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Factors for severe outcomes following SARS-CoV-2 infection in people with cystic fibrosis in Europe
A. Jung, A. Orenti, F. Dunlevy, E. Aleksejeva, E. Bakkeheim, V. Bobrovnichy, SB. Carr, C. Colombo, H. Corvol, R. Cosgriff, G. Daneau, D. Dogru, P. Drevinek, AD. Vukic, I. Fajac, A. Fox, S. Fustik, V. Gulmans, S. Harutyunyan, E. Hatziagorou, I....
Language English Country Great Britain
Document type Journal Article
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- Journal Article MeSH
Background: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection in people with cystic fibrosis (pwCF) can lead to severe outcomes. Methods: In this observational study, the European Cystic Fibrosis Society Patient Registry collected data on pwCF and SARS-CoV-2 infection to estimate incidence, describe clinical presentation and investigate factors associated with severe outcomes using multivariable analysis. Results: Up to December 31, 2020, 26 countries reported information on 828 pwCF and SARS-CoV-2 infection. Incidence was 17.2 per 1000 pwCF (95% CI: 16.0-18.4). Median age was 24 years, 48.4% were male and 9.4% had lung transplants. SARS-CoV-2 incidence was higher in lung-transplanted (28.6; 95% CI: 22.7-35.5) versus non-lung-transplanted pwCF (16.6; 95% CI: 15.4-17.8) (p≤0.001).SARS-CoV-2 infection caused symptomatic illness in 75.7%. Factors associated with symptomatic SARS-CoV-2 infection were age >40 years, at least one F508del mutation and pancreatic insufficiency.Overall, 23.7% of pwCF were admitted to hospital, 2.5% of those to intensive care, and regretfully 11 (1.4%) died. Hospitalisation, oxygen therapy, intensive care, respiratory support and death were 2- to 6-fold more frequent in lung-transplanted versus non-lung-transplanted pwCF.Factors associated with hospitalisation and oxygen therapy were lung transplantation, cystic fibrosis-related diabetes (CFRD), moderate or severe lung disease and azithromycin use (often considered a surrogate marker for Pseudomonas aeruginosa infection and poorer lung function). Conclusion: SARS-CoV-2 infection yielded high morbidity and hospitalisation in pwCF. PwCF with forced expiratory volume in 1 s <70% predicted, CFRD and those with lung transplants are at particular risk of more severe outcomes.
1 Tsitsishvili Children's Clinic CF Centre Tblisi Georgia
AP HP Hôpital Cochin Service de Physiologie et Explorations Fonctionnelles Paris France
Centre for Cystic Fibrosis Hospital de Santa Maria Lisbon Portugal
Centre for Cystic Fibrosis University Children's Hospital Skopje North Macedonia
Cystic Fibrosis Centre University Hospital of Bratislava Bratislava Slovakia
Cystic Fibrosis Europe Brussels Belgium
Cystic Fibrosis Registry of Turkey Ankara Turkey
Cystic Fibrosis Trust London UK
Dept of Paediatrics Cystic Fibrosis Regional Support Centre University of Brescia Brescia
Dept of Paediatrics Mother Thereza Hospital Center Tirana Albania
Dept of Paediatrics Norwegian Cystic Fibrosis Registry Oslo University Hospital Oslo Norway
Dept of Pneumology Children's Clinical University Hospital Rīga Stradinš University Riga Latvia
Dept of Pulmonology Hôpital Robert Schuman Luxembourg Luxembourg
Dept of Respiratory Paediatrics Royal Brompton Hospital London UK
Dutch Cystic Fibrosis Foundation Baarn The Netherlands
Dziekanow Paediatric Hospital Cystic Fibrosis Centre Institute of Mother and Child Warsaw Poland
European Cystic Fibrosis Society Karup Denmark
Institute of Hereditary Pathology Ukrainian National Academy of Medical Sciences Lviv Ukraine
Medical School University of Cyprus Nicosia Cyprus
NHLI Imperial College London UK
Paediatric Pulmonology University Children's Hospital Zurich Zurich Switzerland
Pediatric Clinic Alexandrovska University Hospital Medical University Sofia Bulgaria
Pediatric Pneumology and Cystic Fibrosis Unit Osakidetza Hospital Universitario Cruces Bizkaia Spain
Pulmonary Institute Schneider Children's Medical Center of Israel Petah Tikva Israel
Regional Respiratory Centre Belfast City Hospital Belfast UK
Sackler Faculty of Medicine Tel Aviv University Tel Aviv Israel
Sciensano Epidemiology and Public Health Health Services Research Brussels Belgium
Scientific Board of Italian CF Registry Rome Italy
St Vincent's University Hospital and University College Dublin School of Medicine Dublin Ireland
Stockholm CF Centre Karolinska University Hospital Karolinska Institutet Stockholm Sweden
Université Paris Descartes Sorbonne Paris Cité Paris France
University Hospital Centre Zagreb Cystic Fibrosis Centre Paediatrics and Adults Zagreb Croatia
Wellcome Wolfson Institute for Experimental Medicine Queen's University Belfast Belfast UK
Yerevan University CF Centre Muratsan Hospital Yerevan Armenia
References provided by Crossref.org
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- $a Background: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection in people with cystic fibrosis (pwCF) can lead to severe outcomes. Methods: In this observational study, the European Cystic Fibrosis Society Patient Registry collected data on pwCF and SARS-CoV-2 infection to estimate incidence, describe clinical presentation and investigate factors associated with severe outcomes using multivariable analysis. Results: Up to December 31, 2020, 26 countries reported information on 828 pwCF and SARS-CoV-2 infection. Incidence was 17.2 per 1000 pwCF (95% CI: 16.0-18.4). Median age was 24 years, 48.4% were male and 9.4% had lung transplants. SARS-CoV-2 incidence was higher in lung-transplanted (28.6; 95% CI: 22.7-35.5) versus non-lung-transplanted pwCF (16.6; 95% CI: 15.4-17.8) (p≤0.001).SARS-CoV-2 infection caused symptomatic illness in 75.7%. Factors associated with symptomatic SARS-CoV-2 infection were age >40 years, at least one F508del mutation and pancreatic insufficiency.Overall, 23.7% of pwCF were admitted to hospital, 2.5% of those to intensive care, and regretfully 11 (1.4%) died. Hospitalisation, oxygen therapy, intensive care, respiratory support and death were 2- to 6-fold more frequent in lung-transplanted versus non-lung-transplanted pwCF.Factors associated with hospitalisation and oxygen therapy were lung transplantation, cystic fibrosis-related diabetes (CFRD), moderate or severe lung disease and azithromycin use (often considered a surrogate marker for Pseudomonas aeruginosa infection and poorer lung function). Conclusion: SARS-CoV-2 infection yielded high morbidity and hospitalisation in pwCF. PwCF with forced expiratory volume in 1 s <70% predicted, CFRD and those with lung transplants are at particular risk of more severe outcomes.
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