest category of PID in Iceland. The prevalence of PID is high in Iceland compared to reports from other nations. Our patient data are easily accessible and a central laboratory measures the immune parameters. This high prevalence may indicate that PID is more common than generally recognized.


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