Factors associated with clinical progression to severe COVID-19 in people with cystic fibrosis: A global observational study
Carr S.B. McClenaghan E. Elbert A. Faro A. Cosgriff R. Abdrakhmanov O. Brownlee K. Burgel P.-R. Byrnes C.A. Cheng S.Y. Colombo C. Corvol H. Daneau G. Goss C.H. Gulmans V. Gutierrez H. Harutyunyan S. Helmick M. Jung A. Kashirskaya N. McKone E. Melo J. Middleton P.G. Mondejar-Lopez P. de Monestrol I. Nährlich L. Padoan R. Parker M. Pastor-Vivero M.D. Rizvi S. Ruseckaite R. Salvatore M. da Silva-Filho L.V.R.F. Versmessen N. Zampoli M. Marshall B.C. Stephenson A.L. Bell S.C. Reid D. Wark P. Van Braeckel E. Gohy S. Knoop C. Pirson J. De Wachter E. Dupont L. Hanssens L. Nowé V. Lequesne M. Athanazio R.A. Meneses D.G. Boussaud V. Brinchault G. Coirier-Duet E. Dubus J.-C. Grenet D. de Miranda S. Beaumont L. Kanaan R. Lauraens M. Martin C. Mittaine M. Prévotat A. Reynaud-Gaubert M. Sermet-Gaudelus I. Tatopoulos A. Chiron R. Dalphin M.-L. Gerardin M. Weiss L. Wizla N. Ramel S. Plant B. Gunaratnam C. Jackson A. de Winter- de Groot K. Luijk B. Wesseling G. Kondratyeva E. Zhekayte E. Amelina E. Mukhina M. Simonova O. Alvarez-Fernandez A. Sole-Jover A. Cortell-Aznar I. Girón-Moreno R. López-Neyra A. Ramos-Cancelo I. Lázaro-Carrasco M. Vivero D.P. de Valbuena M.R. Prados-Sanchez C. Costa-Colomer J. Gartner S. Diab-Caceres L. Gilljam M. Lindberg U. Diemer S. Allenby M. Bourke S.J. Charman S.C. Collinson J. Dempsey O. Denniston S. Desai M. Duckers J. Etherington C. Gunn E. Higton A. Ho T. Hull J. Jones A. Ketchell R.I. Madge S.L. Maitra A. Mujtaba G. Nash E. Nazareth D. OBrien C. Onyon C. Orchard C. Peckham D. Rodgers H. Shafi N. Simmonds N. Southern K. Walshaw M. Watson D. Whitehouse J.L.
July 2022Elsevier B.V.
Journal of Cystic Fibrosis
2022#21Issue 4e221 - e231 pp.
Background: 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 13th 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. Results: SARS-CoV-2 was reported in 1555 people with CF, 1452 were included in the analysis. One third were aged <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<40% were hospitalised with oxygen verses 3.2% with ppFEV >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<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). Conclusions: 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.
Coronavirus , COVID-19 , Cystic fibrosis , SARS-CoV-2 , Transplant
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Royal Brompton Hospital, part of GSST NHS Foundation Trust, London, United Kingdom
NHLI, Imperial College, London, United Kingdom
Cystic Fibrosis Trust, London, United Kingdom
London School of Hygiene and Tropical Medicine, London, United Kingdom
Cystic Fibrosis Foundation, United States
The Second Childrens Hospital, Kazakhstan
Université de Paris, Inserm U1016, Institut Cochin and Cochin Hospital, Assistance Publique Hôpitaux de Paris (APHP), France
Starship Childrens Hospital and University of Auckland, Auckland, New Zealand
Cystic Fibrosis, Canada
Fondazione IRCCS Ca Granda Ospedale Maggiore Policlinico, University of Milan, Italy
Sorbonne Université, Inserm, centre de Recherche Saint-Antoine, Assistance Publique Hôpitaux de Paris (APHP), Hôpital Trousseau, Service de Pneumologie Pédiatrique, Paris, France
Sciensano, Belgium
University of Washington, Seattle, United States
Dutch CF Foundation NCFS, Baarn, Netherlands
University of Alabama at Birmingham, AL, United States
Yerevan University CF Centre, Muratsan Hospital, Yerevan, Armenia
University Childrens Hospital, Zurich, Switzerland
Research Centre for Medical Genetics, Russian Federation
St Vincents University Hospital, Dublin, Ireland
Instituto Nacional Del Torax, Chile
Westmead Hospital, Sydney, Australia
Hospital Clínico Universitario Virgen de la Arrixaca, Murcia, Spain
Department of Pediatrics, CLINTEC, Karolinska Institutet, Karolinska University Hospital Huddinge, Sweden
Department of Pediatrics, Justus-Liebig-University Giessen, Giessen, Germany
Cystic Fibrosis Support Center, Department of Paediatric, University of Brescia, Italy
Hospital Universitario Cruces, IIC Biocruces, Bizkaia, Spain
Monash University, Melbourne, Australia
Istituto Superiore di Sanita, National Center Rare Diseases, Undiagnosed Rare Diseases Interdepartmental Unit, Rome, Italy
Instituto da Criança HCFMUSP, São Paulo, Brazil
Expert by Experience, Ghent University, Belgium
University of Cape Town and Red Cross War Memorial Childrens Hospital, South Africa
Toronto Adult Cystic Fibrosis Centre, St. Michaels Hospital, Toronto, Canada
The Prince Charles Hospital, Brisbane, Australia
John Hunter Hospital, Newcastle, Australia
Ghent University Hospital, Belgium
Cliniques universitaires Saint-Luc, Belgium
Université Libre de Bruxelles, Belgium
CHR de la Citadelle, Belgium
Universitair Ziekenhuis Brussel, Belgium
University of Leuven, Belgium
HUDERF, Belgium
GZA Sint-Vincentius, Belgium
Antwerp University Hospital, Belgium
Instituto do Coração HCFMUSP, São Paulo, Brazil
Universidade Federal de Sergipe, Aracajú, Brazil
Cochin Hospital, Paris, France
Rennes, France
Versailles, France
Marseille, France
Foch Hospital, Suresnes, France
Caen, France
Toulouse, France
Lille, France
Necker Hospital, Paris, France
Nancy, France
Montpellier, France
Besancon, France
Robert Debre Hospital, Paris, France
Strasbourg, France
Roscoff, France
Cork University Hospital, Cork, Ireland
Beaumont Hospital, Dublin, Ireland
CF Registry of Ireland, Dublin, Ireland
Wilhelmina Childrens Hospital - University Medical Center Utrecht, Netherlands
University Medical Center Utrecht, Netherlands
Maastricht UMC, Netherlands
Research Centre for Medical Genetics, Russian Federation
Pulmonology Research Institute under FMBA of Russia, Russian Federation
Morozov State Pediatric Teaching Hospital, Russian Federation
Federal Academic Centre of Childrens Health, Healthcare Ministry of Russia, Russian Federation
Hospital Universitari Vall d´Hebrón, Barcelona, Spain
Hospital Universitario y Politécnico La Fe, Valencia, Spain
Hospital de la Princesa, Madrid, Spain
Hospital Universitario Ramón y Cajal, Madrid, Spain
Hospital Clínico Universitario de Valladolid, Valladolid, Spain
Hospital Universitario Puerta de Hierro, Spain
Hospital Universitario Cruces, Bizkaia, Spain
Hospital Universitario La Paz, Madrid, Spain
Hospital Universitario La Paz, Madrid, Spain
Hospital Sant Joan de Déu, Barcelona, Spain
Hospital Universitario 12 de Octubre, Madrid, Spain
Respiratory Medicine, Sahlgrenska University Hospital, Gothenburg, Sweden
Department of Clinical Sciences, Respiratory medicine and Allergology, Lund University, Skane University Hospital, Lund, Sweden
Department of Pediatrics, Lund University, Skane University Hospital, Lund, Sweden
Wessex Adult CF Service / University Hospital Southampton, United Kingdom
Royal Victoria Infirmary, United Kingdom
Cystic Fibrosis Trust, United Kingdom
Northampton General Hospital, United Kingdom
Aberdeen Royal Infirmary, United Kingdom
Heartlands Hospital, United Kingdom
Birmingham Childrens Hospital, United Kingdom
All Wales Adult Cystic Fibrosis Service, United Kingdom
St Jamess University Hospital, United Kingdom
Frimley Park Hospital, United Kingdom
Childrens Hospital, Oxford, United Kingdom
Royal Brompton Hospital / Imperial College London, United Kingdom
Royal Brompton Hospital, United Kingdom
Manchester Childrens Hospital, United Kingdom
Heartlands Hospital, United Kingdom
Liverpool Heart & Chest Hospital, United Kingdom
Great North Childrens Hospital, United Kingdom
Alexandra Hospital, United Kingdom
Scottish Cystic Fibrosis Service, United Kingdom
St Bartholomews Hospital, United Kingdom
Alder Hey Childrens Hospital / University of Liverpool, United Kingdom
Royal Brompton Hospital
NHLI
Cystic Fibrosis Trust
London School of Hygiene and Tropical Medicine
Cystic Fibrosis Foundation
The Second Childrens Hospital
Université de Paris
Starship Childrens Hospital and University of Auckland
Cystic Fibrosis
Fondazione IRCCS Ca Granda Ospedale Maggiore Policlinico
Sorbonne Université
Sciensano
University of Washington
Dutch CF Foundation NCFS
University of Alabama at Birmingham
Yerevan University CF Centre
University Childrens Hospital
Research Centre for Medical Genetics
St Vincents University Hospital
Instituto Nacional Del Torax
Westmead Hospital
Hospital Clínico Universitario Virgen de la Arrixaca
Department of Pediatrics
Department of Pediatrics
Cystic Fibrosis Support Center
Hospital Universitario Cruces
Monash University
Istituto Superiore di Sanita
Instituto da Criança HCFMUSP
Expert by Experience
University of Cape Town and Red Cross War Memorial Childrens Hospital
Toronto Adult Cystic Fibrosis Centre
The Prince Charles Hospital
John Hunter Hospital
Ghent University Hospital
Cliniques universitaires Saint-Luc
Université Libre de Bruxelles
CHR de la Citadelle
Universitair Ziekenhuis Brussel
University of Leuven
HUDERF
GZA Sint-Vincentius
Antwerp University Hospital
Instituto do Coração HCFMUSP
Universidade Federal de Sergipe
Cochin Hospital
Rennes
Versailles
Marseille
Foch Hospital
Caen
Toulouse
Lille
Necker Hospital
Nancy
Montpellier
Besancon
Robert Debre Hospital
Strasbourg
Roscoff
Cork University Hospital
Beaumont Hospital
CF Registry of Ireland
Wilhelmina Childrens Hospital - University Medical Center Utrecht
University Medical Center Utrecht
Maastricht UMC
Research Centre for Medical Genetics
Pulmonology Research Institute under FMBA of Russia
Morozov State Pediatric Teaching Hospital
Federal Academic Centre of Childrens Health
Hospital Universitari Vall d´Hebrón
Hospital Universitario y Politécnico La Fe
Hospital de la Princesa
Hospital Universitario Ramón y Cajal
Hospital Clínico Universitario de Valladolid
Hospital Universitario Puerta de Hierro
Hospital Universitario Cruces
Hospital Universitario La Paz
Hospital Universitario La Paz
Hospital Sant Joan de Déu
Hospital Universitario 12 de Octubre
Respiratory Medicine
Department of Clinical Sciences
Department of Pediatrics
Wessex Adult CF Service / University Hospital Southampton
Royal Victoria Infirmary
Cystic Fibrosis Trust
Northampton General Hospital
Aberdeen Royal Infirmary
Heartlands Hospital
Birmingham Childrens Hospital
All Wales Adult Cystic Fibrosis Service
St Jamess University Hospital
Frimley Park Hospital
Childrens Hospital
Royal Brompton Hospital / Imperial College London
Royal Brompton Hospital
Manchester Childrens Hospital
Heartlands Hospital
Liverpool Heart & Chest Hospital
Great North Childrens Hospital
Alexandra Hospital
Scottish Cystic Fibrosis Service
St Bartholomews Hospital
Alder Hey Childrens Hospital / University of Liverpool
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