<?xml version="1.0" encoding="UTF-8"?><xml><records><record><source-app name="Biblio" version="7.x">Drupal-Biblio</source-app><ref-type>17</ref-type><contributors><authors><author><style face="normal" font="default" size="100%">Siobhán B Carr</style></author><author><style face="normal" font="default" size="100%">Elliot McClenaghan</style></author><author><style face="normal" font="default" size="100%">Alexander Elbert</style></author><author><style face="normal" font="default" size="100%">Albert Faro</style></author><author><style face="normal" font="default" size="100%">Rebecca Cosgriff</style></author><author><style face="normal" font="default" size="100%">Olzhas Abdrakhmanov</style></author><author><style face="normal" font="default" size="100%">Keith Brownlee</style></author><author><style face="normal" font="default" size="100%">Pierre-Régis Burgel</style></author><author><style face="normal" font="default" size="100%">Catherine A Byrnes</style></author><author><style face="normal" font="default" size="100%">Stephanie Y Cheng</style></author><author><style face="normal" font="default" size="100%">Colombo, Carla</style></author><author><style face="normal" font="default" size="100%">Harriet Corvol</style></author><author><style face="normal" font="default" size="100%">Géraldine Daneau</style></author><author><style face="normal" font="default" size="100%">Christopher H Goss</style></author><author><style face="normal" font="default" size="100%">Gulmans, Vincent</style></author><author><style face="normal" font="default" size="100%">Hector Gutierrez</style></author><author><style face="normal" font="default" size="100%">Harutyunyan, Satenik</style></author><author><style face="normal" font="default" size="100%">Meagan Helmick</style></author><author><style face="normal" font="default" size="100%">Andreas Jung</style></author><author><style face="normal" font="default" size="100%">Kashirskaya, Nataliya</style></author><author><style face="normal" font="default" size="100%">Edward McKone</style></author><author><style face="normal" font="default" size="100%">Joel Melo</style></author><author><style face="normal" font="default" size="100%">Middleton, Peter G</style></author><author><style face="normal" font="default" size="100%">Pedro Mondejar-Lopez</style></author><author><style face="normal" font="default" size="100%">de Monestrol, Isabelle</style></author><author><style face="normal" font="default" size="100%">Nährlich, Lutz</style></author><author><style face="normal" font="default" size="100%">Rita Padoan</style></author><author><style face="normal" font="default" size="100%">Megan Parker</style></author><author><style face="normal" font="default" size="100%">M Dolores Pastor-Vivero</style></author><author><style face="normal" font="default" size="100%">Samar Rizvi</style></author><author><style face="normal" font="default" size="100%">Rasa Ruseckaite</style></author><author><style face="normal" font="default" size="100%">Marco Salvatore</style></author><author><style face="normal" font="default" size="100%">Luiz Vicente R F da Silva-Filho</style></author><author><style face="normal" font="default" size="100%">Nick Versmessen</style></author><author><style face="normal" font="default" size="100%">Marco Zampoli</style></author><author><style face="normal" font="default" size="100%">Bruce C Marshall</style></author><author><style face="normal" font="default" size="100%">Anne L Stephenson</style></author></authors><translated-authors><author><style face="normal" font="default" size="100%">CF Registry Global Collaboration</style></author></translated-authors></contributors><titles><title><style face="normal" font="default" size="100%">Factors associated with clinical progression to severe COVID-19 in people with cystic fibrosis: A global observational study.</style></title><secondary-title><style face="normal" font="default" size="100%">J Cyst Fibros</style></secondary-title></titles><keywords><keyword><style  face="normal" font="default" size="100%">COVID-19</style></keyword><keyword><style  face="normal" font="default" size="100%">Cystic Fibrosis</style></keyword><keyword><style  face="normal" font="default" size="100%">Cystic Fibrosis Transmembrane Conductance Regulator</style></keyword><keyword><style  face="normal" font="default" size="100%">Ethnicity</style></keyword><keyword><style  face="normal" font="default" size="100%">Humans</style></keyword><keyword><style  face="normal" font="default" size="100%">Minority Groups</style></keyword><keyword><style  face="normal" font="default" size="100%">Oxygen</style></keyword><keyword><style  face="normal" font="default" size="100%">SARS-CoV-2</style></keyword></keywords><dates><year><style  face="normal" font="default" size="100%">2022</style></year><pub-dates><date><style  face="normal" font="default" size="100%">2022 07</style></date></pub-dates></dates><volume><style face="normal" font="default" size="100%">21</style></volume><language><style face="normal" font="default" size="100%">eng</style></language><abstract><style face="normal" font="default" size="100%">&lt;p&gt;&lt;b&gt;BACKGROUND: &lt;/b&gt;This international study aimed to characterise the impact of acute SARS-CoV-2 infection in people with cystic fibrosis and investigate factors associated with severe outcomes. Methods Data from 22 countries prior to 13 December 2020 and the introduction of vaccines were included. It was de-identified and included patient demographics, clinical characteristics, treatments, outcomes and sequalae following SARS-CoV-2 infection. Multivariable logistic regression was used to investigate factors associated with clinical progression to severe COVID-19, using the primary outcome of hospitalisation with supplemental oxygen.&lt;/p&gt;

&lt;p&gt;&lt;b&gt;RESULTS: &lt;/b&gt;SARS-CoV-2 was reported in 1555 people with CF, 1452 were included in the analysis. One third were aged &amp;lt;18 years, and 9.4% were solid-organ transplant recipients. 74.5% were symptomatic and 22% were admitted to hospital. In the non-transplanted cohort, 39.5% of patients with ppFEV1&amp;lt;40% were hospitalised with oxygen verses 3.2% with ppFEV &amp;gt;70%: a 17-fold increase in odds. Worse outcomes were independently associated with older age, non-white race, underweight body mass index, and CF-related diabetes. Prescription of highly effective CFTR modulator therapies was associated with a significantly reduced odds of being hospitalised with oxygen (AOR 0.43 95%CI 0.31-0.60 p&amp;lt;0.001). Transplanted patients were hospitalised with supplemental oxygen therapy (21.9%) more often than non-transplanted (8.8%) and was independently associated with the primary outcome (Adjusted OR 2.45 95%CI 1.27-4.71 p=0.007).&lt;/p&gt;

&lt;p&gt;&lt;b&gt;CONCLUSIONS: &lt;/b&gt;This is the first study to show that there is a protective effect from the use of CFTR modulator therapy and that people with CF from an ethnic minority are at more risk of severe infection with SARS-CoV-2.&lt;/p&gt;
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