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1. The expanded French compassionate programme for elexacaftor–tezacaftor–ivacaftor use in people with cystic fibrosis without a F508del CFTR variant: a real-world study

Author

Kanaan, Reem, Carlier, Nicolas, Honoré, Isabelle, Chedevergne, Frédérique, Dreano, Elise, Hatton, Aurélie, Hinzpeter, Alexandre, Pranke, Iwona, Le Clainche-Viala, Laurence, Mayer, Sophie, Corvol, Harriet, Thouvenin, Guillaume, de Miranda, Sandra, Remus, Natascha, Douvry, Benoit, Duthoit, Louise, Perez, Thierry, Le Rouzic, Olivier, Wizla, Nathalie, Bon, Claire, Bui, Stéphanie, Poey, Nora, Stremler, Nathalie, Coltey, Bérengère, Dufeu, Nadine, Lebihan, Jean, Gabsi, Asma, Pouradier, Delphine, Andrejak, Claire, Rames, Cinthia, Dupuy-Grasset, Magali, Languepin, Jeanne, Marguet, Christophe, Pramil, Stéphanie, Arnouat, Baptiste, Fanton, Annlyse, Abely, Michel, Ravoninjatovo, Bruno, Blondé, Aurore, Guillaumot, Anne, Kieffer, Sebastien, Tatopoulos, Aurélie, Nove-Josserand, Raphaële, Ohlmann, Camille, Perrin, Thomas, Reynaud, Quitterie, Llerena, Catherine, Quétant, Sébastien, Valois, Sophie, Dalphin, Marie-Laure, Richaud-Thiriez, Bénédicte, Deneuville, Eric, Chiron, Raphael, Socchi, Floriane, Bihouée, Tiphaine, Mankikian, Julie, Flament, Thomas, Coolen-Allou, Nathalie, Gachelin, Elsa, Périsson, Caroline, Vuillard, Constance, Dupuis, Marion, Alkoussa, Wael, Marchal, Sarah, Leroy, Sylvie, Scalbert, Manuela, Campbell, Karine, Laurans, Muriel, Labbé, Guillaume, Montcouquiol, Sylvie, Priou, Pascaline, de Carli, Paola, Lemonnier, Lydie, Dehillotte, Clémence, Nouvel, Thierry, Burgel, Pierre-Régis, Sermet-Gaudelus, Isabelle, Girodon, Emmanuelle, Durieu, Isabelle, Houdouin, Véronique, Audousset, Camille, Macey, Julie, Grenet, Dominique, Porzio, Michele, Murris-Espin, Marlène, Reix, Philippe, Baravalle, Mélisande, Belleguic, Chantal, Mely, Laurent, Verhille, Juliette, Weiss, Laurence, Reynaud-Gaubert, Martine, Mittaine, Marie, Hamidfar, Rebecca, Ramel, Sophie, Cosson, Laure, Danner-Boucher, Isabelle, Foucaud, Pierre, Roy, Charlotte, Burnet, Espérie, Raynal, Caroline, Audrezet, Marie-Pierre, Da Silva, Jennifer, and Martin, Clémence

Published
2024
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2. Impact of COVID-19 infection on lung function and nutritional status amongst individuals with cystic fibrosis: A global cohort study

Author

Kasmi, Irena, Drali, Ouardia, Burghart, Sabine, Lakatos-Krepcik, Andrea, Eder, Johannes, Jaksch, Peter, Kainz, Katharina, Kallinger, Margit, Leitner, Alexander, Mozdzen, Marta, Pfleger, Andreas, Renner, Sabine, Stadlinger, Martin, Thir, Christina, Nuriyev, Emil, Boboli, Hedwige, De Wachter, Elke, Dupont, Lieven, Gohy, Sophie, Hanssens, Laurence, Knoop, Christiane, Lammertyn, Elise, Nowé, Vicky, Pirson, Jessica, Thimmesch, Matthieu, Van Braeckel, Eva, Van Hoorenbeeck, Kim, Vanderhelst, Eef, Filho, Eduardo Piacentini, Athanazio, Rodrigo Abensur, Martins, Valéria de Carvalho, Duarte, Marta Cristina, Monte, Luciana de Freitas Velloso, de Fuccio, Marcelo Bicalho, Knabben, Adriana de Siqueira Carvalho, Melloti, Roberta, Meneses, Daniela Gois, Petrova, Guergana, Tješić-Drinković, Duška, Dugac, Andrea Vukić, Bambir, Ivan, Yiallouros, Panayiotis, Bilkova, Alena, Drevinek, Pavel, Macek, Milan, Jr, Olesen, Hanne Vebert, Pressler, Tania, Fouda, Eman Mahmoud, Nasr, Samya, Weldetsadik, Abate Yeshidinber, Al-iede, Montaha, Abdrakhmanov, Olzhas, Corvol, Harriet, Lemonnier-Videau, Lydie, Abely, Michel, Piccini, Carole Bailly, Belleguic, Chantal, Bihouee, Tiphaine, Billon, Yves, Bui, Stéphanie, Camara, Boubou, Cheraud, Marie-Christine, Chiron, Raphael, Duet, Emmanuelle Coirier, Cosson, Laure, Dalphin, Marie-Laure, Boucher, Isabelle Danner, De Miranda, Sandra, Deneuville, Eric, Dubus, Jean-Christophe, Durieu, Isabelle, Epaud, Ralph, Gerardin, Michèle, Grenet, Dominique, Houdouin, Véronique, Huet, Frédéric, Reem, Kanaan, Kessler, Romain, Languepin, Jeanne, Laurans, Muriel, Leroy, Sylvie, Llerena, Cathie, Macey, Julie, Mankikian, Julie, Marguet, Christophe, Martin, Clémence, Mely, Laurent, Mittaine, Marie, Murris-Espin, Marlène, Perisson, Caroline, Prevotat, Anne, Ramel, Sophie, Rames, Cinthia, Reix, Philippe, Revillon, Marine, Reynaud-Gaubert, Martine, Richaud-Thiriez, Bénédicte, Rittie, Jean-Luc, Scalbert-Dujardin, Manuëla, Sermet-Gaudelus, Isabelle, Storni, Véronique, Tatopoulos, Aurélie, Thouvenin, Guillaume, Troussier, Françoise, Weiss, Laurence, Wizla, Nathalie, Behl, Eva-Susanne, Brinkmann, Folke, Claßen, Martin, Graepler-Mainka, Ute, Griese, Matthias, Grübl, Armin, Hammermann, Jutta, Hebestreit, Helge, Heinzmann, Andrea, Herz, Alexander, Kiefer, Alexander, Kinder, Birte, Köster, Holger, Kuhnert, Stefan, Mainz, Jochen, Mayer, Angelika, Naehrig, Susanne, Niehues, Tim, Nüßlein, Thomas, Poplawska, Krystyna, Ringshausen, Felix, Rose, Markus, Rosenecker, Josef, Ruppel, Renate, Scharschinger, Anette, Schropp, Christian, Schwarz, Carsten, Smaczny, Christina, Sommerburg, Olaf, Sutharsan, Sivagurunathan, Stolz, Simone, Thomas, Wolfgang, Wege, Sabine, Welzenbach, Britta, Wollschläger, Bettina, Diamantea, Filia, Hatziagorou, Elpis, Manika, Katerina, Cox, Des, Elnazir, Basil, Fletcher, Godfrey, Gunaratnam, Cedric, McKone, Edward F., Plant, Barry J., Cohen-Cymberknoh, Malena, Gur, Michal, Livnat, Galit, Mei-Zahav, Meir, Amato, Annalisa, Ferrari, Gianluca, Badolato, Raffaele, Poli, Piercarlo, Battistini, Fiorella, Donati, Valentina, Bignamini, Elisabetta, Folino, Anna, Carnovale, Vincenzo, Castellani, Carlo, Casciaro, Rosaria, Cimino, Giuseppe, Cipolli, Marco, Lucca, Francesca, Collura, Mirella, Ficili, Francesca, Daccò, Valeria, Gagliano, Vanessa, Pizzamiglio, Giovanna, Mencarini, Valeria, Palladino, Nicola, Leonardi, Salvatore, Rotolo, Novella, Lucanto, Maria Cristina, Quattromano, Ester, Lucidi, Vincenzina, Majo, Fabio, Alghisi, Federico, Ciciriello, Fabiana, Manca, Antonio, Leonetti, Giuseppina, Maschio, Massimo, Messore, Barbara, Pantano, Stefano, Pisi, Giovanna, Spaggiari, Cinzia, Raia, Valeria, Laezza, Caterina, Ros, Mirco, Salvatore, Donatello, Taccetti, Giovanni, Francalanci, Michela, Vitullo, Pamela, Zolin, Anna, Aleksejeva, Elina, Malakauskas, Kestutis, Misevičiene, Valdone, Charatsi, Anna-Maria, la Barrière, Hélène De, Altenburg, Josje, Bannier, Michiel, Heijerman, Harry, Janssens, Hettie, Koppelman, Gerard, van der Meer, Renske, Merkus, Peter, Nuijsink, Marianne, Terheggen, Suzanne, van der Vaart, Hester, Wesseling, Geert-Jan, de Winter, Karin, Danevska, Ivana Arnaudova, Maretti, Tatjana Jakovska, Fustik, Stojka, Dziecichowicz-Latała, Daria, Wojsyk-Banaszak, Irena, Wozniacki, Lukasz, Amorim, Adelina, Santos, Ana Sofia Araújo, Castanhinha, Susana, Gamboa, Fernanda, Silva, Teresa Reis, Gonçalves, Fabienne, Pereira, Luísa, Ciuca, Ioana, Silva, Sónia, Csilla-Enikö, Szabo, Stan, Iustina, Amelina, Elena, Boitсova, Evgeniya, Chernyavskaya, Anastasia, Gorinova, Yuliya, Krasovskiy, Stanislav, Mukhina, Maria, Sherman, Victoria, Simonova, Olga, Kondratyeva, Elena, Bérešová, Eva, Bližnáková, Nina, Kayserová, Hana, Salobir, Barbara, Šelb, Julij, Krivec, Uroš, Fernandez, Antonio José Aguilar, Fernàndez, Antonio Alvarez, García, Félix Baranda, Aparicio, Marina Blanco, Corullón, Silvia Castillo, Cortell-Aznar, Isidoro, Pérez, Inés, Colomer, Jordi Costa i, Roig, María Cols, Pecellín, Isabel Delgado, Cáceres, Layla Diab, Paredes, Carmen Luna, Gartner, Silvia, Martínez, José Ramón Gutiérrez, Labarga, Inés Herrero, Girón-Moreno, Rosa Maria, Nogueira, Esperanza Jiménez, Ferreiro, Adelaida Lamas, Neyra, Alejandro López, Castro, Enrique Blitz, Galarraga, Laura Moreno, de Vincente, Carlos Martin, Navarro, Silvia Merlos, Nieto-Royo, Rosa, Fuster, Casilda Olveira, Pastor, Maria Dolores, Pérez-Ruiz, Estela, Prados-Sánchez, Concepción, Cancelo, Isabel Ramos, de Valbuena, Marta Ruiz, Asensi, José R. Villa, Santiago, Veronica Sanz, García, Patricia Fernández, Tawfeeq, Reem Mustafa, Banki, Adrienn, Gilljam, Marita, Krantz, Christina, Lindberg, Ulrika, Lindblad, Anders, Clarenbach, Christian, Steinack, Carolin, Hage, René, Schuurmans, Macé, Fischer, Reta, Kusche, Rachel, Rochat, Isabelle, Walter, Anna-Lena, Kamalaporn, Harutai, Hamouda, Samia, Tural, Dilber Ademhan, Ozcelik, Ugur, Asfuroğlu, Pelin, Eyüboğlu, Tuğba Şişmanlar, Aslan, Ayse Tana, Bingöl, Ayşen, Çobanoğlu, Nazan, Ozcan, Gizem, Dogru, Deniz, Gökdemir, Yasemin, KÖSE, Mehmet, Pekcan, Sevgi, Cosgriff, Rebecca, Semenchuk, Julie, Naito, Yumi, Charman, Susan C., Carr, Siobhán B, Cheng, Stephanie Y., Marshall, Bruce C., Faro, Albert, Elbert, Alexander, Gutierrez, Hector H., Goss, Christopher H., Karadag, Bulent, Burgel, Pierre-Régis, Colombo, Carla, Salvatore, Marco, Padoan, Rita, Daneau, Géraldine, Harutyunyan, Satenik, Kashirskaya, Nataliya, Kirwan, Laura, Middleton, Peter G, Ruseckaite, Rasa, de Monestrol, Isabelle, Naehrlich, Lutz, Mondejar-Lopez, Pedro, Jung, Andreas, van Rens, Jacqui, Bakkeheim, Egil, Orenti, Annalisa, Zomer-van Ommen, Domenique, da Silva-Filho, Luiz Vicente RF, Fernandes, Flavia Fonseca, Zampoli, Marco, and Stephenson, Anne L.

Published
2024
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3. Sexual dysfunction in cystic fibrosis

Author

Ramel, Sophie, Gueganton, Laetitia, Nowak, Emmanuel, Bihan, Jean Le, Arnouat, Baptiste, Belleguic, Chantal, Danner-Boucher, Isabelle, Mankikian, Julie, Payet, Annabelle, Urban, Thierry, Buyse, Marion, and Hubeaux, Katelyne

Published
2024
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4. CT Imaging Assessment of Response to Treatment in Allergic Bronchopulmonary Aspergillosis in Adults With Bronchial Asthma

Author

Mal, Hervé, Le Guen, Pierre, Dupin, Clairelyne, Meurice, Jean Claude, Verdaguer, Marion, de Keizer, Joe, Delétage-Métreau, Céline, Le Mao, Raphael, Tromer, Cécile, Fajole, Gaëlle, Rayez, Mélanie, Raymond, Christel Saint, Gheerbrant, Hubert, Badatcheff, Anne, Person, Christine, Macey, Julie, Dermant, Xavier, Boitiaux, Jean-François, Gosset-Woimant, Marine, Metz-Favre, Carine, Degot, Tristan, Poulet, Claire, Popin, Elisabeth, Gamez, Anne Sophie, Boissin, Clément, Prevotat, Anne, Mangiapan, Gilles, Brouquières, Danielle, Le Floch, Hervé, Morisse-Pradier, Hélène, Sattler, Caroline, Muti, Daniela, Germaud, Patrick, Dirou, Stéphanie, Paris, Audrey, Melloni, Boris, Ballouhey, Julia, Bondeelle, Louise, Laurent, Lucie, Belleguic, Chantal, Kerjouan, Mallorie, Deslée, Gaëtan, Dury, Sandra, Bergot, Emmanuel, Magnier, Romain, Morel, Hugues, Lemaire, Bertrand, Tumino, Cécile, Sénéchal, Agathe, Choinier, Pascaline, Parrot, Antoine, Godet, Cendrine, Brun, Anne-Laure, Couturaud, Francis, Laurent, François, Frat, Jean-Pierre, Marchand-Adam, Sylvain, Gagnadoux, Frédéric, Blanchard, Elodie, Taillé, Camille, Philippe, Bruno, Hirschi, Sandrine, Andréjak, Claire, Bourdin, Arnaud, Chenivesse, Cécile, Dominique, Stéphane, Murris-Espin, Marlène, Rivière, Frédéric, Garcia, Gilles, Blanc, François-Xavier, Goupil, François, Bergeron, Anne, Flament, Thomas, Priou, Pascaline, Ragot, Stéphanie, and Cadranel, Jacques

Published
2024
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7. The expanded French compassionate programme for elexacaftor–tezacaftor–ivacaftor use in people with cystic fibrosis without a F508del CFTR variant: a real-world study.

Author

Burgel, Pierre-Régis, Sermet-Gaudelus, Isabelle, Girodon, Emmanuelle, Durieu, Isabelle, Houdouin, Véronique, Audousset, Camille, Macey, Julie, Grenet, Dominique, Porzio, Michele, Murris-Espin, Marlène, Reix, Philippe, Baravalle, Mélisande, Belleguic, Chantal, Mely, Laurent, Verhille, Juliette, Weiss, Laurence, Reynaud-Gaubert, Martine, Mittaine, Marie, Hamidfar, Rebecca, and Ramel, Sophie

Subjects
CYSTIC fibrosis, CYSTIC fibrosis transmembrane conductance regulator, LUNGS, CHLORIDES, PERCENTILES
Abstract

Elexacaftor–tezacaftor–ivacaftor has been approved in Europe for people with cystic fibrosis with at least one F508del CFTR variant. Additionally, it is approved by the US Food and Drug Administration (FDA) for people with cystic fibrosis with at least one of 177 rare variants. The aims of this study were to describe the clinical response to elexacaftor–tezacaftor–ivacaftor for people with cystic fibrosis without a F508del CFTR variant in France and to determine CFTR variant responsiveness to elexacaftor–tezacaftor–ivacaftor based on the observed clinical response. The French compassionate programme expanded access to elexacaftor–tezacaftor–ivacaftor to people with cystic fibrosis, aged 6 years and older, without a F508del variant, excluding those with two variants previously characterised as non-responsive. Participants at France's 47 cystic fibrosis centres were given a 4–6 week trial of elexacaftor–tezacaftor–ivacaftor and response was determined by a centralised committee based on evolution of clinical data, lung function, and sweat chloride concentration. Responsiveness of individual CFTR variants was derived from observed clinical responses. The first compassionnate programme was launched on May 19, 2022; by March 8, 2024, 516 people with cystic fibrosis had been identified for inclusion in this real-word study: 37 were not included due to the presence of two variants previously characterised as non-responsive to elexacaftor–tezacaftor–ivacaftor, and 479 (229 females [48%] and 250 males [52%]) received elexacaftor–tezacaftor–ivacaftor for 4–6 weeks. Among 443 participants who received no CFTR modulator before elexacaftor–tezacaftor–ivacaftor, 83 had at least one FDA-approved variant, of whom 81 (98%) were responders and continued elexacaftor–tezacaftor–ivacaftor; in responders, mean absolute change in sweat chloride was –44·5 mmol/L (95% CI –39·1 to –49·8) and percentage of predicted FEV 1 (ppFEV 1) was 11·1 percentage points (95% CI 8·4 to 13·7; both comparisons p<0·0001). Among 360 participants with no FDA-approved variant and no previous CFTR modulator, 177 (49%) were responders; in responders, mean absolute change in sweat chloride was –20·5 mmol/L (–17·2 to –23·8) and ppFEV 1 was 13·2 percentage points (11·4 to 15·0; both comparisons p<0·0001). Among 36 participants who were receiving ivacaftor before elexacaftor–tezacaftor–ivacaftor, 32 (89%) continued elexacaftor–tezacaftor–ivacaftor. Of 251 individual CFTR variants, 64 (28 FDA-approved) were classified as responsive or possibly responsive to elexacaftor–tezacaftor–ivacaftor, and 123 (two FDA-approved) as non-responsive or possibly non-responsive to elexacaftor–tezacaftor–ivacaftor. In France, over half of the population with cystic fibrosis without a F508del variant responded to elexacaftor–tezacaftor–ivacaftor, with most responders having no FDA-approved variant. The treatment period was relatively short and further research is warranted to describe the long-term safety and effectiveness of elexacaftor–tezacaftor–ivacaftor in this population. Association Vaincre la Mucoviscidose, Société Française de la Mucoviscidose, and Filière Maladies Rares MUCO-CFTR. [ABSTRACT FROM AUTHOR]

Published
2024
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8. Long-term safety and efficacy of tezacaftor–ivacaftor in individuals with cystic fibrosis aged 12 years or older who are homozygous or heterozygous for Phe508del CFTR (EXTEND): an open-label extension study

Author

Gibson, Ronald L., Jr., Rowe, Steven M., Lechtzin, Noah, Ahrens, Richard C., McCoy, Karen S., Aitken, Moira, Donaldson, Scott H., McBennett, Kimberly Ann, Pilewski, Joseph M., Billings, Joanne, Milla, Carlos, Rubenstein, Ronald, Rosenbluth, Daniel Brian, Linnemann, Rachel, Powers, Michael R., Fortner, Christopher, Frederick, Carla Anne, Liou, Theodore G., Black, Philip, Wang, Janice, Colombo, John L., Berdella, Maria, Indihar, Maria Veronica, Brown, Cynthia D., Anstead, Michael, Bilodeau, Lara, Sicilian, Leonard, Jain, Manu, Tolle, James Jerome, Moffett, Kathryn, Nasr, Samya, Taylor-Cousar, Jennifer, Barto, Tara Lynn, Antos, Nicholas, Rogers, John S., Quick, Bryon, Thompson, Henry R., Sawicki, Gregory, Barnett, Bruce, Zanni, Robert L., Smith, Thomas C., Schultz, Karen D., Keating, Claire, Flume, Patrick, Omlor, Gregory J., Ashare, Alix, Voter, Karen, Mehdi, Nighat, Ortiz, Maria Gabriela Tupayachi, Gardner, Tonia E., Boas, Steven R., Messore, Barbara, Zemanick, Edith, Jain, Raksha, McCarthy, Michael, Kissner, Dana G., Patel, Kapilkumar, McNamara, John, Philley, Julie, Berlinski, Ariel, Calimano, Francisco J., Chin, Terry, Conrad, Douglas, Daines, Cori, Raissy, Hengameh H., Keens, Thomas G., Lascano, Jorge E., McWilliams, Bennie, Morrissey, Brian, Reyes, Santiago, Chittivelu, Subramanyam, Hussain, Sabiha, Stone, Arvey, Wallace, James, Klingsberg, Ross, Biller, Julie A., Bui, Stephanie, Sommerburg, Olaf, Bignamini, Elisabetta, Collura, Mirella, Biner, Reta Fischer, Moller, Alexander, Salvatore, Donatello, Belleguic, Chantal, Bentur, Lea, Efrati, Ori, Kerem, Eitan, Prais, Dario, Gallego, Esther Quintana, Barry, Peter, Livnat-Levanon, Galit, Asensi, Jose Ramon Villa, Armstrong, David Stuart, de la Cruz, Oscar Asensio, Gilchrist, Francis, Tullis, Diana Elizabeth, Quon, Bradley, Lands, Larry C., Morrison, Nancy, Lavoie, Annick, Linnane, Barry, Elidemir, Okan, Ringshausen, Felix, Kappler, Matthias, Hebestreit, Helge, Mainz, Jochen, Kiefer, Alexander, Koerner-Rettberg, Cordula, Staab, Doris, Gleiber, Wolfgang, Pressler, Tacjana, Stehling, Florian, Hector, Andreas, Sutharsan, Sivagurunathan, Naehrlich, Lutz, Fischer, Rainald, Downey, Damian, Davies, Jane Carolyn, Ketchell, Robert Ian, Carroll, Mary Patricia, Doe, Simon, MacGregor, Gordon, Nash, Edward Fairbairn, Withers, Nicholas, Peckham, Daniel Gavin, Ledson, Martin James, Kansra, Sonal, Lee, Timothy William Rayner, Delaisi, Bertrand, Rault, Gilles, Le Bihan, Jean, Hubert, Dominique, Fajac, Isabelle, Sermet-Gaudelus, Isabelle, Bakker, Marleen, Arets, Bert, De Boeck, Christiane, Chiron, Raphael, Reix, Philippe, Mainguy, Catherine, van Braeckel, Eva, Malfroot, Anne, Durieu, Isabelle, Dufeu, Nadine Desmazes, Prevotat, Anne, van der Meer, Renske, Merkus, Petrus, Weersink, E.J.M., Gomez-Aguero, Isabel Barrio, Gartner, Silvia, Jover, Amparo Sole, Fernandez, Antonio Alvarez, Cox, Desmond William, McKone, Edward F., Plant, Barry James, Selvadurai, Hiranjan, Bowler, Simon David, Wainwright, Claire Elizabeth, Smith, Daniel, Middleton, Peter Gordon, Wilson, John William, Volpi, Sonia, Colombo, Carla, Fabrizzi, Benedetta, Lucidi, Vincenzina, Cresta, Federico, Cucchiara, Salvatore, Eber, Ernst, Ellemunter, Helmut, Huttegger, Isidor, Hjelte, Lena, Krantz, Christina, Gilljam, Marita, Flume, Patrick A, Downey, Damian G, Brown, Cynthia, De Boeck, Kris, Sawicki, Gregory S, Chang, Philip, Paz-Diaz, Hildegarde, Rubin, Jaime L, Yang, Yoojung, Hu, Xingdi, Pasta, David J, Millar, Stefanie J, Campbell, Daniel, Wang, Xin, Ahluwalia, Neil, Owen, Caroline A, and Wainwright, Claire E

Published
2021
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9. The expanded French compassionate programme for elexacaftor–tezacaftor–ivacaftor use in people with cystic fibrosis without a F508del CFTRvariant: a real-world study

Author

Burgel, Pierre-Régis, Sermet-Gaudelus, Isabelle, Girodon, Emmanuelle, Durieu, Isabelle, Houdouin, Véronique, Audousset, Camille, Macey, Julie, Grenet, Dominique, Porzio, Michele, Murris-Espin, Marlène, Reix, Philippe, Baravalle, Mélisande, Belleguic, Chantal, Mely, Laurent, Verhille, Juliette, Weiss, Laurence, Reynaud-Gaubert, Martine, Mittaine, Marie, Hamidfar, Rebecca, Ramel, Sophie, Cosson, Laure, Douvry, Benoit, Danner-Boucher, Isabelle, Foucaud, Pierre, Roy, Charlotte, Burnet, Espérie, Raynal, Caroline, Audrezet, Marie-Pierre, Da Silva, Jennifer, Martin, Clémence, Kanaan, Reem, Carlier, Nicolas, Honoré, Isabelle, Chedevergne, Frédérique, Dreano, Elise, Hatton, Aurélie, Hinzpeter, Alexandre, Pranke, Iwona, Le Clainche-Viala, Laurence, Mayer, Sophie, Corvol, Harriet, Thouvenin, Guillaume, de Miranda, Sandra, Remus, Natascha, Douvry, Benoit, Duthoit, Louise, Perez, Thierry, Le Rouzic, Olivier, Wizla, Nathalie, Bon, Claire, Bui, Stéphanie, Poey, Nora, Stremler, Nathalie, Coltey, Bérengère, Dufeu, Nadine, Lebihan, Jean, Gabsi, Asma, Pouradier, Delphine, Andrejak, Claire, Rames, Cinthia, Dupuy-Grasset, Magali, Languepin, Jeanne, Marguet, Christophe, Pramil, Stéphanie, Arnouat, Baptiste, Fanton, Annlyse, Abely, Michel, Ravoninjatovo, Bruno, Blondé, Aurore, Guillaumot, Anne, Kieffer, Sebastien, Tatopoulos, Aurélie, Nove-Josserand, Raphaële, Ohlmann, Camille, Perrin, Thomas, Reynaud, Quitterie, Llerena, Catherine, Quétant, Sébastien, Valois, Sophie, Dalphin, Marie-Laure, Richaud-Thiriez, Bénédicte, Deneuville, Eric, Chiron, Raphael, Socchi, Floriane, Bihouée, Tiphaine, Mankikian, Julie, Flament, Thomas, Coolen-Allou, Nathalie, Gachelin, Elsa, Périsson, Caroline, Vuillard, Constance, Dupuis, Marion, Alkoussa, Wael, Marchal, Sarah, Leroy, Sylvie, Scalbert, Manuela, Campbell, Karine, Laurans, Muriel, Labbé, Guillaume, Montcouquiol, Sylvie, Priou, Pascaline, de Carli, Paola, Lemonnier, Lydie, Dehillotte, Clémence, and Nouvel, Thierry

Abstract

Elexacaftor–tezacaftor–ivacaftor has been approved in Europe for people with cystic fibrosis with at least one F508del CFTRvariant. Additionally, it is approved by the US Food and Drug Administration (FDA) for people with cystic fibrosis with at least one of 177 rare variants. The aims of this study were to describe the clinical response to elexacaftor–tezacaftor–ivacaftor for people with cystic fibrosis without a F508del CFTRvariant in France and to determine CFTRvariant responsiveness to elexacaftor–tezacaftor–ivacaftor based on the observed clinical response.

Published
2024
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10. CT Imaging Assessment of Response to Treatment in Allergic Bronchopulmonary Aspergillosis in Adults With Bronchial Asthma

Author

Godet, Cendrine, primary, Brun, Anne-Laure, additional, Couturaud, Francis, additional, Laurent, François, additional, Frat, Jean-Pierre, additional, Marchand-Adam, Sylvain, additional, Gagnadoux, Frédéric, additional, Blanchard, Elodie, additional, Taillé, Camille, additional, Philippe, Bruno, additional, Hirschi, Sandrine, additional, Andréjak, Claire, additional, Bourdin, Arnaud, additional, Chenivesse, Cécile, additional, Dominique, Stéphane, additional, Mangiapan, Gilles, additional, Murris-Espin, Marlène, additional, Rivière, Frédéric, additional, Garcia, Gilles, additional, Blanc, François-Xavier, additional, Goupil, François, additional, Bergeron, Anne, additional, Flament, Thomas, additional, Priou, Pascaline, additional, Mal, Hervé, additional, de Keizer, Joe, additional, Ragot, Stéphanie, additional, Cadranel, Jacques, additional, Le Guen, Pierre, additional, Dupin, Clairelyne, additional, Meurice, Jean Claude, additional, Verdaguer, Marion, additional, Delétage-Métreau, Céline, additional, Le Mao, Raphael, additional, Tromer, Cécile, additional, Fajole, Gaëlle, additional, Rayez, Mélanie, additional, Raymond, Christel Saint, additional, Gheerbrant, Hubert, additional, Badatcheff, Anne, additional, Person, Christine, additional, Macey, Julie, additional, Dermant, Xavier, additional, Boitiaux, Jean-François, additional, Gosset-Woimant, Marine, additional, Metz-Favre, Carine, additional, Degot, Tristan, additional, Poulet, Claire, additional, Popin, Elisabeth, additional, Gamez, Anne Sophie, additional, Boissin, Clément, additional, Prevotat, Anne, additional, Brouquières, Danielle, additional, Le Floch, Hervé, additional, Morisse-Pradier, Hélène, additional, Sattler, Caroline, additional, Muti, Daniela, additional, Germaud, Patrick, additional, Dirou, Stéphanie, additional, Paris, Audrey, additional, Melloni, Boris, additional, Ballouhey, Julia, additional, Bondeelle, Louise, additional, Laurent, Lucie, additional, Belleguic, Chantal, additional, Kerjouan, Mallorie, additional, Deslée, Gaëtan, additional, Dury, Sandra, additional, Bergot, Emmanuel, additional, Magnier, Romain, additional, Morel, Hugues, additional, Lemaire, Bertrand, additional, Tumino, Cécile, additional, Sénéchal, Agathe, additional, Choinier, Pascaline, additional, and Parrot, Antoine, additional

Published
2024
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11. Prevalence and severity of functional urinary and anorectal disorders and their impact on quality of life in cystic fibrosis

Author

Hubeaux, Katelyne, primary, Gueganton, Laetitia, additional, Nowak, Emmanuel, additional, Arnouat, Baptiste, additional, Belleguic, Chantal, additional, Danner-Boucher, Isabelle, additional, Mankikian, Julie, additional, Payet, Annabelle, additional, Urban, Thierry, additional, Buyse, Marion, additional, and Ramel, Sophie, additional

Published
2023
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12. Pneumocystis jirovecii and Cystic Fibrosis in Brittany, France

Author

Nevez, Gilles, Robert-Gangneux, Florence, Pougnet, Laurence, Virmaux, Michèle, Belleguic, Chantal, Deneuville, Eric, Rault, Gilles, Chevrier, Sylviane, Ramel, Sophie, Le Bihan, Jean, Guillaud-Saumur, Thibaud, Calderon, Enrique, Le Govic, Yohann, Gangneux, Jean-Pierre, and Le Gal, Solène

Published
2017
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13. Prevalence and risk factors of asthma in dairy farmers: Ancillary analysis of AIRBAg

Author

Jouneau, Stéphane, primary, Chapron, Anthony, additional, Ropars, Cécile, additional, Marette, Solenne, additional, Robert, Ange-Marie, additional, Gouyet, Thomas, additional, Belleguic, Chantal, additional, Rochefort-Morel, Cécile, additional, Guillot, Stéphanie, additional, Mailloux, Carole, additional, Desrues, Benoît, additional, Viel, Jean-François, additional, Barakat, Jean, additional, Barel, Anne-Laure, additional, Baron, Henri-Pierre, additional, Bassen, Rachel, additional, Behaghel-Baruteau, Florence, additional, Bernier, Cyril, additional, Beyer, Séverine, additional, Boissel, Romain, additional, Brault, Vanessa, additional, Brinchault, Graziella, additional, Bureau, Dominique, additional, Castel, Odile, additional, Cazuguel, Anne-France, additional, Decelle-Trochet, Catherine, additional, Delaunay, Marcel, additional, Delaunay-Lelièvre, Isabelle, additional, Eveno, Bruno, additional, Fekete, Krisztina, additional, Fourmont, Lisa, additional, Guervin, Amandine, additional, Guy, Tiphaine, additional, Héry, Guillaume, additional, Hardouin, Pauline, additional, Hespel, Anne, additional, Jeffray, Nathalie, additional, Jouneau, Stéphane, additional, Koskas, Yves, additional, de Lauzun, Virginie, additional, Lebossé, Antoine, additional, Lecot, Sandrine, additional, Marquette, David, additional, Martin, Virginie, additional, Merzoug, Ahmed, additional, Morel, Hugues, additional, Morer, Jean, additional, Morisseau, Patrick, additional, Oksenhendler, Aurélie, additional, Orhant, Elisabeth, additional, Peron, Dominique, additional, Piguel-Royer, Mauricette, additional, Pinault, Marie, additional, Pousset, Jean-Paul, additional, Presle, Jean-Claude, additional, Raillard, Patrick, additional, Regnault, Bernard, additional, Séguineau, Antoine, additional, Sevin, Anne, additional, Thépault, Fanny, additional, and Verger, Christian, additional

Published
2022
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16. Using chest CT scan and unsupervised machine learning for predicting and evaluating response to lumacaftor-ivacaftor in people with cystic fibrosis

Author

Campredon, Alienor, primary, Battistella, Enzo, additional, Martin, Clémence, additional, Durieu, Isabelle, additional, Mely, Laurent, additional, Marguet, Christophe, additional, Belleguic, Chantal, additional, Murris-Espin, Marlène, additional, Chiron, Raphaël, additional, Fanton, Annlyse, additional, Bui, Stéphanie, additional, Reynaud-Gaubert, Martine, additional, Reix, Philippe, additional, Hoang-Thi, Trieu-Nghi, additional, Vakalopoulou, Maria, additional, Revel, Marie-Pierre, additional, Da Silva, Jennifer, additional, Burgel, Pierre-Régis, additional, and Chassagnon, Guillaume, additional

Published
2021
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18. Long-term safety and efficacy of tezacaftor–ivacaftor in individuals with cystic fibrosis aged 12 years or older who are homozygous or heterozygous for Phe508del CFTR (EXTEND): an open-label extension study

Author

Flume, Patrick A, primary, Biner, Reta Fischer, additional, Downey, Damian G, additional, Brown, Cynthia, additional, Jain, Manu, additional, Fischer, Rainald, additional, De Boeck, Kris, additional, Sawicki, Gregory S, additional, Chang, Philip, additional, Paz-Diaz, Hildegarde, additional, Rubin, Jaime L, additional, Yang, Yoojung, additional, Hu, Xingdi, additional, Pasta, David J, additional, Millar, Stefanie J, additional, Campbell, Daniel, additional, Wang, Xin, additional, Ahluwalia, Neil, additional, Owen, Caroline A, additional, Wainwright, Claire E, additional, Gibson, Ronald L., additional, Rowe, Steven M., additional, Lechtzin, Noah, additional, Ahrens, Richard C., additional, McCoy, Karen S., additional, Aitken, Moira, additional, Donaldson, Scott H., additional, McBennett, Kimberly Ann, additional, Pilewski, Joseph M., additional, Billings, Joanne, additional, Milla, Carlos, additional, Rubenstein, Ronald, additional, Rosenbluth, Daniel Brian, additional, Linnemann, Rachel, additional, Powers, Michael R., additional, Fortner, Christopher, additional, Frederick, Carla Anne, additional, Liou, Theodore G., additional, Black, Philip, additional, Wang, Janice, additional, Colombo, John L., additional, Berdella, Maria, additional, Indihar, Maria Veronica, additional, Brown, Cynthia D., additional, Anstead, Michael, additional, Bilodeau, Lara, additional, Sicilian, Leonard, additional, Tolle, James Jerome, additional, Moffett, Kathryn, additional, Nasr, Samya, additional, Taylor-Cousar, Jennifer, additional, Barto, Tara Lynn, additional, Antos, Nicholas, additional, Rogers, John S., additional, Quick, Bryon, additional, Thompson, Henry R., additional, Sawicki, Gregory, additional, Barnett, Bruce, additional, Zanni, Robert L., additional, Smith, Thomas C., additional, Schultz, Karen D., additional, Keating, Claire, additional, Flume, Patrick, additional, Omlor, Gregory J., additional, Ashare, Alix, additional, Voter, Karen, additional, Mehdi, Nighat, additional, Ortiz, Maria Gabriela Tupayachi, additional, Gardner, Tonia E., additional, Boas, Steven R., additional, Messore, Barbara, additional, Zemanick, Edith, additional, Jain, Raksha, additional, McCarthy, Michael, additional, Kissner, Dana G., additional, Patel, Kapilkumar, additional, McNamara, John, additional, Philley, Julie, additional, Berlinski, Ariel, additional, Calimano, Francisco J., additional, Chin, Terry, additional, Conrad, Douglas, additional, Daines, Cori, additional, Raissy, Hengameh H., additional, Keens, Thomas G., additional, Lascano, Jorge E., additional, McWilliams, Bennie, additional, Morrissey, Brian, additional, Reyes, Santiago, additional, Chittivelu, Subramanyam, additional, Hussain, Sabiha, additional, Stone, Arvey, additional, Wallace, James, additional, Klingsberg, Ross, additional, Biller, Julie A., additional, Bui, Stephanie, additional, Sommerburg, Olaf, additional, Bignamini, Elisabetta, additional, Collura, Mirella, additional, Moller, Alexander, additional, Salvatore, Donatello, additional, Belleguic, Chantal, additional, Bentur, Lea, additional, Efrati, Ori, additional, Kerem, Eitan, additional, Prais, Dario, additional, Gallego, Esther Quintana, additional, Barry, Peter, additional, Livnat-Levanon, Galit, additional, Asensi, Jose Ramon Villa, additional, Armstrong, David Stuart, additional, de la Cruz, Oscar Asensio, additional, Gilchrist, Francis, additional, Tullis, Diana Elizabeth, additional, Quon, Bradley, additional, Lands, Larry C., additional, Morrison, Nancy, additional, Lavoie, Annick, additional, Linnane, Barry, additional, Elidemir, Okan, additional, Ringshausen, Felix, additional, Kappler, Matthias, additional, Hebestreit, Helge, additional, Mainz, Jochen, additional, Kiefer, Alexander, additional, Koerner-Rettberg, Cordula, additional, Staab, Doris, additional, Gleiber, Wolfgang, additional, Pressler, Tacjana, additional, Stehling, Florian, additional, Hector, Andreas, additional, Sutharsan, Sivagurunathan, additional, Naehrlich, Lutz, additional, Downey, Damian, additional, Davies, Jane Carolyn, additional, Ketchell, Robert Ian, additional, Carroll, Mary Patricia, additional, Doe, Simon, additional, MacGregor, Gordon, additional, Nash, Edward Fairbairn, additional, Withers, Nicholas, additional, Peckham, Daniel Gavin, additional, Ledson, Martin James, additional, Kansra, Sonal, additional, Lee, Timothy William Rayner, additional, Delaisi, Bertrand, additional, Rault, Gilles, additional, Le Bihan, Jean, additional, Hubert, Dominique, additional, Fajac, Isabelle, additional, Sermet-Gaudelus, Isabelle, additional, Bakker, Marleen, additional, Arets, Bert, additional, De Boeck, Christiane, additional, Chiron, Raphael, additional, Reix, Philippe, additional, Mainguy, Catherine, additional, van Braeckel, Eva, additional, Malfroot, Anne, additional, Durieu, Isabelle, additional, Dufeu, Nadine Desmazes, additional, Prevotat, Anne, additional, van der Meer, Renske, additional, Merkus, Petrus, additional, Weersink, E.J.M., additional, Gomez-Aguero, Isabel Barrio, additional, Gartner, Silvia, additional, Jover, Amparo Sole, additional, Fernandez, Antonio Alvarez, additional, Cox, Desmond William, additional, McKone, Edward F., additional, Plant, Barry James, additional, Selvadurai, Hiranjan, additional, Bowler, Simon David, additional, Wainwright, Claire Elizabeth, additional, Smith, Daniel, additional, Middleton, Peter Gordon, additional, Wilson, John William, additional, Volpi, Sonia, additional, Colombo, Carla, additional, Fabrizzi, Benedetta, additional, Lucidi, Vincenzina, additional, Cresta, Federico, additional, Cucchiara, Salvatore, additional, Eber, Ernst, additional, Ellemunter, Helmut, additional, Huttegger, Isidor, additional, Hjelte, Lena, additional, Krantz, Christina, additional, and Gilljam, Marita, additional

Published
2021
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19. Rapid Improvement after Starting Elexacaftor–Tezacaftor–Ivacaftor in Patients with Cystic Fibrosis and Advanced Pulmonary Disease

Author

Burgel, Pierre-Régis, primary, Durieu, Isabelle, additional, Chiron, Raphaël, additional, Ramel, Sophie, additional, Danner-Boucher, Isabelle, additional, Prevotat, Anne, additional, Grenet, Dominique, additional, Marguet, Christophe, additional, Reynaud-Gaubert, Martine, additional, Macey, Julie, additional, Mely, Laurent, additional, Fanton, Annlyse, additional, Quetant, Sébastien, additional, Lemonnier, Lydie, additional, Paillasseur, Jean-Louis, additional, Da Silva, Jennifer, additional, Martin, Clémence, additional, Andrejak, Claire, additional, Becourt, Arnaud, additional, Mounard, Julie, additional, Poulet, Claire, additional, Rames, Cinthia, additional, Talleux, Marie, additional, Chevalier, Marie-Chantal, additional, Darviot, Estelle, additional, Jouvenot, Marie, additional, Marien, Caroline, additional, Paris, Audrey, additional, Pelatan, Cécile, additional, Person, Christine, additional, Priou, Pascaline, additional, Troussier, Françoise, additional, Urban, Thierry, additional, Dalphin, Marie-Laure, additional, Dalphin, Jean-Charles, additional, Ladaurade, Alice, additional, Pernet, Didier, additional, Richaud-Thiriez, Bénédicte, additional, Roux-Claude, Pauline, additional, Blanc, Nathalia, additional, Boisserie-Lacroix, Vincent, additional, Bui, Stephanie, additional, Collet, Cyrielle, additional, Debelleix, Stéphane, additional, Bergot, Emmanuel, additional, Brouard, Jacques, additional, Campbell, Karine, additional, Laurans, Muriel, additional, Ribault, Virginie, additional, Borderon, Corinne, additional, Heraud, Marie-Christine, additional, Labbe, Guillaume, additional, Montcouquiol, Sylvie, additional, Petit, Isabelle, additional, Ruivard, Marc, additional, Delestrain, Céline, additional, Douvry, Benoit, additional, Epaud, Ralph, additional, Maitre, Bernard, additional, Remus, Natascha, additional, Beltramo, Guillaume, additional, Houzel, Anne, additional, Huet, Frédéric, additional, Perez, Stéphanie, additional, Boldron-Ghaddar, Amale, additional, Scalbert, Manuela, additional, Bouzioukh, Rabah, additional, Simon, Charles, additional, Camara, Boubou, additional, Hamidfar, Rébecca, additional, Llerena, Catherine, additional, Pin, Isabelle, additional, Deschildre, Antoine, additional, Gicquello, Alice, additional, Le Rouzic, Olivier, additional, Leroy, Clara, additional, Paris, Nicolas, additional, Perez, Thierry, additional, Thumerelle, Caroline, additional, Turck, Dominique, additional, Wizla, Nathalie, additional, Dupuy-Grasset, Magali, additional, Languepin, Jane, additional, Masson-Rouchaud, Alexandra, additional, Menetrey, Céline, additional, Durupt, Stéphane, additional, L’Excellent, Sophie, additional, Nove-Josserand, Raphaele, additional, Ohlmann, Camille, additional, Reix, Phillipe, additional, Reynaud, Quitterie, additional, Werck-Gallois, Marie-Christine, additional, Baravalle, Mélissandre, additional, Coltey, Bérangère, additional, Desmazes-Dufeu, Nadine, additional, Dubus, Jean-Christophe, additional, Gautier, Clarisse, additional, Rey, Jean-Baptiste, additional, Stremler, Nathalie, additional, Caimmi, Davide, additional, Devrait, Margot, additional, Moreau, Johan, additional, Billon, Yves, additional, Blondé, Aurore, additional, Guillaumot, Anne, additional, Kiefer, Sébastien, additional, Peretti, Laura, additional, Tatopoulos, Aurélie, additional, Tiotiu, Angélica, additional, Benhamida, Myriam, additional, Bihouee, Tiphaine, additional, Eschapasse, Emmanuel, additional, Tissot, Adrien, additional, Giannantonio, Marie, additional, Leroy, Sylvie, additional, Piccini-Bailly, Carole, additional, Pradelli, Johana, additional, Messika, Jonathan, additional, Boussaud, Véronique, additional, Burgel, Pierre-Régis, additional, Carlier, Nicolas, additional, Honoré, Isabelle, additional, Hubert, Dominique, additional, Kanaan, Reem, additional, Bailly-Botuha, Céline, additional, Chedevergne, Frédérique, additional, De Blic, Jacques, additional, Delacourt, Christophe, additional, Drummond, David, additional, Fauroux, Brigitte, additional, Karila, Chantal, additional, Le Bourgeois, Muriel, additional, Sermet, Isabelle, additional, Delaisi, Bertrand, additional, Gerardin, Michèle, additional, Houdouin, Veronique, additional, Leclainche, Laurence, additional, Aubertin, Guillaume, additional, Berdah, Laura, additional, Clement, Annick, additional, Corvol, Harriet, additional, Nathan, Nadia, additional, Prevost, Blandine, additional, Richard, Nicolas, additional, Tamalet, Aline, additional, Taytard, Jessica, additional, Thouvenin, Guillaume, additional, Tourniaire, Barbara, additional, Abely, Michel, additional, Bessaci-Kabouya, Katia, additional, Dury, Sandra, additional, Ravoninjatovo, Bruno, additional, Dabadie, Alain, additional, Dagorne, Michel, additional, Deneuville, Eric, additional, Jamin, Marie, additional, Ribault, Mélanie, additional, Vigier, Clémentine, additional, Belleguic, Chantal, additional, Brinchault, Graziella, additional, Desrues, Benoit, additional, Barzic, Audrey, additional, Dirou-Prigent, Anne, additional, Le Bihan, Jean, additional, Revert, Krista, additional, Ropars, Thomas, additional, Couderc, Laure, additional, Dominique, Stéphane, additional, Morisse-Pradier, Hélène, additional, Pramil, Stéphanie, additional, Thiberville, Luc, additional, Allou, Nathalie, additional, Enaud, Laurent, additional, Gachelin, Elsa, additional, Huchot, Eric, additional, Payet, Annabelle, additional, Perisson, Caroline, additional, Piyaraly, Saguiraly, additional, Valois, Sophie, additional, Herzog, Audrey, additional, Kessler, Romain, additional, Porzio, Michele, additional, Weiss, Laurence, additional, Beaumont, Laurence, additional, Brugiere, Olivier, additional, Colin, Sylvie, additional, Verdiere, de, additional, Cuquemelle, Elise, additional, De Miranda, Sandra, additional, Monem Hamid, Adbdul, additional, Parquin, François, additional, Picard, Clément, additional, Roux, Antoine, additional, Roy, Charlotte, additional, Bremont, François, additional, Didier, Alain, additional, Dupuis, Marion, additional, Faviez, Guillaume, additional, Labouret, Géraldine, additional, Mittaine, Marie, additional, Murris-Espin, Marlène, additional, Roditis, Léa, additional, Cosson, Laure, additional, Diot, Patrice, additional, Flament, Thomas, additional, Giraut, Charlotte, additional, Mankikian, Julie, additional, Arnouat, Baptiste, additional, Mousset, Gaétane, additional, Storni, Véronique, additional, Vigneron, Philippe, additional, Coirier-Duet, Emmanuelle, additional, and Gabsi, Asma, additional

Published
2021
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20. Real-Life Safety and Effectiveness of Lumacaftor–Ivacaftor in Patients with Cystic Fibrosis

Author

Burgel, Pierre-Régis, primary, Munck, Anne, additional, Durieu, Isabelle, additional, Chiron, Raphaël, additional, Mely, Laurent, additional, Prevotat, Anne, additional, Murris-Espin, Marlene, additional, Porzio, Michele, additional, Abely, Michel, additional, Reix, Philippe, additional, Marguet, Christophe, additional, Macey, Julie, additional, Sermet-Gaudelus, Isabelle, additional, Corvol, Harriet, additional, Bui, Stéphanie, additional, Lemonnier, Lydie, additional, Dehillotte, Clémence, additional, Da Silva, Jennifer, additional, Paillasseur, Jean-Louis, additional, Hubert, Dominique, additional, Mounard, Julie, additional, Poulet, Claire, additional, Rames, Cinthia, additional, Person, Christine, additional, Troussier, Françoise, additional, Urban, Thierry, additional, Dalphin, Marie-Laure, additional, Dalphin, Jean-Claude, additional, Pernet, Didier, additional, Richaud-Thiriez, Bénédicte, additional, Fayon, Mickael, additional, Macey-Caro, Julie, additional, Campbell, Karine, additional, Laurans, Muriel, additional, Borderon, Corinne, additional, Heraud, Marie-Christine, additional, Labbé, André, additional, Montcouquiol, Sylvie, additional, Bassinet, Laurence, additional, Remus, Natascha, additional, Fanton, Annlyse, additional, Houzel-Charavel, Anne, additional, Huet, Frédéric, additional, Perez-Martin, Stéphanie, additional, Boldron-Ghaddar, Amale, additional, Scalbert, Manuela, additional, Camara, Boubou, additional, Llerena, Catherine, additional, Pin, Isabelle, additional, Quétant, Sébastien, additional, Cottereau, Aurélie, additional, Deschildre, Antoine, additional, Gicquello, Alice, additional, Perez, Thierry, additional, Stervinou-Wemeau, Lidwine, additional, Thumerelle, Caroline, additional, Wallaert, Benoit, additional, Wizla, Nathalie, additional, Languepin, Jane, additional, Ménétrey, Céline, additional, Dupuy-Grasset, Magalie, additional, Bazus, Lucie, additional, Buchs, Clelia, additional, Jubin, Virginie, additional, Werck-Gallois, Marie-Christine, additional, Mainguy, Catherine, additional, Perrin, Thomas, additional, Toutain-Rigolet, Agnès, additional, Durupt, Stéphane, additional, Reynaud, Quitterie, additional, Nove-Josserand, Raphaele, additional, Baravalle-Einaudi, Melisande, additional, Coltey, Bérangère, additional, Dufeu, Nadine, additional, Dubus, Jean-Christophe, additional, Stremler, Nathalie, additional, Caimmi, Davide, additional, Billon, Yves, additional, Derelle, Jocelyne, additional, Kieffer, Sébastien, additional, Pichon, Anne-Sophie, additional, Schweitzer, Cyril, additional, Tatopoulos, Aurélie, additional, Abbes, Sarah, additional, Bihouée, Tiphaine, additional, Danner-Boucher, Isabelle, additional, David, Valérie, additional, Haloun, Alain, additional, Tissot, Adrien, additional, Leroy, Sylvie, additional, Bailly-Piccini, Carole, additional, Clément, Annick, additional, Tamalet, Aline, additional, Burgel, Pierre-Régis, additional, Honoré, Isabelle, additional, Kanaan, Reem, additional, Martin, Clémence, additional, Bailly, Cécile, additional, Chédevergne, Frédérique, additional, De Blic, Jacques, additional, Fauroux, Brigitte, additional, Le Bourgeois, Murielle, additional, Delaisi, Bertrand, additional, Gérardin, Michèle, additional, Abély, Michel, additional, Ravoninjatovo, Bruno, additional, Belleguic, Chantal, additional, Desrues, Benoit, additional, Brinchault, Graziella, additional, Dagorne, Michel, additional, Deneuville, Eric, additional, Lefeuvre, Sylvaine, additional, Dirou, Anne, additional, Le Bihan, Jean, additional, Ramel, Sophie, additional, Dominique, Stéphane, additional, Payet, Annabelle, additional, Kessler, Romain, additional, Rosner, Vincent, additional, Weiss, Laurence, additional, de Miranda, Sandra, additional, Grenet, Dominique, additional, Hamid, Abdoul, additional, Picard, Clément, additional, Brémont, François, additional, Didier, Alain, additional, Labouret, Géraldine, additional, Mittaine, Marie, additional, Murris-Espin, Marlène, additional, Têtu, Laurent, additional, Cosson, Laure, additional, Giraut, Charlotte, additional, Henriet, Anne-Cécile, additional, Mankikian, Julie, additional, Marchand, Sophie, additional, Hugé, Sandrine, additional, Storni, Véronique, additional, and Coirier-Duet, Emmanuelle, additional

Published
2020
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21. Phagocytosis depends on TRPV2-mediated calcium influx and requires TRPV2 in lipids rafts: alteration in macrophages from patients with cystic fibrosis

Author

Lévêque, Manuella, Penna, Aubin, Le Trionnaire, Sophie, Belleguic, Chantal, Desrues, Benoît, Brinchault, Graziella, Jouneau, Stéphane, Lagadic-Gossmann, Dominique, Martin-Chouly, Corinne, Institut de recherche en santé, environnement et travail (Irset), Université d'Angers (UA)-Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-École des Hautes Études en Santé Publique [EHESP] (EHESP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), CHU Pontchaillou [Rennes], Chemistry, Oncogenesis, Stress and Signaling (COSS), Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-CRLCC Eugène Marquis (CRLCC)-Institut National de la Santé et de la Recherche Médicale (INSERM), Université d'Angers (UA)-Université de Rennes (UR)-École des Hautes Études en Santé Publique [EHESP] (EHESP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), Université de Rennes (UR)-CRLCC Eugène Marquis (CRLCC)-Institut National de la Santé et de la Recherche Médicale (INSERM), and Jonchère, Laurent

Subjects
Adult, Male, Adolescent, Cystic Fibrosis, Macrophages, lcsh:R, TRPV Cation Channels, lcsh:Medicine, Middle Aged, Article, Membrane Microdomains, Phagocytosis, [SDV.SPEE] Life Sciences [q-bio]/Santé publique et épidémiologie, Humans, Calcium, Female, lcsh:Q, [SDV.SPEE]Life Sciences [q-bio]/Santé publique et épidémiologie, lcsh:Science, Cells, Cultured
Abstract

International audience; Whereas many phagocytosis steps involve ionic fluxes, the underlying ion channels remain poorly defined. As reported in mice, the calcium conducting TRPV2 channel impacts the phagocytic process. Macrophage phagocytosis is critical for defense against pathogens. In cystic fibrosis (CF), macrophages have lost their capacity to act as suppressor cells and thus play a significant role in the initiating stages leading to chronic inflammation/infection. In a previous study, we demonstrated that impaired function of CF macrophages is due to a deficient phagocytosis. The aim of the present study was to investigate TRPV2 role in the phagocytosis capacity of healthy primary human macrophage by studying its activity, its membrane localization and its recruitment in lipid rafts. In primary human macrophages, we showed that P. aeruginosa recruits TRPV2 channels at the cell surface and induced a calcium influx required for bacterial phagocytosis. We presently demonstrate that to be functional and play a role in phagocytosis, TRPV2 might require a preferential localization in lipid rafts. Furthermore, CF macrophage displays a perturbed calcium homeostasis due to a defect in TRPV2. In this context, deregulated TRPV2-signaling in CF macrophages could explain their defective phagocytosis capacity that contribute to the maintenance of chronic infection.

Published
2018

24. Soluble CD14 acts as a DAMP in human macrophages: origin and involvement in inflammatory cytokine/chemokine production

Author

Lévêque, Manuella, primary, Jeune, Karin Simonin-Le, additional, Jouneau, Stéphane, additional, Moulis, Solenn, additional, Desrues, Benoit, additional, Belleguic, Chantal, additional, Brinchault, Graziella, additional, Le Trionnaire, Sophie, additional, Gangneux, Jean-Pierre, additional, Dimanche-Boitrel, Marie-Thérèse, additional, and Martin-Chouly, Corinne, additional

Published
2017
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27. Pneumocystis jirovecii and Cystic Fibrosis in Brittany, France.

Author

Nevez, Gilles, Robert-Gangneux, Florence, Pougnet, Laurence, Virmaux, Michèle, Belleguic, Chantal, Deneuville, Eric, Rault, Gilles, Chevrier, Sylviane, Ramel, Sophie, Le Bihan, Jean, Guillaud-Saumur, Thibaud, Calderon, Enrique, Le Govic, Yohann, Gangneux, Jean-Pierre, and Le Gal, Solène

Abstract

Pneumocystis jirovecii is a transmissible fungus with a high pulmonary tropism. The prevalence of P. jirovecii in patients with cystic fibrosis (CF) has been estimated in Germany at 7.4%, in Spain at 21.5% and in Brazil at 38.2%. Data on the prevalence of P. jirovecii in CF patients in France remain scarce, particularly in Brittany, where the prevalence of CF is high (from 1/1600 to 1/4500). Our objectives were to determine the prevalence of colonization of the airways by P. jirovecii in Brittany in CF patients monitored at the ' Centre de Ressources et de Compétences de la Mucoviscidose (CRCM)' of Rennes compared to that previously observed at the CRCM of Roscoff-Brest. Sputa from 86 patients (178 specimens) followed in Rennes were analyzed retrospectively. The detection of P. jirovecii was performed using real-time PCR targeting the gene encoding the mitochondrial large subunit of ribosomal RNA. Pneumocystis jirovecii DNA was detected in 3/86 patients (3.5%) monitored at Rennes, whereas it had previously been detected in 1/76 patients (1.3%) monitored at Roscoff-Brest, thus showing an overall prevalence of 2.5% in Brittany. These results obtained from two Breton centers taken together show that P. jirovecii prevalence in patients with CF in Brittany is lower than those observed in Germany, Spain, Brazil or in other regions of France. This study is a preliminary step in determining the risk factors for P. jirovecii acquisition, its epidemiological and clinical significance in CF patients through a prospective multicenter study. [ABSTRACT FROM AUTHOR]

Published
2018
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29. Infection à Nocardia farcinica chez un patient porteur d’une mucoviscidose [Nocardia farcinica infection in a patient with cystic fibrosis]

Author

Beucher, J., Belleguic, Chantal, Brinchault, Graziella, Deneuville, Eric, Donnio, Pierre-Yves, Roussey, Michel, Service de pneumologie, Centre de Ressources et de Compétences de la Mucoviscidose, hôpital Sud, Microbiologie : Risques Infectieux, Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-CHU Pontchaillou [Rennes]-Faculté de Chirurgie Dentaire de Rennes-Faculté d'Odontologie-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), Université de Rennes (UR)-CHU Pontchaillou [Rennes]-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique )-Université de Rennes - UFR d'Odontologie (UR Odontologie), Université de Rennes (UR)-Université de Rennes (UR), Université de Rennes (UR)-CHU Pontchaillou [Rennes]-Faculté de Chirurgie Dentaire de Rennes-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), and Brébion, Alice

Subjects
MESH: Bronchiectasis, MESH: Cystic Fibrosis, MESH: Pneumonia, Bacterial, Nocardia farcinica, MESH: Nocardia, MESH: Bacteriological Techniques, Détresse respiratoire, MESH: Sputum, MESH: Anti-Bacterial Agents, Mucoviscidose, MESH: Infusions, Intravenous, MESH: Adolescent, [SDV.MHEP] Life Sciences [q-bio]/Human health and pathology, MESH: Humans, MESH: Pseudomonas Infections, MESH: Opportunistic Infections, MESH: Male, MESH: Recurrence, MESH: Drug Therapy, Combination, MESH: Nocardia Infections, MESH: Administration, Oral, MESH: Pseudomonas aeruginosa, MESH: Pneumothorax, MESH: Tomography, X-Ray Computed, MESH: Respiratory Insufficiency, [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology
Abstract

International audience; Infections by Nocardia species are uncommon and generally affect immunocompromised patients. This bacteria has rarely been isolated from cystic fibrosis patients (CF), especially those who are not taking oral corticosteroids. We report a case of a patient with CF harbouring Nocardia farcinica. An 18-year-old male diagnosed with CF at the age of eight (F508 del/G85E) had been treated for allergic bronchopulmonary aspergillosis in 1998 with itraconazole, and a first colonization with Pseudomonas aeruginosa was eradicated in 2003. From May 2006, he presented with recurrent left- and right-sided pneumothorax. In June 2006, he presented with dyspnoea, fever, and nodular eruption on his ankles. Chest X-ray and CT scan revealed a right pneumothorax, severe bronchiectasis and bilateral alveolar consolidation. N. farcinica was idolated from his sputum without any other pathogens. Treatment with intravenous cotrimoxazole associated with imipenem and amikacin was initiated for three weeks followed by oral cotrimoxazole for a further nine months. The patient's symptoms and alveolar consolidation on CT scan improved. During 2007, his respiratory condition worsened and his FEV(1) declined from 50 to 26 % predicted. His pneumothorax recurred. He had chronic colonization with P. aeruginosa and was on the list for lung transplantation. Nocardia, a Gram positive bacillus, causes mainly pulmonary infection, usually in the context of immune suppression. The most frequent species is N. asteroides. In CF, very few cases have been reported; almost always N. asteroides, but exceptionally N. farcinica. In CF patients with worsening pulmonary condition, Nocardia should be considered, as well as other unusual pathogens.

Published
2010

30. Indications des antifongiques systémiques, spécialité par spécialité

Author

Guibert, Sophie De, Gandemer, Virginie, Belleguic, Chantal, Revest, Matthieu, Camus, Christophe, Caulet-Maugendre, Sylvie, Cardiet, Isabelle, Faucheux, Monique, Meunier, Catherine, Gangneux, Jean-Pierre, Institut de Génétique et Développement de Rennes (IGDR), Centre National de la Recherche Scientifique (CNRS)-Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES), Hématogoie pédiatrique, Centre de Ressource et de Compétences de la Mucoviscidose, Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-CHU Pontchaillou [Rennes], Service des maladies infectieuses et réanimation médicale [Rennes] = Infectious Disease and Intensive Care [Rennes], CHU Pontchaillou [Rennes], Institut de recherche en santé, environnement et travail (Irset), Université d'Angers (UA)-Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-École des Hautes Études en Santé Publique [EHESP] (EHESP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), Université de Rennes (UR)-Centre National de la Recherche Scientifique (CNRS), Centre de Ressource et de Compétences de la Mucoviscidose [CHU Rennes], and Université d'Angers (UA)-Université de Rennes (UR)-École des Hautes Études en Santé Publique [EHESP] (EHESP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique )

Subjects
[SDV]Life Sciences [q-bio], ComputingMilieux_MISCELLANEOUS
Abstract

National audience

Published
2010

31. Clinical value of Aspergillus detection in sputum obtained from 84 patients with cystic fibrosis

Author

Roussey, M., Chevrier, Sylviane, Giroux, Fabienne, Desrues, B., Belleguic, Chantal, Guiguen, Claude, Gangneux, Jean-Pierre, Service de Parasitologie-Mycologie, Université de Rennes 1 ( UR1 ), Université de Rennes ( UNIV-RENNES ) -Université de Rennes ( UNIV-RENNES ) -CHU Pontchaillou [Rennes], Virologie et Pathologie Humaine ( VirPath ), École normale supérieure - Lyon ( ENS Lyon ) -Université Claude Bernard Lyon 1 ( UCBL ), Université de Lyon-Université de Lyon-Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Centre National de la Recherche Scientifique ( CNRS ), Signalisation et Réponses aux Agents Infectieux et Chimiques ( SeRAIC ), Université de Rennes ( UNIV-RENNES ) -Université de Rennes ( UNIV-RENNES ) -IFR140, Service de Parasitologie-Mycologie [Rennes], Université de Rennes ( UNIV-RENNES ) -Université de Rennes ( UNIV-RENNES ) -Hôpital Pontchaillou-CHU Pontchaillou [Rennes], Université de Rennes (UR)-Hôpital Pontchaillou-CHU Pontchaillou [Rennes], Virologie et Pathologie Humaine (VirPath), École normale supérieure de Lyon (ENS de Lyon)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Signalisation et Réponses aux Agents Infectieux et Chimiques (SeRAIC), Université de Rennes (UR), Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Hôpital Pontchaillou-CHU Pontchaillou [Rennes], École normale supérieure - Lyon (ENS Lyon)-Université Claude Bernard Lyon 1 (UCBL), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES), and Brébion, Alice

Subjects
[SDV.MP]Life Sciences [q-bio]/Microbiology and Parasitology, [ SDV.MP ] Life Sciences [q-bio]/Microbiology and Parasitology, [SDV.MP] Life Sciences [q-bio]/Microbiology and Parasitology
Published
2008

32. Comparison of Clinical and Biological Features of Invasive Aspergillosis in Neutropenic and Non-Neutropenic Patients

Author

Cornillet, A., Camus, Christophe, Nimubona, S., Gandemer, Virginie, Tattevin, Pierre, Belleguic, Chantal, Chevrier, Sylviane, Leray, Emmanuelle, Guiguen, Claude, Gangneux, Jean-Pierre, Service des urgences [Rennes] = Emergency [Rennes], CHU Pontchaillou [Rennes], Service de médecine de l'enfant et de l'adolescent, Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES), Service des maladies infectieuses et réanimation médicale [Rennes] = Infectious Disease and Intensive Care [Rennes], Service de pneumologie, Service de Parasitologie-Mycologie [Rennes], Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Hôpital Pontchaillou-CHU Pontchaillou [Rennes], Signalisation et Réponses aux Agents Infectieux et Chimiques (SeRAIC), Brébion, Alice, Service de médecine de l'enfant et de l'adolescent [CHU Rennes], Université de Rennes (UR)-Hôpital Pontchaillou-CHU Pontchaillou [Rennes], and Université de Rennes (UR)

Subjects
[SDV.MP]Life Sciences [q-bio]/Microbiology and Parasitology, [SDV.MP] Life Sciences [q-bio]/Microbiology and Parasitology
Published
2006

34. Impaired Functions of Macrophage from Cystic Fibrosis Patients: CD11b, TLR-5 Decrease and sCD14, Inflammatory Cytokines Increase

Author

Simonin-Le Jeune, Karin, primary, Le Jeune, André, additional, Jouneau, Stéphane, additional, Belleguic, Chantal, additional, Roux, Pierre-François, additional, Jaguin, Marie, additional, Dimanche-Boitre, Marie-Thérèse, additional, Lecureur, Valérie, additional, Leclercq, Caroline, additional, Desrues, Benoît, additional, Brinchault, Graziella, additional, Gangneux, Jean-Pierre, additional, and Martin-Chouly, Corinne, additional

Published
2013
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37. Bronchectasies

Author

Belleguic, Chantal, primary, Léna, Hervé, additional, Renaud, Jean-Christophe, additional, Desrues, Benoit, additional, and Delaval, Philippe, additional

Published
2004
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41. Long-term safety and efficacy of tezacaftor–ivacaftor in individuals with cystic fibrosis aged 12 years or older who are homozygous or heterozygous for Phe508del CFTR(EXTEND): an open-label extension study

Author

Flume, Patrick A, Biner, Reta Fischer, Downey, Damian G, Brown, Cynthia, Jain, Manu, Fischer, Rainald, De Boeck, Kris, Sawicki, Gregory S, Chang, Philip, Paz-Diaz, Hildegarde, Rubin, Jaime L, Yang, Yoojung, Hu, Xingdi, Pasta, David J, Millar, Stefanie J, Campbell, Daniel, Wang, Xin, Ahluwalia, Neil, Owen, Caroline A, Wainwright, Claire E, Gibson, Ronald L., Rowe, Steven M., Lechtzin, Noah, Ahrens, Richard C., McCoy, Karen S., Aitken, Moira, Donaldson, Scott H., McBennett, Kimberly Ann, Pilewski, Joseph M., Billings, Joanne, Milla, Carlos, Rubenstein, Ronald, Rosenbluth, Daniel Brian, Linnemann, Rachel, Powers, Michael R., Fortner, Christopher, Frederick, Carla Anne, Liou, Theodore G., Black, Philip, Wang, Janice, Colombo, John L., Berdella, Maria, Indihar, Maria Veronica, Brown, Cynthia D., Anstead, Michael, Bilodeau, Lara, Sicilian, Leonard, Jain, Manu, Tolle, James Jerome, Moffett, Kathryn, Nasr, Samya, Taylor-Cousar, Jennifer, Barto, Tara Lynn, Antos, Nicholas, Rogers, John S., Quick, Bryon, Thompson, Henry R., Sawicki, Gregory, Barnett, Bruce, Zanni, Robert L., Smith, Thomas C., Schultz, Karen D., Keating, Claire, Flume, Patrick, Omlor, Gregory J., Ashare, Alix, Voter, Karen, Mehdi, Nighat, Ortiz, Maria Gabriela Tupayachi, Gardner, Tonia E., Boas, Steven R., Messore, Barbara, Zemanick, Edith, Jain, Raksha, McCarthy, Michael, Kissner, Dana G., Patel, Kapilkumar, McNamara, John, Philley, Julie, Berlinski, Ariel, Calimano, Francisco J., Chin, Terry, Conrad, Douglas, Daines, Cori, Raissy, Hengameh H., Keens, Thomas G., Lascano, Jorge E., McWilliams, Bennie, Morrissey, Brian, Reyes, Santiago, Chittivelu, Subramanyam, Hussain, Sabiha, Stone, Arvey, Wallace, James, Klingsberg, Ross, Biller, Julie A., Bui, Stephanie, Sommerburg, Olaf, Bignamini, Elisabetta, Collura, Mirella, Biner, Reta Fischer, Moller, Alexander, Salvatore, Donatello, Belleguic, Chantal, Bentur, Lea, Efrati, Ori, Kerem, Eitan, Prais, Dario, Gallego, Esther Quintana, Barry, Peter, Livnat-Levanon, Galit, Asensi, Jose Ramon Villa, Armstrong, David Stuart, de la Cruz, Oscar Asensio, Gilchrist, Francis, Tullis, Diana Elizabeth, Quon, Bradley, Lands, Larry C., Morrison, Nancy, Lavoie, Annick, Linnane, Barry, Elidemir, Okan, Ringshausen, Felix, Kappler, Matthias, Hebestreit, Helge, Mainz, Jochen, Kiefer, Alexander, Koerner-Rettberg, Cordula, Staab, Doris, Gleiber, Wolfgang, Pressler, Tacjana, Stehling, Florian, Hector, Andreas, Sutharsan, Sivagurunathan, Naehrlich, Lutz, Fischer, Rainald, Downey, Damian, Davies, Jane Carolyn, Ketchell, Robert Ian, Carroll, Mary Patricia, Doe, Simon, MacGregor, Gordon, Nash, Edward Fairbairn, Withers, Nicholas, Peckham, Daniel Gavin, Ledson, Martin James, Kansra, Sonal, Lee, Timothy William Rayner, Delaisi, Bertrand, Rault, Gilles, Le Bihan, Jean, Hubert, Dominique, Fajac, Isabelle, Sermet-Gaudelus, Isabelle, Bakker, Marleen, Arets, Bert, De Boeck, Christiane, Chiron, Raphael, Reix, Philippe, Mainguy, Catherine, van Braeckel, Eva, Malfroot, Anne, Durieu, Isabelle, Dufeu, Nadine Desmazes, Prevotat, Anne, van der Meer, Renske, Merkus, Petrus, Weersink, E.J.M., Gomez-Aguero, Isabel Barrio, Gartner, Silvia, Jover, Amparo Sole, Fernandez, Antonio Alvarez, Cox, Desmond William, McKone, Edward F., Plant, Barry James, Selvadurai, Hiranjan, Bowler, Simon David, Wainwright, Claire Elizabeth, Smith, Daniel, Middleton, Peter Gordon, Wilson, John William, Volpi, Sonia, Colombo, Carla, Fabrizzi, Benedetta, Lucidi, Vincenzina, Cresta, Federico, Cucchiara, Salvatore, Eber, Ernst, Ellemunter, Helmut, Huttegger, Isidor, Hjelte, Lena, Krantz, Christina, and Gilljam, Marita

Abstract

Tezacaftor–ivacaftor is an approved cystic fibrosis transmembrane conductance regulator (CFTR) modulator shown to be efficacious and generally safe and well tolerated over 8–24 weeks in phase 3 clinical studies in participants aged 12 years or older with cystic fibrosis homozygous for the Phe508del CFTRmutation (F/F; study 661-106 [EVOLVE]) or heterozygous for the Phe508del CFTRmutation and a residual function mutation (F/RF; study 661-108 [EXPAND]). Longer-term (>24 weeks) safety and efficacy of tezacaftor–ivacaftor has not been assessed in clinical studies. Here, we present results of study 661-110 (EXTEND), a 96-week open-label extension study that assessed long-term safety, tolerability, and efficacy of tezacaftor–ivacaftor in participants aged 12 years or older with cystic fibrosis who were homozygous or heterozygous for the Phe508del CFTRmutation.

Published
2021
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42. Intimacy and sexual life of females with cystic fibrosis.

Author

Simonnet Bisson F, Fauvernier M, Belleguic C, Boucher ID, Grenet D, Hamidfar R, Hubert D, Macey J, Murris-Espin M, Porzio M, Ramel S, Reynaud Q, Reynaud-Gaubert M, and Burgel PR

Abstract

Introduction: The effects of cystic fibrosis (CF) on females' sexuality have not been described. The aims of the present study were to describe and characterize sexual issues in females with CF., Methods: We included adult (≥18 years) females with CF currently or previously in a sexual relationship from 11 adult CF centres in France. We collected quantitative data using a modified version of the self-administered Pelvic Incontinence Sexual Questionnaire IUGA-Revised (PISQ-IR). We performed one-to-one interviews using a semi-directive framework in volunteer females to further characterize the effects of CF on sexual life. We summarized answers to questionnaire as percentages and analysed interviews by theme according to discourse analysis method., Results: Between November 2019 and July 2021, 212 females completed the PISQR-IR, of whom 15 were interviewed. Of the females who completed the questionnaire, 93.4% were concerned about the discomfort, pain, or unpleasantness they experienced during sexual intercourse. The most frequent cause of sexual difficulties was a lack of vaginal lubrication (78.8%), followed by pain (74.1%) and discomfort. Interviews revealed sexual lives that were uncomfortable or painful, unsatisfying or avoided for most females, with a strong impression of being sexually different, incompetent, and betrayed by their bodies in terms of sexual desire., Conclusion: Sexual difficulties faced by females with CF are highly prevalent. Increasing awareness regarding sex life issues in females with CF appears necessary to improve their management by CF multidisciplinary teams., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper. Françoise Simonnet reports financial support was provided by Association Gregory Lemarchal., (Copyright © 2024. Published by Elsevier B.V.)

Published
2024
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43. [Adverse effects of the tezacaftor/ivacaftor/elexacaftor combination that may lead to discontinuation: About a series of 10 cases].

Author

Blaisonneau E, Le Daré B, Mercerolle M, Bacle A, Triquet L, Osmont MN, Belleguic C, and Polard E

Abstract

Introduction: Cystic fibrosis transmembrane regulator (CFTR) channel modulators (ivacaftor, lumacaftor, tezacaftor and elexacaftor) represent a major advance in the management of cystic fibrosis. However, few data are available on the real-life safety profile of these medications, in particular on adverse events that may lead to their discontinuation. The aim of this study is to describe the characteristics and evolution of adverse reactions to the tezacaftor/ivacaftor/elexacaftor combination that led to discontinuation and were reported to the Centre régional de pharmacovigilance (CRPV) in Rennes (France)., Materials and Methods: A retrospective study was conducted from December 2021 to May 2023, focusing on cases of discontinuation of the tezacaftor/ivacaftor/elexacaftor combination due to the occurrence of one or more adverse effects, and reported to the CRPV of Rennes, France., Results: Ten cases of drug discontinuation were reported to the Rennes CRPV (6 women/4 men). Adverse effects mainly involved neuropsychiatric disorders (n=6), followed by liver disorders (n=2), ear, nose and throat disorders (n=1), and digestive disorders (n=1). The average duration of treatment at discontinuation was 339.8 [39-668] days. The drug was reintroduced in 7 patients on average 48.7 [7-123] days after discontinuation, with a dosage adjustments (n=4) consisting of changes in dosing times or a reduction in daily doses, with varying success in alleviating adverse symptoms depending on the case., Conclusion: This small case series suggests that neuropsychiatric adverse effects may occur more frequently than initially described after initiation of tezacaftor/ivacaftor/elexacaftor, and should be carefully screened and monitored. Dosage or administration schedule modifications may be considered for patients experiencing these adverse effects. Further pharmacovigilance studies are needed to better understand the adverse effect profiles of "caftors", their possible risk factors, and the impact of adjusting dosing modalities., (Copyright © 2024 Société française de pharmacologie et de thérapeutique. Published by Elsevier Masson SAS. All rights reserved.)

Published
2024
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44. Phagocytosis depends on TRPV2-mediated calcium influx and requires TRPV2 in lipids rafts: alteration in macrophages from patients with cystic fibrosis.

Author

Lévêque M, Penna A, Le Trionnaire S, Belleguic C, Desrues B, Brinchault G, Jouneau S, Lagadic-Gossmann D, and Martin-Chouly C

Subjects
Adolescent, Adult, Cells, Cultured, Female, Humans, Male, Middle Aged, Calcium metabolism, Cystic Fibrosis metabolism, Macrophages metabolism, Membrane Microdomains metabolism, Phagocytosis, TRPV Cation Channels metabolism
Abstract

Whereas many phagocytosis steps involve ionic fluxes, the underlying ion channels remain poorly defined. As reported in mice, the calcium conducting TRPV2 channel impacts the phagocytic process. Macrophage phagocytosis is critical for defense against pathogens. In cystic fibrosis (CF), macrophages have lost their capacity to act as suppressor cells and thus play a significant role in the initiating stages leading to chronic inflammation/infection. In a previous study, we demonstrated that impaired function of CF macrophages is due to a deficient phagocytosis. The aim of the present study was to investigate TRPV2 role in the phagocytosis capacity of healthy primary human macrophage by studying its activity, its membrane localization and its recruitment in lipid rafts. In primary human macrophages, we showed that P. aeruginosa recruits TRPV2 channels at the cell surface and induced a calcium influx required for bacterial phagocytosis. We presently demonstrate that to be functional and play a role in phagocytosis, TRPV2 might require a preferential localization in lipid rafts. Furthermore, CF macrophage displays a perturbed calcium homeostasis due to a defect in TRPV2. In this context, deregulated TRPV2-signaling in CF macrophages could explain their defective phagocytosis capacity that contribute to the maintenance of chronic infection.

Published
2018
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