Respiratory viral infections in otherwise healthy humans with inherited IRF7 deficiency

buir.contributor.authorÖzçelik, Tayfun
buir.contributor.orcidÖzçelik, Tayfun|0000-0001-5937-1082
dc.citation.epage17en_US
dc.citation.issueNumber7en_US
dc.citation.spage1en_US
dc.citation.volumeNumber219en_US
dc.contributor.authorÖzçelik, Tayfun
dc.contributor.authorCampbell, Tessa Mollie
dc.contributor.authorLiu, Zhiyong
dc.contributor.authorZhang, Qian
dc.contributor.authorMoncada-Velez, Marcela
dc.contributor.authorCovill, Laura E.
dc.contributor.authorZhang, Peng
dc.contributor.authorDarazam, Ilad Alavi
dc.contributor.authorBastard, Paul
dc.contributor.authorBizien, Lucy
dc.contributor.authorBucciol, Giorgia
dc.contributor.authorEnoksson, Sara Lind
dc.contributor.authorJouanguy, Emmanuelle
dc.contributor.authorKarabela, Şemsi Nur
dc.contributor.authorKhan, Taushif
dc.contributor.authorKendir-Demirkol, Yasemin
dc.contributor.authorArias, Andres Augusto
dc.contributor.authorMansouri, Davood
dc.contributor.authorMarits, Per
dc.contributor.authorMarr, Nico
dc.contributor.authorMigeotte, Isabelle
dc.contributor.authorMoens, Leen
dc.contributor.authorPellier, Isabelle
dc.contributor.authorSendel, Anton
dc.contributor.authorShahrooei, Mohammad
dc.contributor.authorEdvard Smith C.I.
dc.contributor.authorVandernoot, Isabelle
dc.contributor.authorWillekens, Karen
dc.contributor.authorBergman, Peter
dc.contributor.authorAbel, Laurent
dc.contributor.authorCobat, Aurélie
dc.contributor.authorCasanova, Jean-Laurent
dc.contributor.authorMeyts, Isabelle
dc.contributor.authorBryceson, Yenan T.
dc.date.accessioned2023-02-27T19:59:12Z
dc.date.available2023-02-27T19:59:12Z
dc.date.issued2022
dc.departmentDepartment of Molecular Biology and Geneticsen_US
dc.description.abstractAutosomal recessive IRF7 deficiency was previously reported in three patients with single critical influenza or COVID-19 pneumonia episodes. The patients’ fibroblasts and plasmacytoid dendritic cells produced no detectable type I and III IFNs, except IFN-β. Having discovered four new patients, we describe the genetic, immunological, and clinical features of seven IRF7-deficient patients from six families and five ancestries. Five were homozygous and two were compound heterozygous for IRF7 variants. Patients typically had one episode of pulmonary viral disease. Age at onset was surprisingly broad, from 6 mo to 50 yr (mean age 29 yr). The respiratory viruses implicated included SARS-CoV-2, influenza virus, respiratory syncytial virus, and adenovirus. Serological analyses indicated previous infections with many common viruses. Cellular analyses revealed strong antiviral immunity and expanded populations of influenza-and SARS-CoV-2–specific memory CD4+ and CD8+ Tcells. IRF7-deficient individuals are prone to viral infections of the respiratory tract but are otherwise healthy, potentially due to residual IFN-β and compensatory adaptive immunity. © 2022 Campbell et al.en_US
dc.identifier.doi10.1084/jem.20220202en_US
dc.identifier.eissn0022-1007
dc.identifier.urihttp://hdl.handle.net/11693/111854
dc.language.isoEnglishen_US
dc.publisherRockefeller University Pressen_US
dc.relation.isversionofhttps://dx.doi.org/10.1084/jem.20220202en_US
dc.source.titleJournal of Experimental Medicineen_US
dc.titleRespiratory viral infections in otherwise healthy humans with inherited IRF7 deficiencyen_US
dc.typeArticleen_US
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