Rapid Improvement after Starting Elexacaftor–Tezacaftor–Ivacaftor in Patients with Cystic Fibrosis and Advanced Pulmonary Disease
- Burgel, Pierre-Régis
- Durieu, Isabelle
- Chiron, Raphaël
- Ramel, Sophie
- Danner-Boucher, Isabelle
- Prevotat, Anne
- Grenet, Dominique
- Marguet, Christophe
- Reynaud-Gaubert, Martine
- Macey, Julie
- Mely, Laurent
- Fanton, Annlyse
- Quetant, Sébastien
- Lemonnier, Lydie
- Paillasseur, Jean-Louis
- da Silva, Jennifer
- Martin, Clémence
- Andrejak, Claire
- Becourt, Arnaud
- Mounard, Julie
- Poulet, Claire
- Rames, Cinthia
- Talleux, Marie
- Chevalier, Marie-Chantal
- Darviot, Estelle
- Jouvenot, Marie
- Marien, Caroline
- Paris, Audrey
- Pelatan, Cécile
- Person, Christine
- Priou, Pascaline
- Troussier, Françoise
- Urban, Thierry
- Dalphin, Marie-Laure
- Dalphin, Jean-Charles
- Ladaurade, Alice
- Pernet, Didier
- Richaud-Thiriez, Bénédicte
- Roux-Claude, Pauline
- Blanc, Nathalia
- Boisserie-Lacroix, Vincent
- Bui, Stephanie
- Collet, Cyrielle
- Debelleix, Stéphane
- Macey, Julie
- Bergot, Emmanuel
- Brouard, Jacques
- Campbell, Karine
- Laurans, Muriel
- Ribault, Virginie
- Borderon, Corinne
- Heraud, Marie-Christine
- Labbe, Guillaume
- Montcouquiol, Sylvie
- Petit, Isabelle
- Ruivard, Marc
- Delestrain, Céline
- Douvry, Benoit
- Epaud, Ralph
- Maitre, Bernard
- Remus, Natascha
- Beltramo, Guillaume
- Fanton, Annlyse
- Houzel, Anne
- Huet, Frédéric
- Perez, Stéphanie
- Boldron-Ghaddar, Amale
- Scalbert, Manuela
- Bouzioukh, Rabah
- Mely, Laurent
- Simon, Charles
- Camara, Boubou
- Hamidfar, Rébecca
- Llerena, Catherine
- Pin, Isabelle
- Quetant, Sébastien
- Deschildre, Antoine
- Gicquello, Alice
- Le Rouzic, Olivier
- Leroy, Clara
- Paris, Nicolas
- Perez, Thierry
- Prevotat, Anne
- Thumerelle, Caroline
- Turck, Dominique
- Wizla, Nathalie
- Dupuy-Grasset, Magali
- Languepin, Jane
- Masson-Rouchaud, Alexandra
- Menetrey, Céline
- Durieu, Isabelle
- Durupt, Stéphane
- L’excellent, Sophie
- Nove-Josserand, Raphaele
- Ohlmann, Camille
- Reix, Phillipe
- Reynaud, Quitterie
- Werck-Gallois, Marie-Christine
- Baravalle, Mélissandre
- Coltey, Bérangère
- Desmazes-Dufeu, Nadine
- Dubus, Jean-Christophe
- Gautier, Clarisse
- Rey, Jean-Baptiste
- Reynaud-Gaubert, Martine
- Stremler, Nathalie
- Caimmi, Davide
- Chiron, Raphaël
- Devrait, Margot
- Moreau, Johan
- Billon, Yves
- Blondé, Aurore
- Guillaumot, Anne
- Kiefer, Sébastien
- Peretti, Laura
- Tatopoulos, Aurélie
- Tiotiu, Angélica
- Benhamida, Myriam
- Bihouee, Tiphaine
- Danner-Boucher, Isabelle
- Eschapasse, Emmanuel
- Tissot, Adrien
- Giannantonio, Marie
- Leroy, Sylvie
- Piccini-Bailly, Carole
- Pradelli, Johana
- Messika, Jonathan
- Boussaud, Véronique
- Burgel, Pierre-Régis
- Carlier, Nicolas
- Honoré, Isabelle
- Hubert, Dominique
- Kanaan, Reem
- Martin, Clémence
- Bailly-Botuha, Céline
- Chedevergne, Frédérique
- de Blic, Jacques
- Delacourt, Christophe
- Drummond, David
- Fauroux, Brigitte
- Karila, Chantal
- Le Bourgeois, Muriel
- Sermet, Isabelle
- Delaisi, Bertrand
- Gerardin, Michèle
- Houdouin, Veronique
- Leclainche, Laurence
- Aubertin, Guillaume
- Berdah, Laura
- Clement, Annick
- Corvol, Harriet
- Nathan, Nadia
- Prevost, Blandine
- Richard, Nicolas
- Tamalet, Aline
- Taytard, Jessica
- Thouvenin, Guillaume
- Tourniaire, Barbara
- Abely, Michel
- Bessaci-Kabouya, Katia
- Dury, Sandra
- Ravoninjatovo, Bruno
- Dabadie, Alain
- Dagorne, Michel
- Deneuville, Eric
- Jamin, Marie
- Ribault, Mélanie
- Vigier, Clémentine
- Belleguic, Chantal
- Brinchault, Graziella
- Desrues, Benoit
- Barzic, Audrey
- Dirou-Prigent, Anne
- Le Bihan, Jean
- Ramel, Sophie
- Revert, Krista
- Ropars, Thomas
- Couderc, Laure
- Dominique, Stéphane
- Marguet, Christophe
- Morisse-Pradier, Hélène
- Pramil, Stéphanie
- Thiberville, Luc
- Allou, Nathalie
- Enaud, Laurent
- Gachelin, Elsa
- Huchot, Eric
- Payet, Annabelle
- Perisson, Caroline
- Piyaraly, Saguiraly
- Valois, Sophie
- Herzog, Audrey
- Kessler, Romain
- Porzio, Michele
- Weiss, Laurence
- Beaumont, Laurence
- Brugiere, Olivier
- Colin, Sylvie
- Verdiere, De
- Cuquemelle, Elise
- de Miranda, Sandra
- Grenet, Dominique
- Monem Hamid, Adbdul
- Parquin, François
- Picard, Clément
- Roux, Antoine
- Roy, Charlotte
- Bremont, François
- Didier, Alain
- Dupuis, Marion
- Faviez, Guillaume
- Labouret, Géraldine
- Mittaine, Marie
- Murris-Espin, Marlène
- Roditis, Léa
- Cosson, Laure
- Diot, Patrice
- Flament, Thomas
- Giraut, Charlotte
- Mankikian, Julie
- Arnouat, Baptiste
- Mousset, Gaétane
- Storni, Véronique
- Vigneron, Philippe
- Coirier-Duet, Emmanuelle
- Gabsi, Asma
DOIAbstract
International audienceRationale: Elexacaftor-tezacaftor-ivacaftor is a CFTR (cystic fibrosis [CF] transmembrane conductance regulator) modulator combination, developed for patients with CF with at least one Phe508del mutation. Objectives: To evaluate the effects of elexacaftor-tezacaftor- ivacaftor in patients with CF and advanced respiratory disease. Methods: A prospective observational study, including all patients aged ⩾12 years and with a percent-predicted FEV1 (ppFEV1) <40 who initiated elexacaftor-tezacaftor-ivacaftor from December 2019 to August 2020 in France was conducted. Clinical characteristics were collected at initiation and at 1 and 3 months. Safety and effectiveness were evaluated by September 2020. National-level transplant...
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