{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T16:39:29Z","timestamp":1791218369392,"version":"4.1.0"},"reference-count":25,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2021,8,10]],"date-time":"2021-08-10T00:00:00Z","timestamp":1628553600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Allergy"],"published-print":{"date-parts":[[2022,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Genetic deficiencies of immune system, referred to as inborn errors of immunity (IEI), serve as a valuable model to study human immune responses. In a multicenter prospective cohort, we evaluated the outcome of SARS\u2010CoV\u20102 infection among IEI subjects and analyzed genetic and immune characteristics that determine adverse COVID\u201019 outcomes.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We studied 34\u00a0IEI patients (19M\/15F, 12 [min: 0.6\u2010max: 43] years) from six centers. We diagnosed COVID\u201019 infection by finding a positive SARS\u2010CoV\u20102 PCR test (<jats:italic>n<\/jats:italic>\u00a0=\u00a025) and\/or a lung tomography scoring (CORADS) \u22654 (<jats:italic>n<\/jats:italic>\u00a0=\u00a09). We recorded clinical and laboratory findings prospectively, fitted survival curves, and calculated fatality rates for the entire group and each IEI subclass.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Nineteen patients had combined immune deficiency (CID), six with predominantly antibody deficiency (PAD), six immune dysregulation (ID), two innate immune defects, and one in the autoinflammatory class. Overall, 23.5% of cases died, with disproportionate fatality rates among different IEI categories. PAD group had a relatively favorable outcome at any age, but CIDs and IDs were particularly vulnerable. At admission, presence of dyspnea was an independent risk for COVID\u2010related death (OR: 2.630, 95% CI; 1.198\u20135.776, <jats:italic>p<\/jats:italic>\u00a0&lt;\u00a0.001). Concerning predictive roles of laboratory markers at admission, deceased subjects compared to survived had significantly higher CRP, procalcitonin, Troponin\u2010T, ferritin, and total\u2010lung\u2010score (<jats:italic>p<\/jats:italic>\u00a0=\u00a0.020, <jats:italic>p<\/jats:italic>\u00a0=\u00a0.003, <jats:italic>p<\/jats:italic>\u00a0=\u00a0.014, <jats:italic>p<\/jats:italic>\u00a0=\u00a0.013, <jats:italic>p<\/jats:italic>\u00a0=\u00a0.020; respectively), and lower absolute lymphocyte count, albumin, and trough IgG (<jats:italic>p<\/jats:italic>\u00a0=\u00a0.012, <jats:italic>p<\/jats:italic>\u00a0=\u00a0.022, <jats:italic>p<\/jats:italic>\u00a0=\u00a0.011; respectively).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Our data disclose a highly vulnerable IEI subgroup particularly disadvantaged for COVID\u201019 despite their youth. Future studies should address this vulnerability and consider giving priority to these subjects in SARS\u2010Cov\u20102 therapy trials.<\/jats:p><\/jats:sec>","DOI":"10.1111\/all.15025","type":"journal-article","created":{"date-parts":[[2021,7,27]],"date-time":"2021-07-27T17:06:00Z","timestamp":1627405560000},"page":"282-295","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":37,"title":["Adverse COVID\u201019 outcomes in immune deficiencies: Inequality exists between subclasses"],"prefix":"10.1111","volume":"77","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4150-5200","authenticated-orcid":false,"given":"Elif","family":"Karakoc Aydiner","sequence":"first","affiliation":[{"name":"Faculty of Medicine Division of Pediatric Allergy\u2010Immunology Marmara University Istanbul Turkey"},{"name":"Istanbul Jeffrey Modell Diagnostic and Research Center for Primary Immunodeficiencies Istanbul Turkey"},{"name":"The Isil Berat Barlan 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