Coronavirus disease 2019 in patients with inborn errors of immunity: An international study.
Coronavirus disease 2019 in patients with inborn errors of immunity: An international study.
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DOI：
10.1016/j.jaci.2020.09.010
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发表时间：
2021-03
期刊：
影响因子：
--
通讯作者：
Tangye SG
中科院分区：
文献类型：
--
作者：
Meyts I;Bucciol G;Quinti I;Neven B;Fischer A;Seoane E;Lopez-Granados E;Gianelli C;Robles-Marhuenda A;Jeandel PY;Paillard C;Sankaran VG;Demirdag YY;Lougaris V;Aiuti A;Plebani A;Milito C;Dalm VA;Guevara-Hoyer K;Sánchez-Ramón S;Bezrodnik L;Barzaghi F;Gonzalez-Granado LI;Hayman GR;Uzel G;Mendonça LO;Agostini C;Spadaro G;Badolato R;Soresina A;Vermeulen F;Bosteels C;Lambrecht BN;Keller M;Mustillo PJ;Abraham RS;Gupta S;Ozen A;Karakoc-Aydiner E;Baris S;Freeman AF;Yamazaki-Nakashimada M;Scheffler-Mendoza S;Espinosa-Padilla S;Gennery AR;Jolles S;Espinosa Y;Poli MC;Fieschi C;Hauck F;Cunningham-Rundles C;Mahlaoui N;IUIS Committee of Inborn Errors of Immunity;Warnatz K;Sullivan KE;Tangye SG
严重急性呼吸综合征冠状病毒2（SARS-CoV-2）感染对罕见先天性免疫缺陷（IEI）患者的影响尚不确定，IEI是2019年有发生严重冠状病毒病风险的人群。这不仅与这些患者有关，而且与一般人群有关，因为IEI的研究可以揭示宿主防御的关键要求。我们试图描述IEI中SARS-CoV-2感染的表现、表现和结局，以告知医生并提高对SARS-CoV-2宿主防御的理解。在全球范围内向参与IEI患者护理和宣传的科学、医学和患者协会分发了参与回顾性研究的邀请。我们收集了94例SARS-CoV-2感染的IEI患者的信息。他们的中位年龄为25至34岁。53例患者（56%）患有一抗缺乏症，9例（9.6%）患有免疫失调综合征，6例（6.4%）吞噬细胞缺陷，7例（7.4%）自身炎症性疾病，14例（15%）联合免疫缺陷，3例（3%）先天免疫缺陷，2例（2%）骨髓衰竭。10例无症状，25例作为门诊患者治疗，28例需要住院，无需重症监护或通气，13例需要无创通气或输氧，18例入住重症监护室，12例需要有创通气，3例需要体外膜肺氧合。9名患者（7名成人和2名儿童）死亡。这项研究表明，（1）超过30%的IEI患者患有2019年轻度冠状病毒病（COVID-19），（2）一般人群中导致严重疾病/死亡的风险因素似乎也会影响IEI患者，包括更多年轻患者。进一步的研究将确定与严重疾病风险增加相关的途径，这些途径对于预防SARS-CoV-2是非冗余或冗余的。
There is uncertainty about the impact of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection in individuals with rare inborn errors of immunity (IEI), a population at risk of developing severe coronavirus disease 2019. This is relevant not only for these patients but also for the general population, because studies of IEIs can unveil key requirements for host defense. We sought to describe the presentation, manifestations, and outcome of SARS-CoV-2 infection in IEI to inform physicians and enhance understanding of host defense against SARS-CoV-2. An invitation to participate in a retrospective study was distributed globally to scientific, medical, and patient societies involved in the care and advocacy for patients with IEI. We gathered information on 94 patients with IEI with SARS-CoV-2 infection. Their median age was 25 to 34 years. Fifty-three patients (56%) suffered from primary antibody deficiency, 9 (9.6%) had immune dysregulation syndrome, 6 (6.4%) a phagocyte defect, 7 (7.4%) an autoinflammatory disorder, 14 (15%) a combined immunodeficiency, 3 (3%) an innate immune defect, and 2 (2%) bone marrow failure. Ten were asymptomatic, 25 were treated as outpatients, 28 required admission without intensive care or ventilation, 13 required noninvasive ventilation or oxygen administration, 18 were admitted to intensive care units, 12 required invasive ventilation, and 3 required extracorporeal membrane oxygenation. Nine patients (7 adults and 2 children) died. This study demonstrates that (1) more than 30% of patients with IEI had mild coronavirus disease 2019 (COVID-19) and (2) risk factors predisposing to severe disease/mortality in the general population also seemed to affect patients with IEI, including more younger patients. Further studies will identify pathways that are associated with increased risk of severe disease and are nonredundant or redundant for protection against SARS-CoV-2.
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危及生命的Covid-19患者中针对I型IFN的自身抗体。
DOI：
10.1126/science.abd4585
发表时间：
2020-10-23
期刊：
Science (New York, N.Y.)
影响因子：
--
作者：
Bastard P;Rosen LB;Zhang Q;Michailidis E;Hoffmann HH;Zhang Y;Dorgham K;Philippot Q;Rosain J;Béziat V;Manry J;Shaw E;Haljasmägi L;Peterson P;Lorenzo L;Bizien L;Trouillet-Assant S;Dobbs K;de Jesus AA;Belot A;Kallaste A;Catherinot E;Tandjaoui-Lambiotte Y;Le Pen J;Kerner G;Bigio B;Seeleuthner Y;Yang R;Bolze A;Spaan AN;Delmonte OM;Abers MS;Aiuti A;Casari G;Lampasona V;Piemonti L;Ciceri F;Bilguvar K;Lifton RP;Vasse M;Smadja DM;Migaud M;Hadjadj J;Terrier B;Duffy D;Quintana-Murci L;van de Beek D;Roussel L;Vinh DC;Tangye SG;Haerynck F;Dalmau D;Martinez-Picado J;Brodin P;Nussenzweig MC;Boisson-Dupuis S;Rodríguez-Gallego C;Vogt G;Mogensen TH;Oler AJ;Gu J;Burbelo PD;Cohen JI;Biondi A;Bettini LR;D'Angio M;Bonfanti P;Rossignol P;Mayaux J;Rieux-Laucat F;Husebye ES;Fusco F;Ursini MV;Imberti L;Sottini A;Paghera S;Quiros-Roldan E;Rossi C;Castagnoli R;Montagna D;Licari A;Marseglia GL;Duval X;Ghosn J;HGID Lab;NIAID-USUHS Immune Response to COVID Group;COVID Clinicians;COVID-STORM Clinicians;Imagine COVID Group;French COVID Cohort Study Group;Milieu Intérieur Consortium;CoV-Contact Cohort;Amsterdam UMC Covid-19 Biobank;COVID Human Genetic Effort;Tsang JS;Goldbach-Mansky R;Kisand K;Lionakis MS;Puel A;Zhang SY;Holland SM;Gorochov G;Jouanguy E;Rice CM;Cobat A;Notarangelo LD;Abel L;Su HC;Casanova JL
通讯作者：
Casanova JL
影响因子：
12.7
作者：
Quartuccio, Luca;Sonaglia, Arianna;De Vita, Salvatore
通讯作者：
De Vita, Salvatore
DOI：
10.2807/1560-7917.es.2020.25.12.2000256
发表时间：
2020-03-01
期刊：
Euro surveillance : bulletin Europeen sur les maladies transmissibles = European communicable disease bulletin
影响因子：
--
作者：
Russell, Timothy W;Hellewell, Joel;Kucharski, Adam J
通讯作者：
Kucharski, Adam J
影响因子：
8.8
作者：
Levy, MM;Fink, MP;Ramsay, G
通讯作者：
Ramsay, G
影响因子：
20.3
作者：
Ito, T;Kanzler, H;Liu, YJ
通讯作者：
Liu, YJ