Sweat chloride and lung function responses to elexacaftor–tezacaftor–ivacaftor in people with cystic fibrosis with two versus one responsive CFTR variants: an analysis of two real-world observational studies

Pierre-Régis Burgel, Jennifer Da Silva, Emmanuelle Girodon, Isabelle Durieu, Martine Reynaud-Gaubert, Marlene Murris-Espin, Raphael Chiron, Dominique Grenet, Sophie Ramel, Laurent Mely, Rebecca Hamidfar, Benoit Douvry, Véronique Houdouin, Camille Audousset, Julie Macey, Marie Mittaine, Laurence Weiss, Laure Cosson, Isabelle Danner-Boucher, Philippe Reix, Mélisande Baravalle, Espérie Burnet, Isabelle Sermet-Gaudelus, Clémence Martin, Jean-Louis Paillasseur, Claire ANDREJAK, Claire POULET, Cinthia RAMES, Pascaline PRIOU, Françoise TROUSSIER, Bénédicte RICHAUD-THIRIEZ, Marie-Laure DALPHIN, Alice LADAURADE, Julie MACEY, Claire BON, Stephanie BUI, Mickael FAYON, Karine CAMPBELL, Muriel LAURANS, Sylvie MONTCOUQUIOL, Guillaume LABBE, Benoit DOUVRY, Ralph EPAUD, Natascha REMUS, Annlyse FANTON, Frédéric HUET, Amale BOLDRON-GHADDAR, Manuela SCALBERT, Célia TILLARD, Laurent MELY, Rébecca HAMIDFAR, Sébastien QUETANT, Catherine LLERENA, Sophie VALOIS, Camille AUDOUSSET, Thierry PEREZ, Nathalie WIZLA-DERAMBURE, Caroline THUMERELLE, Magali DUPUY-GRASSET, Jeanne LANGUEPIN, Alexandra MASSON-ROUCHAUD, Isabelle DURIEU, Raphaële NOVE-JOSSERAND, Quitterie REYNAUD, Maël RICHARD, Philippe REIX, Camille OHLMANN, Thomas PERRIN, Tom TOIN, Marie-Christine WERCK-GALLOIS, Martine REYNAUD-GAUBERT, Bérengère COLTEY, Nadine DESMAZES-DUFEU, Jean-Christophe DUBUS, Mélisande BARAVALLE, Nathalie STREMLER, Raphaël CHIRON, Floriane SOCCHI, Anne GUILLAUMOT, Aurélie TATOPOULOS, Sébastien KIEFER, Isabelle DANNER-BOUCHER, Adrien TISSOT, Tiphaine ROUSSEY-BIHOUEE, Myriam BENHAMIDA, Sylvie LEROY, Wael ALKOUSSA, Sarah MARCHAL, Pierre-Régis BURGEL, Nicolas CARLIER, Johanna FESENBECKH, Isabelle HONORÉ, Reem KANAAN, Clémence MARTIN, Isabelle SERMET-GAUDELUS, Frédérique CHEDEVERGNE, Véronique HOUDOUIN, Laurence LE CLAINCHE, Sophie MAYER, Harriet CORVOL, Sophie DENAMUR, Jessica TAYTARD, Guillaume THOUVENIN, Katia BESSACI-KABOUYA, Sandra DURY, Chantal BELLEGUIC, Eric DENEUVILLE, Marie JAMIN, Sophie RAMEL, Jean LE BIHAN, Christophe MARGUET, Stéphane DOMINIQUE, Hélène MORISSE-PRADIER, Stéphanie PRAMIL, Laure COUDERC, Elsa GACHELIN, Juliette VERHILLE, Caroline PERISSON, Romain KESSLER, Loïc KASSEGNE, Michele PORZIO, Laurence WEISS, Dominique GRENET, Sylvie COLIN-DE-VERDIERE, Sandra DE MIRANDA, Marlène MURRIS-ESPIN, Marion DUPUIS, Marie MITTAINE, Léa RODITIS, Julie MANKIKIAN, Thomas FLAMENT, Laure COSSON, Charlotte GIRAUT, Baptiste ARNOUAT, Asma GABSI, Delphine POURADIER, Anne-Sophie BONNEL

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DOI: 10.1016/s2213-2600(25)00190-0
Published: 2025-11
Container: The Lancet Respiratory Medicine
Publisher: Elsevier BV
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BibTeX

@article{allodium:10.1016/s2213-2600-25-00190-0,
  title = {Sweat chloride and lung function responses to elexacaftor–tezacaftor–ivacaftor in people with cystic fibrosis with two versus one responsive CFTR variants: an analysis of two real-world observational studies},
  author = {Pierre-Régis Burgel and Jennifer Da Silva and Emmanuelle Girodon and Isabelle Durieu and Martine Reynaud-Gaubert and Marlene Murris-Espin and Raphael Chiron and Dominique Grenet and Sophie Ramel and Laurent Mely and Rebecca Hamidfar and Benoit Douvry and Véronique Houdouin and Camille Audousset and Julie Macey and Marie Mittaine and Laurence Weiss and Laure Cosson and Isabelle Danner-Boucher and Philippe Reix and Mélisande Baravalle and Espérie Burnet and Isabelle Sermet-Gaudelus and Clémence Martin and Jean-Louis Paillasseur and Claire ANDREJAK and Claire POULET and Cinthia RAMES and Pascaline PRIOU and Françoise TROUSSIER and Bénédicte RICHAUD-THIRIEZ and Marie-Laure DALPHIN and Alice LADAURADE and Julie MACEY and Claire BON and Stephanie BUI and Mickael FAYON and Karine CAMPBELL and Muriel LAURANS and Sylvie MONTCOUQUIOL and Guillaume LABBE and Benoit DOUVRY and Ralph EPAUD and Natascha REMUS and Annlyse FANTON and Frédéric HUET and Amale BOLDRON-GHADDAR and Manuela SCALBERT and Célia TILLARD and Laurent MELY and Rébecca HAMIDFAR and Sébastien QUETANT and Catherine LLERENA and Sophie VALOIS and Camille AUDOUSSET and Thierry PEREZ and Nathalie WIZLA-DERAMBURE and Caroline THUMERELLE and Magali DUPUY-GRASSET and Jeanne LANGUEPIN and Alexandra MASSON-ROUCHAUD and Isabelle DURIEU and Raphaële NOVE-JOSSERAND and Quitterie REYNAUD and Maël RICHARD and Philippe REIX and Camille OHLMANN and Thomas PERRIN and Tom TOIN and Marie-Christine WERCK-GALLOIS and Martine REYNAUD-GAUBERT and Bérengère COLTEY and Nadine DESMAZES-DUFEU and Jean-Christophe DUBUS and Mélisande BARAVALLE and Nathalie STREMLER and Raphaël CHIRON and Floriane SOCCHI and Anne GUILLAUMOT and Aurélie TATOPOULOS and Sébastien KIEFER and Isabelle DANNER-BOUCHER and Adrien TISSOT and Tiphaine ROUSSEY-BIHOUEE and Myriam BENHAMIDA and Sylvie LEROY and Wael ALKOUSSA and Sarah MARCHAL and Pierre-Régis BURGEL and Nicolas CARLIER and Johanna FESENBECKH and Isabelle HONORÉ and Reem KANAAN and Clémence MARTIN and Isabelle SERMET-GAUDELUS and Frédérique CHEDEVERGNE and Véronique HOUDOUIN and Laurence LE CLAINCHE and Sophie MAYER and Harriet CORVOL and Sophie DENAMUR and Jessica TAYTARD and Guillaume THOUVENIN and Katia BESSACI-KABOUYA and Sandra DURY and Chantal BELLEGUIC and Eric DENEUVILLE and Marie JAMIN and Sophie RAMEL and Jean LE BIHAN and Christophe MARGUET and Stéphane DOMINIQUE and Hélène MORISSE-PRADIER and Stéphanie PRAMIL and Laure COUDERC and Elsa GACHELIN and Juliette VERHILLE and Caroline PERISSON and Romain KESSLER and Loïc KASSEGNE and Michele PORZIO and Laurence WEISS and Dominique GRENET and Sylvie COLIN-DE-VERDIERE and Sandra DE MIRANDA and Marlène MURRIS-ESPIN and Marion DUPUIS and Marie MITTAINE and Léa RODITIS and Julie MANKIKIAN and Thomas FLAMENT and Laure COSSON and Charlotte GIRAUT and Baptiste ARNOUAT and Asma GABSI and Delphine POURADIER and Anne-Sophie BONNEL},
  year = {2025},
  journal = {The Lancet Respiratory Medicine},
  doi = {10.1016/s2213-2600(25)00190-0},
  url = {https://doi.org/10.1016/s2213-2600(25)00190-0}
}

RIS

TY  - JOUR
TI  - Sweat chloride and lung function responses to elexacaftor–tezacaftor–ivacaftor in people with cystic fibrosis with two versus one responsive CFTR variants: an analysis of two real-world observational studies
AU  - Pierre-Régis Burgel
AU  - Jennifer Da Silva
AU  - Emmanuelle Girodon
AU  - Isabelle Durieu
AU  - Martine Reynaud-Gaubert
AU  - Marlene Murris-Espin
AU  - Raphael Chiron
AU  - Dominique Grenet
AU  - Sophie Ramel
AU  - Laurent Mely
AU  - Rebecca Hamidfar
AU  - Benoit Douvry
AU  - Véronique Houdouin
AU  - Camille Audousset
AU  - Julie Macey
AU  - Marie Mittaine
AU  - Laurence Weiss
AU  - Laure Cosson
AU  - Isabelle Danner-Boucher
AU  - Philippe Reix
AU  - Mélisande Baravalle
AU  - Espérie Burnet
AU  - Isabelle Sermet-Gaudelus
AU  - Clémence Martin
AU  - Jean-Louis Paillasseur
AU  - Claire ANDREJAK
AU  - Claire POULET
AU  - Cinthia RAMES
AU  - Pascaline PRIOU
AU  - Françoise TROUSSIER
AU  - Bénédicte RICHAUD-THIRIEZ
AU  - Marie-Laure DALPHIN
AU  - Alice LADAURADE
AU  - Julie MACEY
AU  - Claire BON
AU  - Stephanie BUI
AU  - Mickael FAYON
AU  - Karine CAMPBELL
AU  - Muriel LAURANS
AU  - Sylvie MONTCOUQUIOL
AU  - Guillaume LABBE
AU  - Benoit DOUVRY
AU  - Ralph EPAUD
AU  - Natascha REMUS
AU  - Annlyse FANTON
AU  - Frédéric HUET
AU  - Amale BOLDRON-GHADDAR
AU  - Manuela SCALBERT
AU  - Célia TILLARD
AU  - Laurent MELY
AU  - Rébecca HAMIDFAR
AU  - Sébastien QUETANT
AU  - Catherine LLERENA
AU  - Sophie VALOIS
AU  - Camille AUDOUSSET
AU  - Thierry PEREZ
AU  - Nathalie WIZLA-DERAMBURE
AU  - Caroline THUMERELLE
AU  - Magali DUPUY-GRASSET
AU  - Jeanne LANGUEPIN
AU  - Alexandra MASSON-ROUCHAUD
AU  - Isabelle DURIEU
AU  - Raphaële NOVE-JOSSERAND
AU  - Quitterie REYNAUD
AU  - Maël RICHARD
AU  - Philippe REIX
AU  - Camille OHLMANN
AU  - Thomas PERRIN
AU  - Tom TOIN
AU  - Marie-Christine WERCK-GALLOIS
AU  - Martine REYNAUD-GAUBERT
AU  - Bérengère COLTEY
AU  - Nadine DESMAZES-DUFEU
AU  - Jean-Christophe DUBUS
AU  - Mélisande BARAVALLE
AU  - Nathalie STREMLER
AU  - Raphaël CHIRON
AU  - Floriane SOCCHI
AU  - Anne GUILLAUMOT
AU  - Aurélie TATOPOULOS
AU  - Sébastien KIEFER
AU  - Isabelle DANNER-BOUCHER
AU  - Adrien TISSOT
AU  - Tiphaine ROUSSEY-BIHOUEE
AU  - Myriam BENHAMIDA
AU  - Sylvie LEROY
AU  - Wael ALKOUSSA
AU  - Sarah MARCHAL
AU  - Pierre-Régis BURGEL
AU  - Nicolas CARLIER
AU  - Johanna FESENBECKH
AU  - Isabelle HONORÉ
AU  - Reem KANAAN
AU  - Clémence MARTIN
AU  - Isabelle SERMET-GAUDELUS
AU  - Frédérique CHEDEVERGNE
AU  - Véronique HOUDOUIN
AU  - Laurence LE CLAINCHE
AU  - Sophie MAYER
AU  - Harriet CORVOL
AU  - Sophie DENAMUR
AU  - Jessica TAYTARD
AU  - Guillaume THOUVENIN
AU  - Katia BESSACI-KABOUYA
AU  - Sandra DURY
AU  - Chantal BELLEGUIC
AU  - Eric DENEUVILLE
AU  - Marie JAMIN
AU  - Sophie RAMEL
AU  - Jean LE BIHAN
AU  - Christophe MARGUET
AU  - Stéphane DOMINIQUE
AU  - Hélène MORISSE-PRADIER
AU  - Stéphanie PRAMIL
AU  - Laure COUDERC
AU  - Elsa GACHELIN
AU  - Juliette VERHILLE
AU  - Caroline PERISSON
AU  - Romain KESSLER
AU  - Loïc KASSEGNE
AU  - Michele PORZIO
AU  - Laurence WEISS
AU  - Dominique GRENET
AU  - Sylvie COLIN-DE-VERDIERE
AU  - Sandra DE MIRANDA
AU  - Marlène MURRIS-ESPIN
AU  - Marion DUPUIS
AU  - Marie MITTAINE
AU  - Léa RODITIS
AU  - Julie MANKIKIAN
AU  - Thomas FLAMENT
AU  - Laure COSSON
AU  - Charlotte GIRAUT
AU  - Baptiste ARNOUAT
AU  - Asma GABSI
AU  - Delphine POURADIER
AU  - Anne-Sophie BONNEL
PY  - 2025
JO  - The Lancet Respiratory Medicine
DO  - 10.1016/s2213-2600(25)00190-0
UR  - https://doi.org/10.1016/s2213-2600(25)00190-0
ER  -

APA

Burgel, P., Silva, J. D., Girodon, E., Durieu, I., Reynaud-Gaubert, M., Murris-Espin, M., Chiron, R., Grenet, D., Ramel, S., Mely, L., Hamidfar, R., Douvry, B., Houdouin, V., Audousset, C., Macey, J., Mittaine, M., Weiss, L., Cosson, L., Danner-Boucher, I., Reix, P., Baravalle, M., Burnet, E., Sermet-Gaudelus, I., Martin, C., Paillasseur, J., ANDREJAK, C., POULET, C., RAMES, C., PRIOU, P., TROUSSIER, F., RICHAUD-THIRIEZ, B., DALPHIN, M., LADAURADE, A., MACEY, J., BON, C., BUI, S., FAYON, M., CAMPBELL, K., LAURANS, M., MONTCOUQUIOL, S., LABBE, G., DOUVRY, B., EPAUD, R., REMUS, N., FANTON, A., HUET, F., BOLDRON-GHADDAR, A., SCALBERT, M., TILLARD, C., MELY, L., HAMIDFAR, R., QUETANT, S., LLERENA, C., VALOIS, S., AUDOUSSET, C., PEREZ, T., WIZLA-DERAMBURE, N., THUMERELLE, C., DUPUY-GRASSET, M., LANGUEPIN, J., MASSON-ROUCHAUD, A., DURIEU, I., NOVE-JOSSERAND, R., REYNAUD, Q., RICHARD, M., REIX, P., OHLMANN, C., PERRIN, T., TOIN, T., WERCK-GALLOIS, M., REYNAUD-GAUBERT, M., COLTEY, B., DESMAZES-DUFEU, N., DUBUS, J., BARAVALLE, M., STREMLER, N., CHIRON, R., SOCCHI, F., GUILLAUMOT, A., TATOPOULOS, A., KIEFER, S., DANNER-BOUCHER, I., TISSOT, A., ROUSSEY-BIHOUEE, T., BENHAMIDA, M., LEROY, S., ALKOUSSA, W., MARCHAL, S., BURGEL, P., CARLIER, N., FESENBECKH, J., HONORÉ, I., KANAAN, R., MARTIN, C., SERMET-GAUDELUS, I., CHEDEVERGNE, F., HOUDOUIN, V., CLAINCHE, L. L., MAYER, S., CORVOL, H., DENAMUR, S., TAYTARD, J., THOUVENIN, G., BESSACI-KABOUYA, K., DURY, S., BELLEGUIC, C., DENEUVILLE, E., JAMIN, M., RAMEL, S., BIHAN, J. L., MARGUET, C., DOMINIQUE, S., MORISSE-PRADIER, H., PRAMIL, S., COUDERC, L., GACHELIN, E., VERHILLE, J., PERISSON, C., KESSLER, R., KASSEGNE, L., PORZIO, M., WEISS, L., GRENET, D., COLIN-DE-VERDIERE, S., MIRANDA, S. D., MURRIS-ESPIN, M., DUPUIS, M., MITTAINE, M., RODITIS, L., MANKIKIAN, J., FLAMENT, T., COSSON, L., GIRAUT, C., ARNOUAT, B., GABSI, A., POURADIER, D., & BONNEL, A. (2025). Sweat chloride and lung function responses to elexacaftor–tezacaftor–ivacaftor in people with cystic fibrosis with two versus one responsive CFTR variants: an analysis of two real-world observational studies. The Lancet Respiratory Medicine. https://doi.org/10.1016/s2213-2600(25)00190-0

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