{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,27]],"date-time":"2026-08-27T00:09:47Z","timestamp":1787789387499,"version":"build-2784847793"},"reference-count":48,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2023,2,8]],"date-time":"2023-02-08T00:00:00Z","timestamp":1675814400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Int J Immunogenetics"],"published-print":{"date-parts":[[2023,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Autoimmune neutropenia (AIN) in early childhood is caused by autoantibodies directed against antigens on the neutrophil membrane and is a frequent cause of neutropenia in children. Association of AIN with Fc\u03b3 receptor (FCGR) 3B variants is well described. In this study, we investigate genetic variations in the FCGR locus and copy number variation of <jats:italic>FCGR3B<\/jats:italic>. A total of 130 antibody\u2010positive AIN patients, 64 with specific anti\u2010HNA\u20101a antibodies and 66 with broad\u2010reacting anti\u2010Fc\u03b3RIIIb antibodies, were genotyped with a multiplex ligation probe assay and compared with healthy controls. Positive findings were confirmed with real\u2010time q\u2010PCR. We determined copy numbers of the FCGR2 and FCGR3 genes and the following SNPs: <jats:italic>FCGR2A<\/jats:italic> Q62W (rs201218628), <jats:italic>FCGR2A<\/jats:italic> H166R (rs1801274), <jats:italic>FCGR2B<\/jats:italic> I232T (rs1050501), <jats:italic>FCGR3A<\/jats:italic> V176F (rs396991), haplotypes for <jats:italic>FCGR2B\/C<\/jats:italic> promoters (rs3219018\/rs780467580), <jats:italic>FCGR2C<\/jats:italic> STOP\/ORF and HNA\u20101 genotypes in <jats:italic>FCGR3B<\/jats:italic> (rs447536, rs448740, rs52820103, rs428888 and rs2290834). Generally, associations were antibody specific, with all associations being representative of the anti\u2010HNA\u20101a\u2010positive group, while the only association found in the anti\u2010Fc\u03b3RIIIb group was with the HNA\u20101 genotype. An increased risk of AIN was observed for patients with one copy of <jats:italic>FCGR3B<\/jats:italic>; the HNA genotypes HNA\u20101a, HNA\u20101aa or HNA\u20101aac; the <jats:italic>FCGR2A<\/jats:italic> 166H and <jats:italic>FCGR2B<\/jats:italic> 232I variations; and no copies of <jats:italic>FCGR2B<\/jats:italic> 2B.4. A decreased risk was observed for HNA genotype HNA\u20101bb; <jats:italic>FCGR2A<\/jats:italic> 166R; <jats:italic>FCGR2B<\/jats:italic> 232T; and one copy of <jats:italic>FCGR2B<\/jats:italic> promoter 2B.4. We conclude that in our Danish cohort, there was a strong association between variation in the FCGR locus and AIN. The findings of different genetic associations between autoantibody groups could indicate the presence of two different disease entities and disease heterogeneity.<\/jats:p>","DOI":"10.1111\/iji.12614","type":"journal-article","created":{"date-parts":[[2023,2,9]],"date-time":"2023-02-09T02:08:31Z","timestamp":1675908511000},"page":"65-74","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Genetic variations in low\u2010to\u2010medium\u2010affinity Fc\u03b3 receptors and autoimmune neutropenia in early childhood in a Danish cohort"],"prefix":"10.1111","volume":"50","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4095-8291","authenticated-orcid":false,"given":"Kirstine","family":"Kl\u00f8ve\u2010Mogensen","sequence":"first","affiliation":[{"name":"Department of Clinical Immunology Aalborg University Hospital  Aalborg Denmark"},{"name":"Department of Clinical Medicine Aalborg University  Aalborg 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Aalborg University Hospital  Aalborg Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thure Mors","family":"Haunstrup","sequence":"additional","affiliation":[{"name":"Department of Clinical Immunology Aalborg University Hospital  Aalborg Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul","family":"Ratcliffe","sequence":"additional","affiliation":[{"name":"Department of medicine Huddinge Karolinska Institute  Stockholm Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Petter","family":"H\u00f6glund","sequence":"additional","affiliation":[{"name":"Department of medicine Huddinge Karolinska Institute  Stockholm Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Henrik","family":"Hasle","sequence":"additional","affiliation":[{"name":"Department of Pediatrics and Adolescent Medicine Aarhus University Hospital  Aarhus Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kaspar 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