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Determinants of early Elexacaftor-Tezacaftor-Ivacaftor use in adults with cystic fibrosis and preserved lung function: insights from a European multicenter survey.

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Title: Determinants of early Elexacaftor-Tezacaftor-Ivacaftor use in adults with cystic fibrosis and preserved lung function: insights from a European multicenter survey.
Authors: Gramegna A; Department of Pathophysiology and Transplantation, University of Milan, Milan, Italy; Respiratory Unit and Cystic Fibrosis Adult Center, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy., Magnoni C; Department of Pathophysiology and Transplantation, University of Milan, Milan, Italy; Respiratory Unit and Cystic Fibrosis Adult Center, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy., Premuda C; Respiratory Unit and Cystic Fibrosis Adult Center, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy. Electronic address: chiara.premuda@policlinico.mi.it., Alicandro G; Department of Pathophysiology and Transplantation, University of Milan, Milan, Italy; Mother and Child Department, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy., Akkerman O; University of Groningen, University Medical Center Groningen, Groningen, The Netherlands; Department of Pulmonary Diseases and Tuberculosis, Groningen, The Netherlands., Amorim A; Pulmonology Department, Unidade de Saúde Local S.João, Porto, Portugal; Faculty of Medicine, University of Porto, Porto, Portugal., Azevedo P; Cystic Fibrosis Centre, Centro Hospitalar Universitário Lisboa Norte, Lisboa, Portugal; Faculdade de Medicina, Centro Académico Universitário de Lisboa, Lisboa, Portugal., Bourke S; Adult Cystic Fibrosis Centre, Newcastle upon Tyne Hospitals NHS Trust, Newcastle upon Tyne, United Kingdom., Carnovale V; Department of Translational Medical Sciences, University of Naples 'Federico II', Cystic Fibrosis Centre Adult Unit, Naples, Italy., Diamantea F; Adult Cystic Fibrosis Unit, Sismanoglio General Hospital of Athens, Greece., Fila L; Department of Pneumology, Second Faculty of Medicine, Charles University, Prague, Czech Republic; Motol University Hospital, Prague, Czech Republic., Frost F; Department of Cardiovascular & Metabolic Medicine, Institute of Lifecourse and Medical Sciences, University of Liverpool, Liverpool, United Kingdom; Respiratory Department, Liverpool Heart and Chest Hospital NHS Foundation Trust, Liverpool, United Kingdom., Giron Moreno RM; Servicio de Neumología, Hospital Universitario La Princesa, Madrid 28006, Spain., Pescaru C; Center for Research and Innovation in Personalized Medicine of Respiratory Diseases (CRIPMRD), 'Victor Babes' University of Medicine and Pharmacy Timisoara, Timisoara 300041, Romania; Pulmonary Rehabilitation Center, Clinical Hospital of Infectious Diseases and Pulmonology,'Victor Babes, Timisoara 300310, Romania., Polverino E; Department of Respiratory Medicine (Adult Cystic Fibrosis Unit), Vall d'Hebron University Hospital, Vall d'Hebron Research Institute (VHIR), Barcelona, Spain; CIBER Respiratory Diseases (CIBERES). Instituto de Salud Carlos III, Madrid, Spain., Felipe Montiel A; Department of Respiratory Medicine (Adult Cystic Fibrosis Unit), Vall d'Hebron University Hospital, Vall d'Hebron Research Institute (VHIR), Barcelona, Spain., Rachel M; Institu University of Rzeszów Medical College, Rzeszów, Poland; Allergy and Cystic Fibrosis Clinic, Provincial Hospital No. 2, Rzeszów, Poland., Stoyanova GP; Cystic Fibrosis Center, Pediatric Clinic, UMHAT 'Alexandrovska', Sofia, Bulgaria; Pediatric Department, Medical University, Sofia, Bulgaria., Van Braeckel E; Department of Respiratory Medicine, Ghent University Hospital, Ghent, Belgium; Department of Internal Medicine and Paediatrics, Faculty of Medicine and Health Sciences, Ghent University, Ghent, Belgium; European Reference Network on rare respiratory diseases (ERN-LUNG), CF core network, Frankfurt, Germany., Volpi S; Cystic fibrosis center, Azienda Ospedaliera Universitaria Integrata Verona, Verona, Italy., Vukić-Dugac A; Cystic Fibrosis Centre for Children and Adults, Clinic for respiratory diseases Jordanovac, University Hospital Centre Zagreb, Croatia; School of Medicine, University of Zagreb, Croatia., Wege S; Department of Pulmonology and Respiratory Medicine, Thoraxklinik at the University Hospital of Heidelberg, Germany., Simmonds NJ; Adult Cystic Fibrosis Centre, Royal Brompton Hospital, part of Guy's and St Thomas's NHS Foundation Trust, London, United Kingdom; National Heart and Lung Institute, Imperial College, London, United Kingdom., Plant BJ; Cork Centre for Cystic Fibrosis (3CF), Cork University Hospital, University College Cork, Wilton, Cork, Ireland; The HRB funded Clinical Research Facility, University College Cork, Cork, Ireland; Department of Medicine, University College Cork, Cork, Ireland., Downey DG; Wellcome-Wolfson Institute for Experimental Medicine, Queen's University Belfast, Belfast, United Kingdom., van Koningsbruggen-Rietschel S; European Reference Network on rare respiratory diseases (ERN-LUNG), CF core network, Frankfurt, Germany; Cystic Fibrosis Centre Cologne, University Hospital, Faculty of Medicine and University of Cologne, Cologne, Germany; Centre for Rare Diseases, Faculty of Medicine and University Hospital Cologne, University of Cologne, Cologne, Germany., Burgel PR; European Reference Network on rare respiratory diseases (ERN-LUNG), CF core network, Frankfurt, Germany; Respiratory Medicine and French National CF Reference Center, Assistance Publique-Hôpitaux de Paris, Paris, France; Université Paris Cité, Institut Cochin, INSERM U1016, CNRS, Paris, France., Blasi F; Department of Pathophysiology and Transplantation, University of Milan, Milan, Italy; Respiratory Unit and Cystic Fibrosis Adult Center, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.
Source: Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society [J Cyst Fibros] 2026 Mar 19. Date of Electronic Publication: 2026 Mar 19.
Publication Type: Journal Article
Journal Info: Publisher: Elsevier Country of Publication: Netherlands NLM ID: 101128966 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1873-5010 (Electronic) Linking ISSN: 15691993 NLM ISO Abbreviation: J Cyst Fibros Subsets: MEDLINE
Database: MEDLINE Ultimate
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  Data: Determinants of early Elexacaftor-Tezacaftor-Ivacaftor use in adults with cystic fibrosis and preserved lung function: insights from a European multicenter survey.
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  Data: <searchLink fieldCode="AU" term="%22Gramegna+A%22">Gramegna A</searchLink>; Department of Pathophysiology and Transplantation, University of Milan, Milan, Italy; Respiratory Unit and Cystic Fibrosis Adult Center, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.<br /><searchLink fieldCode="AU" term="%22Magnoni+C%22">Magnoni C</searchLink>; Department of Pathophysiology and Transplantation, University of Milan, Milan, Italy; Respiratory Unit and Cystic Fibrosis Adult Center, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.<br /><searchLink fieldCode="AU" term="%22Premuda+C%22">Premuda C</searchLink>; Respiratory Unit and Cystic Fibrosis Adult Center, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy. Electronic address: chiara.premuda@policlinico.mi.it.<br /><searchLink fieldCode="AU" term="%22Alicandro+G%22">Alicandro G</searchLink>; Department of Pathophysiology and Transplantation, University of Milan, Milan, Italy; Mother and Child Department, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.<br /><searchLink fieldCode="AU" term="%22Akkerman+O%22">Akkerman O</searchLink>; University of Groningen, University Medical Center Groningen, Groningen, The Netherlands; Department of Pulmonary Diseases and Tuberculosis, Groningen, The Netherlands.<br /><searchLink fieldCode="AU" term="%22Amorim+A%22">Amorim A</searchLink>; Pulmonology Department, Unidade de Saúde Local S.João, Porto, Portugal; Faculty of Medicine, University of Porto, Porto, Portugal.<br /><searchLink fieldCode="AU" term="%22Azevedo+P%22">Azevedo P</searchLink>; Cystic Fibrosis Centre, Centro Hospitalar Universitário Lisboa Norte, Lisboa, Portugal; Faculdade de Medicina, Centro Académico Universitário de Lisboa, Lisboa, Portugal.<br /><searchLink fieldCode="AU" term="%22Bourke+S%22">Bourke S</searchLink>; Adult Cystic Fibrosis Centre, Newcastle upon Tyne Hospitals NHS Trust, Newcastle upon Tyne, United Kingdom.<br /><searchLink fieldCode="AU" term="%22Carnovale+V%22">Carnovale V</searchLink>; Department of Translational Medical Sciences, University of Naples 'Federico II', Cystic Fibrosis Centre Adult Unit, Naples, Italy.<br /><searchLink fieldCode="AU" term="%22Diamantea+F%22">Diamantea F</searchLink>; Adult Cystic Fibrosis Unit, Sismanoglio General Hospital of Athens, Greece.<br /><searchLink fieldCode="AU" term="%22Fila+L%22">Fila L</searchLink>; Department of Pneumology, Second Faculty of Medicine, Charles University, Prague, Czech Republic; Motol University Hospital, Prague, Czech Republic.<br /><searchLink fieldCode="AU" term="%22Frost+F%22">Frost F</searchLink>; Department of Cardiovascular & Metabolic Medicine, Institute of Lifecourse and Medical Sciences, University of Liverpool, Liverpool, United Kingdom; Respiratory Department, Liverpool Heart and Chest Hospital NHS Foundation Trust, Liverpool, United Kingdom.<br /><searchLink fieldCode="AU" term="%22Giron+Moreno+RM%22">Giron Moreno RM</searchLink>; Servicio de Neumología, Hospital Universitario La Princesa, Madrid 28006, Spain.<br /><searchLink fieldCode="AU" term="%22Pescaru+C%22">Pescaru C</searchLink>; Center for Research and Innovation in Personalized Medicine of Respiratory Diseases (CRIPMRD), 'Victor Babes' University of Medicine and Pharmacy Timisoara, Timisoara 300041, Romania; Pulmonary Rehabilitation Center, Clinical Hospital of Infectious Diseases and Pulmonology,'Victor Babes, Timisoara 300310, Romania.<br /><searchLink fieldCode="AU" term="%22Polverino+E%22">Polverino E</searchLink>; Department of Respiratory Medicine (Adult Cystic Fibrosis Unit), Vall d'Hebron University Hospital, Vall d'Hebron Research Institute (VHIR), Barcelona, Spain; CIBER Respiratory Diseases (CIBERES). Instituto de Salud Carlos III, Madrid, Spain.<br /><searchLink fieldCode="AU" term="%22Felipe+Montiel+A%22">Felipe Montiel A</searchLink>; Department of Respiratory Medicine (Adult Cystic Fibrosis Unit), Vall d'Hebron University Hospital, Vall d'Hebron Research Institute (VHIR), Barcelona, Spain.<br /><searchLink fieldCode="AU" term="%22Rachel+M%22">Rachel M</searchLink>; Institu University of Rzeszów Medical College, Rzeszów, Poland; Allergy and Cystic Fibrosis Clinic, Provincial Hospital No. 2, Rzeszów, Poland.<br /><searchLink fieldCode="AU" term="%22Stoyanova+GP%22">Stoyanova GP</searchLink>; Cystic Fibrosis Center, Pediatric Clinic, UMHAT 'Alexandrovska', Sofia, Bulgaria; Pediatric Department, Medical University, Sofia, Bulgaria.<br /><searchLink fieldCode="AU" term="%22Van+Braeckel+E%22">Van Braeckel E</searchLink>; Department of Respiratory Medicine, Ghent University Hospital, Ghent, Belgium; Department of Internal Medicine and Paediatrics, Faculty of Medicine and Health Sciences, Ghent University, Ghent, Belgium; European Reference Network on rare respiratory diseases (ERN-LUNG), CF core network, Frankfurt, Germany.<br /><searchLink fieldCode="AU" term="%22Volpi+S%22">Volpi S</searchLink>; Cystic fibrosis center, Azienda Ospedaliera Universitaria Integrata Verona, Verona, Italy.<br /><searchLink fieldCode="AU" term="%22Vukić-Dugac+A%22">Vukić-Dugac A</searchLink>; Cystic Fibrosis Centre for Children and Adults, Clinic for respiratory diseases Jordanovac, University Hospital Centre Zagreb, Croatia; School of Medicine, University of Zagreb, Croatia.<br /><searchLink fieldCode="AU" term="%22Wege+S%22">Wege S</searchLink>; Department of Pulmonology and Respiratory Medicine, Thoraxklinik at the University Hospital of Heidelberg, Germany.<br /><searchLink fieldCode="AU" term="%22Simmonds+NJ%22">Simmonds NJ</searchLink>; Adult Cystic Fibrosis Centre, Royal Brompton Hospital, part of Guy's and St Thomas's NHS Foundation Trust, London, United Kingdom; National Heart and Lung Institute, Imperial College, London, United Kingdom.<br /><searchLink fieldCode="AU" term="%22Plant+BJ%22">Plant BJ</searchLink>; Cork Centre for Cystic Fibrosis (3CF), Cork University Hospital, University College Cork, Wilton, Cork, Ireland; The HRB funded Clinical Research Facility, University College Cork, Cork, Ireland; Department of Medicine, University College Cork, Cork, Ireland.<br /><searchLink fieldCode="AU" term="%22Downey+DG%22">Downey DG</searchLink>; Wellcome-Wolfson Institute for Experimental Medicine, Queen's University Belfast, Belfast, United Kingdom.<br /><searchLink fieldCode="AU" term="%22van+Koningsbruggen-Rietschel+S%22">van Koningsbruggen-Rietschel S</searchLink>; European Reference Network on rare respiratory diseases (ERN-LUNG), CF core network, Frankfurt, Germany; Cystic Fibrosis Centre Cologne, University Hospital, Faculty of Medicine and University of Cologne, Cologne, Germany; Centre for Rare Diseases, Faculty of Medicine and University Hospital Cologne, University of Cologne, Cologne, Germany.<br /><searchLink fieldCode="AU" term="%22Burgel+PR%22">Burgel PR</searchLink>; European Reference Network on rare respiratory diseases (ERN-LUNG), CF core network, Frankfurt, Germany; Respiratory Medicine and French National CF Reference Center, Assistance Publique-Hôpitaux de Paris, Paris, France; Université Paris Cité, Institut Cochin, INSERM U1016, CNRS, Paris, France.<br /><searchLink fieldCode="AU" term="%22Blasi+F%22">Blasi F</searchLink>; Department of Pathophysiology and Transplantation, University of Milan, Milan, Italy; Respiratory Unit and Cystic Fibrosis Adult Center, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.
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