749 results on '"Burgel, Pierre-Régis"'
Search Results
2. 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
- Full Text
- View/download PDF
3. Influenza and pneumococcal vaccination in patients with COPD from 3 French cohorts: Insufficient coverage and associated factors
- Author
-
Raherison, Chantal, Aguilaniu, Bernard, Zysman, Maeva, Burgel, Pierre-Régis, Hess, David, Ouaalaya, El Hassane, Tran, Thi Chien, and Roche, Nicolas
- Published
- 2024
- Full Text
- View/download PDF
4. 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
- Full Text
- View/download PDF
5. Health care resource utilization preceding death or lung transplantation in people with cystic fibrosis: HCRU before transplant or death in cystic fibrosis
- Author
-
Guyot, Erika, Reynaud, Quitterie, Belhassen, Manon, Bérard, Marjorie, Dehillotte, Clémence, Lemonnier, Lydie, Viprey, Marie, Van Ganse, Eric, Burgel, Pierre-Régis, and Durieu, Isabelle
- Published
- 2024
- Full Text
- View/download PDF
6. Bronchiectasis and asthma: Data from the European Bronchiectasis Registry (EMBARC)
- Author
-
Polverino, Eva, Dimakou, Katerina, Traversi, Letizia, Bossios, Apostolos, Haworth, Charles S., Loebinger, Michael R., De Soyza, Anthony, Vendrell, Montserrat, Burgel, Pierre-Régis, Mertsch, Pontus, McDonnell, Melissa, Škrgat, Sabina, Maiz Carro, Luis, Sibila, Oriol, van der Eerden, Menno, Kauppi, Paula, Hill, Adam T., Wilson, Robert, Milenkovic, Branislava, Menendez, Rosario, Murris, Marlene, Digalaki, Tonia, Crichton, Megan L., Borecki, Sermin, Obradovic, Dusanka, Nowinski, Adam, Amorim, Adelina, Torres, Antoni, Lorent, Natalie, Welte, Tobias, Blasi, Francesco, Van Braeckel, Eva, Altenburg, Josje, Shoemark, Amelia, Shteinberg, Michal, Boersma, Wim, Elborn, J. Stuart, Aliberti, Stefano, Ringshausen, Felix C., Chalmers, James D., and Goeminne, Pieter C.
- Published
- 2024
- Full Text
- View/download PDF
7. CT imaging shows specific pancreatic abnormalities in persons with cystic fibrosis related diabetes
- Author
-
Alexandre-Heymann, Laure, Puerto, Marie, Martin, Clémence, Burnet, Espérie, Mosnier-Pudar, Helen, Burgel, Pierre-Régis, and Larger, Etienne
- Published
- 2023
- Full Text
- View/download PDF
8. Standards for the care of people with cystic fibrosis (CF); Planning for a longer life
- Author
-
Gramegna, Andrea, Addy, Charlotte, Allen, Lorna, Bakkeheim, Egil, Brown, Catherine, Daniels, Thomas, Davies, Gwyneth, Davies, Jane C., De Marie, Ken, Downey, Damian, Felton, Imogen, Hafkemeyer, Sylvia, Hamouda, Samia, Kendall, Victoria, Lindberg, Ulrika, Macek, Milan, Mayell, Sarah, Pearlsman, Oren, Schechter, Michael S., Salvatori, Lidia, Sands, Dorota, Schwarz, Carsten, Shteinberg, Michal, Taylor, Julia, Taylor-Cousar, Jennifer L., Taylor-Robinson, David, Watkins, Bethan, Verkleij, Marieke, Bevan, Amanda, Castellani, Carlo, Drevinek, Pavel, Gartner, Silvia, Lammertyn, Elise, Landau, Eddie (Edwina) C., Middleton, Peter G., Plant, Barry J., Smyth, Alan R., van Koningsbruggen-Rietschel, Silke, Burgel, Pierre-Régis, and Southern, Kevin W.
- Published
- 2024
- Full Text
- View/download PDF
9. Burden of Uncontrolled Severe Asthma With and Without Elevated Type-2 Inflammatory Biomarkers
- Author
-
del Olmo, Ricardo, Anderson, Gary, Reddel, Helen, Rabahi, Marcelo, McIvor, Andrew, Sadatsafavi, Mohsen, Weinreich, Ulla, Burgel, Pierre-Régis, Devouassoux, Gilles, Papi, Alberto, Inoue, Hiromasa, Rendon, Adrián, van den Berge, Maarten, Beasley, Richard, García-Navarro, Alvar Agusti, Faner, Rosa, Rivera, José Olaguibel, Janson, Christer, Bilińska-Izydorczyk, Magdalena, Fagerås, Malin, Fihn-Wikander, Titti, Franzén, Stefan, Keen, Christina, Ostridge, Kristoffer, Chalmers, James, Harrison, Timothy, Pavord, Ian, Price, David, Azim, Adnan, Belton, Laura, Blé, Francois-Xavier, Erhard, Clement, Gairy, Kerry, Hughes, Rod, Lassi, Glenda, Müllerová, Hana, Rapsomaniki, Eleni, Scott, Ian Christopher, Chipps, Bradley, Christenson, Stephanie, Make, Barry, Tomaszewski, Erin, Benhabib, Gabriel, Ruiz, Xavier Bocca, Lisanti, Raul Eduardo, Marino, Gustavo, Mattarucco, Walter, Nogueira, Juan, Parody, Maria, Pascale, Pablo, Rodriguez, Pablo, Silva, Damian, Svetliza, Graciela, Victorio, Carlos F., Rolon, Roxana Willigs, Yañez, Anahi, Baines, Stuart, Bowler, Simon, Bremner, Peter, Bull, Sheetal, Carroll, Patrick, Chaalan, Mariam, Farah, Claude, Hammerschlag, Gary, Hancock, Kerry, Harrington, Zinta, Katsoulotos, Gregory, Kim, Joshua, Langton, David, Lee, Donald, Peters, Matthew, Prassad, Lakshman, Sajkov, Dimitar, Santiago, Francis, Simpson, Frederick Graham, Tai, Sze, Thomas, Paul, Wark, Peter, Delfini Cançado, José Eduardo, Cunha, Thúlio, Lima, Marina, Cardoso, Alexandre Pinto, FitzGerald, J. Mark, Anees, Syed, Bertley, John, Bell, Alan, Cheema, Amarjit, Chouinard, Guy, Csanadi, Michael, Dhar, Anil, Dhillon, Ripple, Kanawaty, David, Kelly, Allan, Killorn, William, Landry, Daniel, Luton, Robert, Mandhane, Piushkumar, Pek, Bonavuth, Petrella, Robert, Stollery, Daniel, Wang, Chen, Chen, Meihua, Chen, Yan, Gu, Wei, Christopher Hui, Kim Ming, Li, Manxiang, Li, Shiyue, Lijun, Ma, Qin, Guangyue, Song, Weidong, Tan, Wei, Tang, Yijun, Wang, Tan, Wen, Fuqiang, Wu, Feng, Xiang, PingChao, Xiao, Zuke, Xiong, Shengdao, Yang, Jinghua, Yang, Jingping, Zhang, Caiqing, Zhang, Min, Zhang, Ping, Zhang, Wei, Zheng, Xiaohe, Zhu, Dan, Bueno, Carlos Matiz, Grimaldos, Fabio Bolivar, Arboleda, Alejandra Cañas, Molina de Salazar, Dora, Bendstrup, Elisabeth, Hilberg, Ole, Kjellerup, Carsten, Raherison, Chantal, Bonniaud, Philippe, Brun, Olivier, Chouaid, Christos, Couturaud, Francis, de Blic, Jacques, Debieuvre, Didier, Delsart, Dominique, Demaegdt, Axelle, Demoly, Pascal, Deschildre, Antoine, Egron, Carole, Falchero, Lionel, Goupil, François, Kessler, Romain, Le Roux, Pascal, Mabire, Pascal, Mahay, Guillaume, Martinez, Stéphanie, Melloni, Boris, Moreau, Laurent, Riviere, Emilie, Roux-Claudé, Pauline, Soulier, Michel, Vignal, Guillaume, Yaici, Azzedine, Bals, Robert, Aries, Sven Philip, Beck, Ekkehard, Deimling, Andreas, Feimer, Jan, Grimm-Sachs, Vera, Groth, Gesine, Herth, Felix, Hoheisel, Gerhard, Kanniess, Frank, Lienert, Thomas, Mronga, Silke, Reinhardt, Jörg, Schlenska, Christian, Stolpe, Christoph, Teber, Ishak, Timmermann, Hartmut, Ulrich, Thomas, Velling, Peter, Wehgartner-Winkler, Sabina, Welling, Juergen, Winkelmann, Ernst-Joachim, Barbetta, Carlo, Braido, Fulvio, Cardaci, Vittorio, Clini, Enrico Maria, Costantino, Maria Teresa, Cuttitta, Giuseppina, di Gioacchino, Mario, Fois, Alessandro, Foschino-Barbaro, Maria Pia, Gammeri, Enrico, Inchingolo, Riccardo, Lavorini, Federico, Molino, Antonio, Nucera, Eleonora, Patella, Vincenzo, Pesci, Alberto, Ricciardolo, Fabio, Rogliani, Paola, Sarzani, Riccardo, Vancheri, Carlo, Vincenti, Rigoletta, Endo, Takeo, Fujita, Masaki, Hara, Yu, Horiguchi, Takahiko, Hosoi, Keita, Ide, Yumiko, Inomata, Minehiko, Inoue, Koji, Inoue, Sumito, Kato, Motokazu, Kawasaki, Masayuki, Kawayama, Tomotaka, Kita, Toshiyuki, Kobayashi, Kanako, Koto, Hiroshi, Nishi, Koichi, Saito, Junpei, Shimizu, Yasuo, Shirai, Toshihiro, Sugihara, Naruhiko, Takahashi, Ken-ichi, Tashimo, Hiroyuki, Tomii, Keisuke, Yamada, Takashi, Yanai, Masaru, Rendon, Adrian, Javier, Ruth Cerino, Peregrina, Alfredo Domínguez, Corzo, Marco Fernández, Gonzalez, Efraín Montano, Ramírez-Venegas, Alejandra, Boersma, Willem, Djamin, R.S., Eijsvogel, Michiel, Franssen, Frits, Goosens, Martijn, Graat-Verboom, Lidwien, Veen, Johannes in 't, Janssen, Rob, Kuppens, Kim, van de Ven, Mario, Bakke, Per, Brunstad, Ole Petter, Einvik, Gunnar, Høines, Kristian Jong, Khusrawi, Alamdar, Oien, Torbjorn, Yoon, Ho Joo, Chang, Yoon-Seok, Cho, Young Joo, Hwang, Yong Il, Kim, Woo Jin, Koh, Young-Il, Lee, Byung-Jae, Lee, Kwan-Ho, Lee, Sang-Pyo, Lee, Yong Chul, Lim, Seong Yong, Min, Kyung Hun, Oh, Yeon-Mok, Park, Choon-Sik, Park, Hae-Sim, Park, Heung-Woo, Rhee, Chin Kook, Yoon, Hyoung-Kyu, Andújar, Rubén, Anoro, Laura, García, María Buendía, Mozo, Paloma Campo, Campos, Sergio, Maldonado, Francisco Casas, Martínez, Manuel Castilla, Serrano, Carolina Cisneros, Casanova, Lorena Comeche, Corbacho, Dolores, Del Campo Matías, Felix, Echave-Sustaeta, Jose, Corral, Gloria Francisco, Setién, Pedro Gamboa, Clemente, Marta García, Núñez, Ignacio García, Robaina, Jose García, Salmones, Mercedes García, Marín Trigo, Jose Maria, Fernandez, Marta Nuñez, Palomo, Sara Nuñez, Pérez de Llano, Luis, Bastida, Ana Pueyo, Rañó, Ana, González-Moro, José Rodríguez, Reig, Albert Roger, Garrido, José Velasco, Curiac, Dan, Lif-Tiberg, Cornelia, Luts, Anders, Råhlen, Lennart, Rustscheff, Stefan, Adams, Frances, Bradman, Drew, Broughton, Emma, Cosgrove, John, Flood-Page, Patrick, Fuller, Elizabeth, Hartley, David, Hattotuwa, Keith, Jones, Gareth, Lewis, Keir, McGarvey, Lorcan, Morice, Alyn, Pandya, Preeti, Patel, Manish, Roy, Kay, Sathyamurthy, Ramamurthy, Thiagarajan, Swaminathan, Turner, Alice, Vestbo, Jorgen, Wedzicha, Wisia, Wilkinson, Tom, Wilson, Pete, Al-Asadi, Lo’Ay, Anholm, James, Averill, Francis, Bansal, Sandeep, Baptist, Alan, Campbell, Colin, Campos, Michael A., Crook, Gretchen, DeLeon, Samuel, Eid, Alain, Epstein, Ellen, Fritz, Stephen, Harris, Hoadley, Hewitt, Mitzie, Holguin, Fernando, Hudes, Golda, Jackson, Richard, Kaufman, Alan, Kaufman, David, Klapholz, Ari, Krishna, Harshavardhan, Lee, Daria, Lin, Robert, Maselli-Caceres, Diego, Mehta, Vinay, Moy, James N., Nwokoro, Ugo, Parikh, Purvi, Parikh, Sudhir, Perrino, Frank, Ruhlmann, James, Sassoon, Catherine, Settipane, Russell A., Sousa, Daniel, Sriram, Peruvemba, Wachs, Richard, Ding, Bo, Chen, Stephanie, Quinton, Anna, Cook, William, and Reddel, Helen K.
- Published
- 2024
- Full Text
- View/download PDF
10. Standards for the care of people with cystic fibrosis (CF); recognising and addressing CF health issues
- Author
-
Burgel, Pierre-Régis, Southern, Kevin W, Addy, Charlotte, Battezzati, Alberto, Berry, Claire, Bouchara, Jean-Philippe, Brokaar, Edwin, Brown, Whitney, Azevedo, Pilar, Durieu, Isabelle, Ekkelenkamp, Miquel, Finlayson, Felicity, Forton, Julian, Gardecki, Johanna, Hodkova, Pavla, Hong, Gina, Lowdon, Jacqueline, Madge, Su, Martin, Clémence, McKone, Edward, Munck, Anne, Ooi, Chee Y, Perrem, Lucy, Piper, Amanda, Prayle, Andrew, Ratjen, Felix, Rosenfeld, Margaret, Sanders, Don B, Schwarz, Carsten, Taccetti, Giovanni, Wainwright, Claire, West, Natalie E, Wilschanski, Michael, Bevan, Amanda, Castellani, Carlo, Drevinek, Pavel, Gartner, Silvia, Gramegna, Andrea, Lammertyn, Elise, Landau, Eddie (Edwina) C, Plant, Barry J, Smyth, Alan R, van Koningsbruggen-Rietschel, Silke, and Middleton, Peter G.
- Published
- 2024
- Full Text
- View/download PDF
11. Monitoring of ECFS quality standards for the clinical management of adults with cystic fibrosis
- Author
-
Gramegna, Andrea, Aliberti, Stefano, Amorim, Adelina, Blasi, Francesco, Bourke, Stephen, Burgel, Pierre-Régis, Diamantea, Filia, Durieu, Isabelle, Fila, Libor, Moreno, Rosa María Girón, Messore, Barbara, Pokojová, Eva, Taccetti, Giovanni, Verhulst, Stijn, Dugac, Andrea Vukić, Wege, Sabine, Duff, Alistair, Southern, Kevin W., and Castellani, Carlo
- Published
- 2024
- Full Text
- View/download PDF
12. Standards for the care of people with cystic fibrosis; establishing and maintaining health
- Author
-
Southern, Kevin W, Addy, Charlotte, Bell, Scott C, Bevan, Amanda, Borawska, Urzula, Brown, Catherine, Burgel, Pierre-Régis, Button, Brenda, Castellani, Carlo, Chansard, Audrey, Chilvers, Mark A, Davies, Gwyneth, Davies, Jane C, De Boeck, Kris, Declercq, Dimitri, Doumit, Michael, Drevinek, Pavel, Fajac, Isabelle, Gartner, Silvia, Georgiopoulos, Anna M, Gursli, Sandra, Gramegna, Andrea, Hansen, Carina ME, Hug, Martin J, Lammertyn, Elise, Landau, Edwina (Eddie) C., Langley, Ross, Mayer-Hamblett, Nicole, Middleton, Anna, Middleton, Peter G, Mielus, Monika, Morrison, Lisa, Munck, Anne, Plant, Barry, Ploeger, Maarten, Bertrand, Dominique Pougheon, Pressler, Tacjana, Quon, Bradley S, Radtke, Thomas, Saynor, Zoe L, Shufer, Ilan, Smyth, Alan R, Smith, Chris, and van Koningsbruggen-Rietschel, Silke
- Published
- 2024
- Full Text
- View/download PDF
13. Standards for the care of people with cystic fibrosis (CF): A timely and accurate diagnosis
- Author
-
Castellani, Carlo, Simmonds, Nicholas J, Barben, Jürg, Addy, Charlotte, Bevan, Amanda, Burgel, Pierre-Régis, Drevinek, Pavel, Gartner, Silvia, Gramegna, Andrea, Lammertyn, Elise, Landau, Eddie (Edwina) C., Middleton, Peter G., Plant, Barry J., Smyth, Alan R., van Koningsbruggen-Rietschel, Silke, Girodon, Emmanuelle, Kashirskaya, Nataliya, Munck, Anne, Nährlich, Lutz, Raraigh, Karen, Sermet-Gaudelus, Isabelle, Sommerburg, Olaf, and Southern, Kevin W.
- Published
- 2023
- Full Text
- View/download PDF
14. Nonsense mutations accelerate lung disease and decrease survival of cystic fibrosis children
- Author
-
Orenti, Annalisa, Pranke, Iwona, Faucon, Caroline, Varilh, Jessica, Hatton, Aurelie, Golec, Anita, Dehillotte, Clemence, Durieu, Isabelle, Reix, Philippe, Burgel, Pierre-Régis, Grenet, Dominique, Tasset, Céline, Gachelin, Elsa, Perisson, Caroline, Lepissier, Agathe, Dreano, Elise, Tondelier, Danielle, Chevalier, Benoit, Weiss, Laurence, Kiefer, Sébastien, Laurans, Muriel, Chiron, Raphael, Lemonnier, Lydie, Marguet, Christophe, Jung, Andreas, Edelman, Aleksander, Kerem, Bat-Sheva, Girodon, Emmanuelle, Taulan-Cadars, Magali, Hinzpeter, Alexandre, Kerem, Eitan, Naehrlich, Lutz, and Sermet-Gaudelus, Isabelle
- Published
- 2023
- Full Text
- View/download PDF
15. Dyspnea-induced Limitation (DYSLIM), a new self-administered concise questionnaire to evaluate dyspnea-related activity limitation in chronic respiratory diseases
- Author
-
Perez, Thierry, Roche, Nicolas, Nunes, Hilario, Burgel, Pierre-Régis, Chaouat, Ari, Surpas, Pascale, Herengt, Frédéric, Garcia, Gilles, Grosbois, Jean-Marie, Stelianides, Sandrine, de Rigal, Philippe, Guérin, Philippe, Arnould, Benoît, and Coste, Joël
- Published
- 2023
- Full Text
- View/download PDF
16. Cystic fibrosis
- Author
-
Fajac, Isabelle and Burgel, Pierre-Régis
- Published
- 2023
- Full Text
- View/download PDF
17. Cluster Analyses From the Real-World NOVELTY Study: Six Clusters Across the Asthma-COPD Spectrum
- Author
-
Olmo, Ricardo del, Anderson, Gary, Reddel, Helen, Rabahi, Marcelo, McIvor, Andrew, Sadatsafavi, Mohsen, Weinreich, Ulla, Burgel, Pierre-Régis, Devouassoux, Gilles, Papi, Alberto, Inoue, Hiromasa, Rendon, Adrián, van den Berge, Maarten, Beasley, Richard, García-Navarro, Alvar Agusti, Faner, Rosa, Olaguibel Rivera, José, Janson, Christer, Bilińska-Izydorczyk, Magdalena, Fagerås, Malin, Fihn-Wikander, Titti, Franzén, Stefan, Keen, Christina, Ostridge, Kristoffer, Chalmers, James, Harrison, Timothy, Pavord, Ian, Price, David, Azim, Adnan, Belton, Laura, Blé, Francois-Xavier, Erhard, Clement, Gairy, Kerry, Hughes, Rod, Lassi, Glenda, Müllerová, Hana, Rapsomaniki, Eleni, Scott, Ian Christopher, Chipps, Bradley, Christenson, Stephanie, Make, Barry, Tomaszewski, Erin, Benhabib, Gabriel, Ruiz, Xavier Bocca, Lisanti, Raul Eduardo, Marino, Gustavo, Mattarucco, Walter, Nogueira, Juan, Parody, Maria, Pascale, Pablo, Rodriguez, Pablo, Silva, Damian, Svetliza, Graciela, Victorio, Carlos F., Rolon, Roxana Willigs, Yañez, Anahi, Baines, Stuart, Bowler, Simon, Bremner, Peter, Bull, Sheetal, Carroll, Patrick, Chaalan, Mariam, Farah, Claude, Hammerschlag, Gary, Hancock, Kerry, Harrington, Zinta, Katsoulotos, Gregory, Kim, Joshua, Langton, David, Lee, Donald, Peters, Matthew, Prassad, Lakshman, Sajkov, Dimitar, Santiago, Francis, Simpson, Frederick Graham, Tai, Sze, Thomas, Paul, Wark, Peter, Cançado, José Eduardo Delfini, Cunha, Thúlio, Lima, Marina, Cardoso, Alexandre Pinto, FitzGerald, J. Mark, Anees, Syed, Bertley, John, Bell, Alan, Cheema, Amarjit, Chouinard, Guy, Csanadi, Michael, Dhar, Anil, Dhillon, Ripple, Kanawaty, David, Kelly, Allan, Killorn, William, Landry, Daniel, Luton, Robert, Mandhane, Piushkumar, Pek, Bonavuth, Petrella, Robert, Stollery, Daniel, Wang, Chen, Chen, Meihua, Chen, Yan, Gu, Wei, Christopher Hui, Kim Ming, Li, Manxiang, Li, Shiyue, Lijun, Ma, Qin, Guangyue, Song, Weidong, Tan, Wei, Tang, Yijun, Wang, Tan, Wen, Fuqiang, Wu, Feng, Xiang, PingChao, Xiao, Zuke, Xiong, Shengdao, Yang, Jinghua, Yang, Jingping, Zhang, Caiqing, Zhang, Min, Zhang, Ping, Zhang, Wei, Zheng, Xiaohe, Zhu, Dan, Bueno, Carlos Matiz, Grimaldos, Fabio Bolivar, Arboleda, Alejandra Cañas, de Salazar, Dora Molina, Bendstrup, Elisabeth, Hilberg, Ole, Kjellerup, Carsten, Raherison, Chantal, Bonniaud, Philippe, Brun, Olivier, Chouaid, Christos, Couturaud, Francis, de Blic, Jacques, Debieuvre, Didier, Delsart, Dominique, Demaegdt, Axelle, Demoly, Pascal, Deschildre, Antoine, Egron, Carole, Falchero, Lionel, Goupil, François, Kessler, Romain, Le Roux, Pascal, Mabire, Pascal, Mahay, Guillaume, Martinez, Stéphanie, Melloni, Boris, Moreau, Laurent, Riviere, Emilie, Roux-Claudé, Pauline, Soulier, Michel, Vignal, Guillaume, Yaici, Azzedine, Bals, Robert, Aries, Sven Philip, Beck, Ekkehard, Deimling, Andreas, Feimer, Jan, Grimm-Sachs, Vera, Groth, Gesine, Herth, Felix, Hoheisel, Gerhard, Kanniess, Frank, Lienert, Thomas, Mronga, Silke, Reinhardt, Jörg, Schlenska, Christian, Stolpe, Christoph, Teber, Ishak, Timmermann, Hartmut, Ulrich, Thomas, Velling, Peter, Wehgartner-Winkler, Sabina, Welling, Juergen, Winkelmann, Ernst-Joachim, Barbetta, Carlo, Braido, Fulvio, Cardaci, Vittorio, Clini, Enrico Maria, Costantino, Maria Teresa, Cuttitta, Giuseppina, di Gioacchino, Mario, Fois, Alessandro, Foschino-Barbaro, Maria Pia, Gammeri, Enrico, Inchingolo, Riccardo, Lavorini, Federico, Molino, Antonio, Nucera, Eleonora, Patella, Vincenzo, Pesci, Alberto, Ricciardolo, Fabio, Rogliani, Paola, Sarzani, Riccardo, Vancheri, Carlo, Vincenti, Rigoletta, Endo, Takeo, Fujita, Masaki, Hara, Yu, Horiguchi, Takahiko, Hosoi, Keita, Ide, Yumiko, Inomata, Minehiko, Inoue, Koji, Inoue, Sumito, Kato, Motokazu, Kawasaki, Masayuki, Kawayama, Tomotaka, Kita, Toshiyuki, Kobayashi, Kanako, Koto, Hiroshi, Nishi, Koichi, Saito, Junpei, Shimizu, Yasuo, Shirai, Toshihiro, Sugihara, Naruhiko, Takahashi, Ken-ichi, Tashimo, Hiroyuki, Tomii, Keisuke, Yamada, Takashi, Yanai, Masaru, Rendon, Adrian, Cerino Javier, Ruth, Domínguez Peregrina, Alfredo, Fernández Corzo, Marco, Montano Gonzalez, Efraín, Ramírez-Venegas, Alejandra, Boersma, Willem, Djamin, R.S., Eijsvogel, Michiel, Franssen, Frits, Goosens, Martijn, Graat-Verboom, Lidwien, Veen, Johannes in 't, Janssen, Rob, Kuppens, Kim, van de Ven, Mario, Bakke, Per, Brunstad, Ole Petter, Einvik, Gunnar, Høines, Kristian Jong, Khusrawi, Alamdar, Oien, Torbjorn, Yoon, Ho Joo, Chang, Yoon-Seok, Cho, Young Joo, Hwang, Yong Il, Kim, Woo Jin, Koh, Young-Il, Lee, Byung-Jae, Lee, Kwan-Ho, Lee, Sang-Pyo, Lee, Yong Chul, Lim, Seong Yong, Min, Kyung Hun, Oh, Yeon-Mok, Park, Choon-Sik, Park, Hae-Sim, Park, Heung-Woo, Rhee, Chin Kook, Yoon, Hyoung-Kyu, García-Navarro, Alvar Agustí, Andújar, Rubén, Anoro, Laura, Buendía García, María, Mozo, Paloma Campo, Campos, Sergio, Casas Maldonado, Francisco, Castilla Martínez, Manuel, Cisneros Serrano, Carolina, Comeche Casanova, Lorena, Corbacho, Dolores, Campo Matías, Felix Del, Echave-Sustaeta, Jose, Corral, Gloria Francisco, Gamboa Setién, Pedro, García Clemente, Marta, Núñez, Ignacio García, García Robaina, Jose, García Salmones, Mercedes, Marín Trigo, Jose Maria, Fernandez, Marta Nuñez, Palomo, Sara Nuñez, Pérez de Llano, Luis, Pueyo Bastida, Ana, Rañó, Ana, Rodríguez González-Moro, José, Reig, Albert Roger, Velasco Garrido, José, Curiac, Dan, Lif-Tiberg, Cornelia, Luts, Anders, Råhlen, Lennart, Rustscheff, Stefan, Adams, Frances, Bradman, Drew, Broughton, Emma, Cosgrove, John, Flood-Page, Patrick, Fuller, Elizabeth, Hartley, David, Hattotuwa, Keith, Jones, Gareth, Lewis, Keir, McGarvey, Lorcan, Morice, Alyn, Pandya, Preeti, Patel, Manish, Roy, Kay, Sathyamurthy, Ramamurthy, Thiagarajan, Swaminathan, Turner, Alice, Vestbo, Jørgen, Wedzicha, Wisia, Wilkinson, Tom, Wilson, Pete, Al-Asadi, Lo’Ay, Anholm, James, Averill, Francis, Bansal, Sandeep, Baptist, Alan, Campbell, Colin, Campos, Michael A., Crook, Gretchen, DeLeon, Samuel, Eid, Alain, Epstein, Ellen, Fritz, Stephen, Harris, Hoadley, Hewitt, Mitzie, Holguin, Fernando, Hudes, Golda, Jackson, Richard, Kaufman, Alan, Kaufman, David, Klapholz, Ari, Krishna, Harshavardhan, Lee, Daria, Lin, Robert, Maselli-Caceres, Diego, Mehta, Vinay, Moy, James N., Nwokoro, Ugo, Parikh, Purvi, Parikh, Sudhir, Perrino, Frank, Ruhlmann, James, Sassoon, Catherine, Settipane, Russell A., Sousa, Daniel, Sriram, Peruvemba, Wachs, Richard, Bansal, Aruna T., Agustí, Alvar, Fageras, Malin, Alacqua, Marianna, and Reddel, Helen K.
- Published
- 2023
- Full Text
- View/download PDF
18. Bronchiectasis in Europe: data on disease characteristics from the European Bronchiectasis registry (EMBARC)
- Author
-
Chalmers, James D, Polverino, Eva, Crichton, Megan L, Ringshausen, Felix C, De Soyza, Anthony, Vendrell, Montserrat, Burgel, Pierre Régis, Haworth, Charles S, Loebinger, Michael R, Dimakou, Katerina, Murris, Marlene, Wilson, Robert, Hill, Adam T, Menendez, Rosario, Torres, Antoni, Welte, Tobias, Blasi, Francesco, Altenburg, Josje, Shteinberg, Michal, Boersma, Wim, Elborn, J Stuart, Goeminne, Pieter C, and Aliberti, Stefano
- Published
- 2023
- Full Text
- View/download PDF
19. 2022 Update of indications and contraindications for lung transplantation in France
- Author
-
Le Pavec, Jérôme, Pison, Christophe, Hirschi, Sandrine, Bunel, Vincent, Mordant, Pierre, Brugière, Olivier, Guen, Morgan Le, Olland, Anne, Coiffard, Benjamin, Renaud-Picard, Benjamin, Tissot, Adrien, Brioude, Geoffrey, Borie, Raphaël, Crestani, Bruno, Deslée, Gaétan, Stelianides, Sandrine, Mal, Hervé, Schuller, Armelle, Falque, Loïc, Lorillon, Gwenaëlle, Tazi, Abdellatif, Burgel, Pierre Regis, Grenet, Dominique, De Miranda, Sandra, Bergeron, Anne, Launay, David, Cottin, Vincent, Nunes, Hilario, Valeyre, Dominique, Uzunhan, Yurdagul, Prévot, Grégoire, Sitbon, Olivier, Montani, David, Savale, Laurent, Humbert, Marc, Fadel, Elie, Mercier, Olaf, Mornex, Jean François, Dauriat, Gaëlle, and Reynaud-Gaubert, Martine
- Published
- 2023
- Full Text
- View/download PDF
20. Screening strategies for glucose tolerance abnormalities and diabetes in people with cystic fibrosis
- Author
-
Weiss, Laurence, Reix, Philippe, Mosnier-Pudar, Helen, Ronsin, Olivia, Beltrand, Jacques, Reynaud, Quitterie, Mely, Laurent, Burgel, Pierre-Régis, Stremler, Nathalie, Rakotoarisoa, Luc, Galderisi, Alfonso, Perge, Kevin, Bendelac, Nathalie, Abely, Michel, and Kessler, Laurence
- Published
- 2023
- Full Text
- View/download PDF
21. Asthma and COPD: distinct diseases or components of a continuum?
- Author
-
Martin, Clémence, primary, Chassagnon, Guillaume, additional, Günther, Sven, additional, Habib, Stéfanie, additional, Pastré, Jean, additional, Burgel, Pierre-Régis, additional, and Roche, Nicolas, additional
- Published
- 2023
- Full Text
- View/download PDF
22. Contributors
- Author
-
Anto, Aram, primary, Anto, Josep M, additional, Balli, Fabio, additional, Bedard, Annabelle, additional, Bedbrook, Anna, additional, Bousquet, Jean, additional, Brussino, Luisa, additional, Burgel, Pierre-Régis, additional, Burte, Emilie, additional, Camargo, Jr., Carlos A., additional, Canonica, G Walter, additional, Carr, Tara F., additional, Casale, Thomas, additional, Casas, Lidia, additional, Chassagnon, Guillaume, additional, Czarlewski, Wienczyslawa, additional, Dharmage, Shyamali C., additional, Dumas, Orianne, additional, Fonseca, Joao A, additional, Gaga, Mina, additional, Gemicioglu, Bilun, additional, Günther, Sven, additional, Habib, Stéfanie, additional, Hasegawa, Kohei, additional, Heinrich, Joachim, additional, Idrose, N. Sabrina, additional, Jacquemin, Bénédicte, additional, Klimek, Ludger, additional, Kvedariene, Violeta, additional, Leynaert, Bénédicte, additional, Li, Zhen, additional, Louis, Renaud, additional, Martin, Clémence, additional, Morin, Andréanne, additional, Moual, Nicole Le, additional, Nadif, Rachel, additional, Nagase, Hiroyuki, additional, Ober, Carole, additional, Pastré, Jean, additional, Patel, Sanjay B., additional, Puggioni, Francesca, additional, Real, Francisco Gómez, additional, Roche, Nicolas, additional, Sastre, Joaquin, additional, Savouré, Marine, additional, Schoettler, Nathan, additional, Scichilone, Nicola, additional, Sousa-Pinto, Bernardo, additional, Suzukawa, Maho, additional, Toivonen, Laura, additional, Valiulis, Arunas, additional, Varraso, Raphaëlle, additional, Yorgancioglu, Arzu, additional, Zervas, Eleptherios, additional, and Zuberbier, Torsten, additional
- Published
- 2023
- Full Text
- View/download PDF
23. The Changing Epidemiology of Cystic Fibrosis: The Implications for Adult Care
- Author
-
Burgel, Pierre-Régis, Burnet, Espérie, Regard, Lucile, and Martin, Clémence
- Published
- 2023
- Full Text
- View/download PDF
24. New concepts in antimicrobial resistance in cystic fibrosis respiratory infections
- Author
-
Drevinek, Pavel, Canton, Rafael, Johansen, Helle Krogh, Hoffman, Lucas, Coenye, Tom, Burgel, Pierre-Regis, and Davies, Jane C
- Published
- 2022
- Full Text
- View/download PDF
25. The burden of mild asthma: Clinical burden and healthcare resource utilisation in the NOVELTY study
- Author
-
Benhabib, Gabriel, Mandhane, Piushkumar, Ruiz, Xavier Bocca, McIvor, Andrew, Olmo, Ricardo del, Pek, Bonavuth, Lisanti, Raul Eduardo, Petrella, Robert, Marino, Gustavo, Stollery, Daniel, Mattarucco, Walter, Chen, Meihua, Nogueira, Juan, Chen, Yan, Parody, Maria, Gu, Wei, Pascale, Pablo, Hui, Kim Ming Christopher, Rodriguez, Pablo, Li, Manxiang, Silva, Damian, Li, Shiyue, Svetliza, Graciela, Ma, Lijun, Victorio, Carlos F., Qin, Guangyue, Rolon, Roxana Willigs, Song, Weidong, Yañez, Anahi, Tan, Wei, Baines, Stuart, Tang, Yijun, Bowler, Simon, Wang, Chen, Bremner, Peter, Wang, Tan, Bull, Sheetal, Wen, Fuqiang, Carroll, Patrick, Wu, Feng, Chaalan, Mariam, Xiang, PingChao, Farah, Claude, Xiao, Zuke, Hammerschlag, Gary, Xiong, Shengdao, Hancock, Kerry, Yang, Jinghua, Harrington, Zinta, Yang, Jingping, Katsoulotos, Gregory, Zhang, Caiqing, Kim, Joshua, Zhang, Min, Langton, David, Zhang, Ping, Lee, Donald, Zhang, Wei, Peters, Matthew, Zheng, Xiaohe, Prassad, Lakshman, Zhu, Dan, Reddel, Helen, Bolivar Grimaldos, Fabio, Sajkov, Dimitar, Arboleda, Alejandra Cañas, Santiago, Francis, Bueno, Carlos Matiz, Simpson, Frederick Graham, Molina de Salazar, Dora, Tai, Sze, Bendstrup, Elisabeth, Thomas, Paul, Hilberg, Ole, Wark, Peter, Kjellerup, Carsten, Cançado, José Eduardo Delfini, Weinreich, Ulla, Cunha, Thúlio, Bonniaud, Philippe, Lima, Marina, Brun, Olivier, Cardoso, Alexandre Pinto, Burgel, Pierre-Régis, Rabahi, Marcelo, Chouaid, Christos, Anees, Syed, Couturaud, Francis, Bertley, John, de Blic, Jacques, Bell, Alan, Debieuvre, Didier, Cheema, Amarjit, Delsart, Dominique, Chouinard, Guy, Demaegdt, Axelle, Csanadi, Michael, Demoly, Pascal, Dhar, Anil, Deschildre, Antoine, Dhillon, Ripple, Devouassoux, Gilles, FitzGerald, J. Mark, Egron, Carole, Kanawaty, David, Falchero, Lionel, Kelly, Allan, Goupil, François, Killorn, William, Kessler, Romain, Landry, Daniel, Le Roux, Pascal, Luton, Robert, Mabire, Pascal, Mahay, Guillaume, Ide, Yumiko, Martinez, Stéphanie, Inomata, Minehiko, Melloni, Boris, Inoue, Hiromasa, Moreau, Laurent, Inoue, Koji, Raherison, Chantal, Inoue, Sumito, Riviere, Emilie, Kato, Motokazu, Roux-Claudé, Pauline, Kawasaki, Masayuki, Soulier, Michel, Kawayama, Tomotaka, Vignal, Guillaume, Kita, Toshiyuki, Yaici, Azzedine, Kobayashi, Kanako, Aries, Sven Philip, Koto, Hiroshi, Bals, Robert, Nishi, Koichi, Beck, Ekkehard, Saito, Junpei, Deimling, Andreas, Shimizu, Yasuo, Feimer, Jan, Shirai, Toshihiro, Grimm-Sachs, Vera, Sugihara, Naruhiko, Groth, Gesine, Takahashi, Ken-ichi, Herth, Felix, Tashimo, Hiroyuki, Hoheisel, Gerhard, Tomii, Keisuke, Kanniess, Frank, Yamada, Takashi, Lienert, Thomas, Yanai, Masaru, Mronga, Silke, Javier, Ruth Cerino, Reinhardt, Jörg, Domínguez Peregrina, Alfredo, Schlenska, Christian, Corzo, Marco Fernández, Stolpe, Christoph, Montano Gonzalez, Efraín, Teber, Ishak, Ramírez-Venegas, Alejandra, Timmermann, Hartmut, Rendon, Adrian, Ulrich, Thomas, Boersma, Willem, Velling, Peter, Djamin, R.S., Wehgartner-Winkler, Sabina, Eijsvogel, Michiel, Welling, Juergen, Franssen, Frits, Winkelmann, Ernst-Joachim, Goosens, Martijn, Barbetta, Carlo, Graat-Verboom, Lidwien, Braido, Fulvio, Veen, Johannes in 't, Cardaci, Vittorio, Janssen, Rob, Clini, Enrico Maria, Kuppens, Kim, Costantino, Maria Teresa, van den Berge, Maarten, Cuttitta, Giuseppina, van de Ven, Mario, di Gioacchino, Mario, Brunstad, Ole Petter, Fois, Alessandro, Einvik, Gunnar, Foschino-Barbaro, Maria Pia, Høines, Kristian Jong, Gammeri, Enrico, Khusrawi, Alamdar, Inchingolo, Riccardo, Oien, Torbjorn, Lavorini, Federico, Chang, Yoon-Seok, Molino, Antonio, Cho, Young Joo, Nucera, Eleonora, Hwang, Yong Il, Papi, Alberto, Kim, Woo Jin, Patella, Vincenzo, Koh, Young-Il, Pesci, Alberto, Lee, Byung-Jae, Ricciardolo, Fabio, Lee, Kwan-Ho, Rogliani, Paola, Lee, Sang-Pyo, Sarzani, Riccardo, Lee, Yong Chul, Vancheri, Carlo, Lim, Seong Yong, Vincenti, Rigoletta, Min, Kyung Hun, Endo, Takeo, Oh, Yeon-Mok, Fujita, Masaki, Park, Choon-Sik, Hara, Yu, Park, Hae-Sim, Horiguchi, Takahiko, Park, Heung-Woo, Hosoi, Keita, Rhee, Chin Kook, Yoon, Ho Joo, Morice, Alyn, Yoon, Hyoung-Kyu, Pandya, Preeti, García-Navarro, Alvar Agusti, Patel, Manish, Andújar, Rubén, Roy, Kay, Anoro, Laura, Sathyamurthy, Ramamurthy, García, María Buendía, Thiagarajan, Swaminathan, Mozo, Paloma Campo, Turner, Alice, Campos, Sergio, Vestbo, Jorgen, Maldonado, Francisco Casas, Wedzicha, Wisia, Castilla Martínez, Manuel, Wilkinson, Tom, Serrano, Carolina Cisneros, Wilson, Pete, Comeche Casanova, Lorena, Al-Asadi, Lo’Ay, Corbacho, Dolores, Anholm, James, Campo Matías, Felix Del, Averill, Frank, Echave-Sustaeta, Jose, Bansal, Sandeep, Corral, Gloria Francisco, Baptist, Alan, Gamboa Setién, Pedro, Campbell, Colin, García Clemente, Marta, Campos, Michael A., Núñez, Ignacio García, Chipps, Bradley, Robaina, Jose García, Crook, Gretchen, García Salmones, Mercedes, DeLeon, Samuel, Marín Trigo, Jose Maria, Eid, Alain, Fernandez, Marta Nuñez, Epstein, Ellen, Palomo, Sara Nuñez, Fritz, Stephen, Olaguibel Rivera, José, Harris, Hoadley, de Llano, Luis Pérez, Hewitt, Mitzie, Pueyo Bastida, Ana, Holguin, Fernando, Rañó, Ana, Hudes, Golda, Rodríguez González-Moro, José, Jackson, Richard, Reig, Albert Roger, Kaufman, Alan, Velasco Garrido, José, Kaufman, David, Curiac, Dan, Klapholz, Ari, Janson, Christer, Krishna, Harshavardhan, Lif-Tiberg, Cornelia, Lee, Daria, Luts, Anders, Lin, Robert, Råhlen, Lennart, Maselli-Caceres, Diego, Rustscheff, Stefan, Mehta, Vinay, Adams, Frances, Moy, James N., Bradman, Drew, Nwokoro, Ugo, Broughton, Emma, Parikh, Purvi, Cosgrove, John, Parikh, Sudhir, Flood-Page, Patrick, Perrino, Frank, Fuller, Elizabeth, Ruhlmann, James, Harrison, Timothy, Sassoon, Catherine, Hartley, David, Settipane, Russell A., Hattotuwa, Keith, Sousa, Daniel, Jones, Gareth, Sriram, Peruvemba, Lewis, Keir, Wachs, Richard, McGarvey, Lorcan, Golam, Sarowar Muhammad, Beasley, Richard, FitzGerald, J Mark, Harrison, Tim, Hughes, Rod, Müllerová, Hana, Olaguibel, José María, Rapsomaniki, Eleni, Reddel, Helen K., and Sadatsafavi, Mohsen
- Published
- 2022
- Full Text
- View/download PDF
26. Frequent productive cough: Symptom burden and future exacerbation risk among patients with asthma and/or COPD in the NOVELTY study
- Author
-
Benhabib, Gabriel, Ruiz, Xavier Bocca, Olmo, Ricardo del, Lisanti, Raul Eduardo, Marino, Gustavo, Mattarucco, Walter, Nogueira, Juan, Parody, Maria, Pascale, Pablo, Rodriguez, Pablo, Silva, Damian, Svetliza, Graciela, Victorio, Carlos F., Rolon, Roxana Willigs, Yañez, Anahi, Baines, Stuart, Bowler, Simon, Bremner, Peter, Bull, Sheetal, Carroll, Patrick, Chaalan, Mariam, Farah, Claude, Hammerschlag, Gary, Hancock, Kerry, Harrington, Zinta, Katsoulotos, Gregory, Kim, Joshua, Langton, David, Lee, Donald, Peters, Matthew, Prassad, Lakshman, Reddel, Helen, Sajkov, Dimitar, Santiago, Francis, Simpson, Frederick Graham, Tai, Sze, Thomas, Paul, Wark, Peter, Delfini Cançado, José Eduardo, Cunha, Thúlio, Lima, Marina, Cardoso, Alexandre Pinto, Rabahi, Marcelo, Anees, Syed, Bertley, John, Bell, Alan, Cheema, Amarjit, Chouinard, Guy, Csanadi, Michael, Dhar, Anil, Dhillon, Ripple, FitzGerald, J. Mark, Kanawaty, David, Kelly, Allan, Killorn, William, Landry, Daniel, Luton, Robert, Mandhane, Piushkumar, McIvor, Andrew, Pek, Bonavuth, Petrella, Robert, Stollery, Daniel, Chen, Meihua, Chen, Yan, Gu, Wei, Christopher Hui, Kim Ming, Li, Manxiang, Li, Shiyue, Lijun, Ma, Qin, Guangyue, Song, Weidong, Tan, Wei, Tang, Yijun, Wang, Chen, Wang, Tan, Wen, Fuqiang, Wu, Feng, Xiang, PingChao, Xiao, Zuke, Xiong, Shengdao, Yang, Jinghua, Yang, Jingping, Zhang, Caiqing, Zhang, Min, Zhang, Ping, Zhang, Wei, Zheng, Xiaohe, Zhu, Dan, Grimaldos, Fabio Bolivar, Arboleda, Alejandra Cañas, Bueno, Carlos Matiz, Molina de Salazar, Dora, Bendstrup, Elisabeth, Hilberg, Ole, Kjellerup, Carsten, Weinreich, Ulla, Bonniaud, Philippe, Brun, Olivier, Burgel, Pierre-Régis, Chouaid, Christos, Couturaud, Francis, de Blic, Jacques, Debieuvre, Didier, Delsart, Dominique, Demaegdt, Axelle, Demoly, Pascal, Deschildre, Antoine, Devouassoux, Gilles, Egron, Carole, Falchero, Lionel, Goupil, François, Kessler, Romain, Le Roux, Pascal, Mabire, Pascal, Mahay, Guillaume, Martinez, Stéphanie, Melloni, Boris, Moreau, Laurent, Raherison, Chantal, Riviere, Emilie, Roux-Claudé, Pauline, Soulier, Michel, Vignal, Guillaume, Yaici, Azzedine, Aries, Sven Philip, Bals, Robert, Beck, Ekkehard, Deimling, Andreas, Feimer, Jan, Grimm-Sachs, Vera, Groth, Gesine, Herth, Felix, Hoheisel, Gerhard, Kanniess, Frank, Lienert, Thomas, Mronga, Silke, Reinhardt, Jörg, Schlenska, Christian, Stolpe, Christoph, Teber, Ishak, Timmermann, Hartmut, Ulrich, Thomas, Velling, Peter, Wehgartner-Winkler, Sabina, Welling, Juergen, Winkelmann, Ernst-Joachim, Barbetta, Carlo, Braido, Fulvio, Cardaci, Vittorio, Clini, Enrico Maria, Costantino, Maria Teresa, Cuttitta, Giuseppina, di Gioacchino, Mario, Fois, Alessandro, Foschino-Barbaro, Maria Pia, Gammeri, Enrico, Inchingolo, Riccardo, Lavorini, Federico, Molino, Antonio, Nucera, Eleonora, Papi, Alberto, Patella, Vincenzo, Pesci, Alberto, Ricciardolo, Fabio, Rogliani, Paola, Sarzani, Riccardo, Vancheri, Carlo, Vincenti, Rigoletta, Endo, Takeo, Fujita, Masaki, Hara, Yu, Horiguchi, Takahiko, Hosoi, Keita, Ide, Yumiko, Inomata, Minehiko, Inoue, Hiromasa, Inoue, Koji, Inoue, Sumito, Kato, Motokazu, Kawasaki, Masayuki, Kawayama, Tomotaka, Kita, Toshiyuki, Kobayashi, Kanako, Koto, Hiroshi, Nishi, Koichi, Saito, Junpei, Shimizu, Yasuo, Shirai, Toshihiro, Sugihara, Naruhiko, Takahashi, Ken-ichi, Tashimo, Hiroyuki, Tomii, Keisuke, Yamada, Takashi, Yanai, Masaru, Javier, Ruth Cerino, Peregrina, Alfredo Domínguez, Corzo, Marco Fernández, Gonzalez, Efraín Montano, Ramírez-Venegas, Alejandra, Rendon, Adrian, Boersma, Willem, Djamin, R.S., Eijsvogel, Michiel, Franssen, Frits, Goosens, Martijn, Graat-Verboom, Lidwien, Veen, Johannes in 't, Janssen, Rob, Kuppens, Kim, van den Berge, Maarten, van de Ven, Mario, Brunstad, Ole Petter, Einvik, Gunnar, Høines, Kristian Jong, Khusrawi, Alamdar, Oien, Torbjorn, Chang, Yoon-Seok, Cho, Young Joo, Hwang, Yong Il, Kim, Woo Jin, Koh, Young-Il, Lee, Byung-Jae, Lee, Kwan-Ho, Lee, Sang-Pyo, Lee, Yong Chul, Lim, Seong Yong, Min, Kyung Hun, Oh, Yeon-Mok, Park, Choon-Sik, Park, Hae-Sim, Park, Heung-Woo, Rhee, Chin Kook, Yoon, Ho Joo, Yoon, Hyoung-Kyu, García-Navarro, Alvar Agusti, Andújar, Rubén, Anoro, Laura, García, María Buendía, Mozo, Paloma Campo, Campos, Sergio, Maldonado, Francisco Casas, Martínez, Manuel Castilla, Serrano, Carolina Cisneros, Casanova, Lorena Comeche, Corbacho, Dolores, Del Campo Matías, Felix, Echave-Sustaeta, Jose, Corral, Gloria Francisco, Setién, Pedro Gamboa, Clemente, Marta García, Núñez, Ignacio García, Robaina, Jose García, Salmones, Mercedes García, Marín Trigo, Jose Maria, Fernandez, Marta Nuñez, Palomo, Sara Nuñez, Rivera, José Olaguibel, Pérez de Llano, Luis, Bastida, Ana Pueyo, Rañó, Ana, González-Moro, José Rodríguez, Reig, Albert Roger, Garrido, José Velasco, Curiac, Dan, Janson, Christer, Lif-Tiberg, Cornelia, Luts, Anders, Råhlen, Lennart, Rustscheff, Stefan, Adams, Frances, Bradman, Drew, Broughton, Emma, Cosgrove, John, Flood-Page, Patrick, Fuller, Elizabeth, Harrison, Timothy, Hartley, David, Hattotuwa, Keith, Jones, Gareth, Lewis, Keir, McGarvey, Lorcan, Morice, Alyn, Pandya, Preeti, Patel, Manish, Roy, Kay, Sathyamurthy, Ramamurthy, Thiagarajan, Swaminathan, Turner, Alice, Vestbo, Jorgen, Wedzicha, Wisia, Wilkinson, Tom, Wilson, Pete, Al-Asadi, Lo’Ay, Anholm, James, Averill, Frank, Bansal, Sandeep, Baptist, Alan, Campbell, Colin, Campos, Michael A., Chipps, Bradley, Crook, Gretchen, DeLeon, Samuel, Eid, Alain, Epstein, Ellen, Fritz, Stephen, Harris, Hoadley, Hewitt, Mitzie, Holguin, Fernando, Hudes, Golda, Jackson, Richard, Kaufman, Alan, Kaufman, David, Klapholz, Ari, Krishna, Harshavardhan, Lee, Daria, Lin, Robert, Maselli-Caceres, Diego, Mehta, Vinay, Moy, James N., Nwokoro, Ugo, Parikh, Purvi, Parikh, Sudhir, Perrino, Frank, Ruhlmann, James, Sassoon, Catherine, Settipane, Russell A., Sousa, Daniel, Sriram, Peruvemba, Wachs, Richard, Hughes, Rod, Rapsomaniki, Eleni, Keen, Christina, Make, Barry J., Tomaszewski, Erin L., Müllerová, Hana, and Reddel, Helen K.
- Published
- 2022
- Full Text
- View/download PDF
27. Position paper of the French Society of Respiratory Diseases regarding pharmacological treatment optimization for stable COPD in 2021
- Author
-
Zysman, Maeva, Baptista, Bruno Ribeiro, Soumagne, Thibaud, da Silva, Vanessa Marques, Martin, Clémence, de Menonville, Charlotte Thibault, Boyer, Laurent, Degano, Bruno, Burgel, Pierre-Régis, Perez, Thierry, Bourdin, Arnaud, Raherison, Chantal, Pégliasco, Hervé, Piperno, Daniel, Zanetti, Christophe, Morel, Hughes, Delclaux, Bertrand, Delafosse, Christian, Lorenzo, Alain, Housset, Bruno, Morélot-Panzini, Capucine, Chabot, François, Devillier, Philippe, Deslée, Gaëtan, and Roche, Nicolas
- Published
- 2022
- Full Text
- View/download PDF
28. Tobramycin safety and efficacy review article
- Author
-
Schwarz, Carsten, Taccetti, Giovanni, Burgel, Pierre-Regis, and Mulrennan, Siobhain
- Published
- 2022
- Full Text
- View/download PDF
29. Seasonal and climatic influence on respiratory infections in children with cystic fibrosis.
- Author
-
Mésinèle, Julie, Ruffin, Manon, Guillot, Loïc, Boëlle, Pierre-Yves, Corvol, Harriet, Abelly, Michel, Allou, Nathalie, Arnouat, Baptiste, Piccini, Carole Bailly, Belleguic, Chantal, Bessaci, Katia, Bihouee, Tiphaine, Billon, Yves, Bremont, François, Brouard, Jacques, Bui, Stéphanie, Burgel, Pierre-Régis, Camara, Boubou, Campbell, Karine, and Chiron, Raphael
- Subjects
CLIMATIC zones ,RESPIRATORY infections in children ,MARINE west coast climate ,SPRING ,CYSTIC fibrosis - Abstract
Pseudomonas aeruginosa (Pa) and Methicillin susceptible Staphylococcus aureus (MSSA) are the predominant bacteria found in the airways of people with cystic fibrosis (pwCF), significantly contributing to lung disease progression. While various factors influencing the initial acquisition (IA) of these pathogens are known, the impact of environmental conditions remains understudied. This epidemiological study assessed the risk of MSSA and Pa initial acquisitions in relation to seasonality and climatic zones among 1,184 French pwCF under 18 years old. The age at IA for Pa (Pa-IA) and MSSA (MSSA-IA) was estimated using the Kaplan–Meier method. Seasonality and climatic zones were analysed as risk factors using time-varying Cox regression models. The median age at MSSA-IA was notably earlier (2.0 years) than that at Pa-IA (5.1 years). MSSA-IA occurred increasingly younger in more recent birth cohorts, while the age at Pa-IA remained stable over time. The risk of Pa-IA was consistently higher in all seasons compared with spring, peaking in autumn (HR = 1.53), irrespective of climatic zones. In Oceanic and Continental climates, the highest risk for MSSA-IA was in winter (HRs = 1.45 and 1.20 respectively). In the Mediterranean climate, the risk of MSSA-IA was lower in winter compared to spring (HRs = 0.68 and 0.61 respectively), and the median age at MSSA-IA later than for Pa-IA. This study demonstrates that seasonality and meteorological factors may influence acquisition of MSSA and Pa in pwCF. These findings suggest that environmental factors play a role in pathogen acquisition dynamics in CF and could inform the development of preventive strategies. [ABSTRACT FROM AUTHOR]
- Published
- 2024
- Full Text
- View/download PDF
30. 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
- Full Text
- View/download PDF
31. Reversal of Bronchiectasis in Children: Early Intervention May Stop the Vicious Circle!
- Author
-
Burgel, Pierre-Régis, primary and Bush, Andrew, additional
- Published
- 2024
- Full Text
- View/download PDF
32. Beyond Kaftrio : mechanistic insights to maximize N1303K-CFTR rescue
- Author
-
Pranke, Iwona, primary, Capurro, Valeria, additional, Chevalier, Benoit, additional, Pesce, Emanuela, additional, Tomati, Valeria, additional, Pastorino, Cristina, additional, Hatton, Aurelie, additional, Urien, Saik, additional, Lena, Mariateresa, additional, Dréano, Elise, additional, Bocciardi, Renata, additional, Zara, Federico, additional, Pantano, Stefano, additional, Terlizzi, Vito, additional, Lucanto, Cristina, additional, Costa, Stefano, additional, Claut, Laura, additional, Daccò, Valeria, additional, Poli, Piercarlo, additional, Maschio, Massimo, additional, Fabrizzi, Benedetta, additional, Caporelli, Nicole, additional, Cipolli, Marco, additional, Volpi, Sonia, additional, Jung, Vincent, additional, Roger, Kevin, additional, Chedevergne, Frederique, additional, Cosson, Laure, additional, Macey, Julie, additional, LeBihan, Jean, additional, Weiss, Laurence, additional, Grenet, Dominique, additional, Viala, Laurence LeClainche, additional, Douvry, Benoit, additional, Ravoninjatovo, Bruno, additional, Audousset, Camille, additional, Tatopoulos, Aurélie, additional, Thiriez, Bénédicte Richaud, additional, Baravalle, Melissa, additional, Thouvenin, Guillaume, additional, Labbé, Guillaume, additional, Mittaine, Marie, additional, Reix, Philippe, additional, Durieu, Isabelle, additional, Mankikian, Julie, additional, Bui, Stéphanie, additional, Kelly-Aubert, Mairead, additional, Nguyen–Khoa, Thao, additional, Khoukh, Karim, additional, Martin, Clémence, additional, Guerrera, Chiarra, additional, Silva, Jennifer Da, additional, di Carli, Paola, additional, Castellani, Carlo, additional, Cresta, Federico, additional, Galietta, Luis, additional, Guillemaut, Anne, additional, Bouazza, Naim, additional, Girodon, Emmanuelle, additional, Remus, Natacha, additional, Burgel, Pierre Régis, additional, Sermet-Gaudelus, Isabelle, additional, Hinzpeter, Alexandre, additional, and Pedemonte, Nicoletta, additional
- Published
- 2024
- Full Text
- View/download PDF
33. “Il faut continuer à poser des questions” patient reported outcome measures in cystic fibrosis: An anthropological perspective
- Author
-
Bend, Jutta, Bresnihan, Claire, Calvert, Anne, Fonts, Anna, Hager, Andreas, Hautrive, Maxime, Havermans, Trudy, Hofmann, Diana, Coucke, Rosa, Chansard, Audrey, Bontemps, Véronique, Grenet, Dominique, Hubert, Dominique, Martin, Clémence, Lammertyn, Elise, Bardin, Emmanuelle, Bulteel, Veerle, Chedevergne, Frédérique, Bourgeois, Muriel Le, Burgel, Pierre-Régis, Honore, Isabelle, de Keyser, Hilde, Kirszenbaum, Maya, de Carli, Paola, Sermet-Gaudelus, Isabelle, and Hayes, Kate
- Published
- 2021
- Full Text
- View/download PDF
34. Incidence of SARS-CoV-2 in people with cystic fibrosis in Europe between February and June 2020
- Author
-
Bobrovnichy, Vladimir, Bambir, Ivan, Vukic, Andrea Dugac, Drevinek, Pavel, Jr, Milan Macek, Corvol, Harriet, Lemonnier-Videau, Lydie, Hatziagorou, Elpis, Fletcher, Godfrey, Padoan, Rita, Gulmans, Vincent, Bakkeheim, Egil, Kondratyeva, Elena, Amelina, Elena, Zhekaite, Elena, Simonova, Olga, Pastor-Vivero, Maria Dolores, Lindblad, Anders, Gökdemir, Yasemin, Pekcan, Sevgi, Brownlee, Keith, McClenaghan, Elliott, Carr, Siobhán, Lammertyn, Elise, Zolin, Anna, Fox, Alice, Krasnyk, Marko, Van Rens, Jacqui, Naehrlich, Lutz, Orenti, Annalisa, Dunlevy, Fiona, Kasmi, Irena, Harutyunyan, Satenik, Pfleger, Andreas, Keegan, Svetlana, Daneau, Géraldine, Petrova, Guergana, Tješić-Drinković, Duška, Yiallouros, Panayiotis, Bilkova, Alena, Olesen, Hanne Vebert, Burgel, Pierre-Régis, Parulava, Tsitsino, Diamantea, Filia, Párniczky, Andrea, McKone, Edward F, Mei-Zahav, Meir, Salvatore, Marco, Colombo, Carla, Aleksejeva, Elina, Malakauskas, Kestutis, Schlesser, Marc, Fustik, Stojka, Turcu, Oxana, Zomer-van Ommen, Domenique, Wathne, Anita Senstad, Woźniacki, Łukasz, Pereira, Luísa, Pop, Liviu, Kashirskaya, Nataliya, Rodić, Milan, Kayserova, Hana, Krivecs, Uro, Mondejar-Lopez, Pedro, de Monestrol, Isabelle, Dogru, Deniz, Makukh, Halyna, Cosgriff, Rebecca, van Koningsbruggen-Rietschel, Silke, and Jung, Andreas
- Published
- 2021
- Full Text
- View/download PDF
35. 5. Le coronavirus dans le Grand Paris
- Author
-
Burgel, Guy, primary, Ghirardi, Raymond, additional, Schirrer, Maxime, additional, and Burgel, Pierre-Régis, additional
- Published
- 2021
- Full Text
- View/download PDF
36. Clinical response to lumacaftor-ivacaftor in patients with cystic fibrosis according to baseline lung function
- Author
-
Burgel, Pierre-Régis, Durieu, Isabelle, Chiron, Raphaël, Mely, Laurent, Prevotat, Anne, Murris-Espin, Marlene, Porzio, Michele, Abely, Michel, Reix, Philippe, Marguet, Christophe, Macey, Julie, Sermet-Gaudelus, Isabelle, Corvol, Harriet, Bui, Stéphanie, Biouhee, Tiphaine, Hubert, Dominique, Munck, Anne, Lemonnier, Lydie, Dehillotte, Clémence, Silva, Jennifer Da, Paillasseur, Jean-Louis, and Martin, Clémence
- Published
- 2021
- Full Text
- View/download PDF
37. 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
- Full Text
- View/download PDF
38. Clinical characteristics of SARS-CoV-2 infection in children with cystic fibrosis: An international observational study
- Author
-
Bain, Robert, Cosgriff, Rebecca, Zampoli, Marco, Elbert, Alexander, Burgel, Pierre-Régis, Carr, Siobhán B, Castaños, Claudio, Colombo, Carla, Corvol, Harriet, Faro, Albert, Goss, Christopher H, Gutierrez, Hector, Jung, Andreas, Kashirskaya, Nataliya, Marshall, Bruce C, Melo, Joel, Mondejar-Lopez, Pedro, de Monestrol, Isabelle, Naehrlich, Lutz, Padoan, Rita, Pastor-Vivero, Maria Dolores, Rizvi, Samar, Salvatore, Marco, Filho, Luiz Vicente Ribeiro Ferreira da Silva, Brownlee, Keith G, Haq, Iram J, and Brodlie, Malcolm
- Published
- 2021
- Full Text
- View/download PDF
39. Standards for the care of people with cystic fibrosis (CF); recognising and addressing CF health issues
- Author
-
MMB, Infection & Immunity, Burgel, Pierre Régis, Southern, Kevin W., Addy, Charlotte, Battezzati, Alberto, Berry, Claire, Bouchara, Jean Philippe, Brokaar, Edwin, Brown, Whitney, Azevedo, Pilar, Durieu, Isabelle, Ekkelenkamp, Miquel, Finlayson, Felicity, Forton, Julian, Gardecki, Johanna, Hodkova, Pavla, Hong, Gina, Lowdon, Jacqueline, Madge, Su, Martin, Clémence, McKone, Edward, Munck, Anne, Ooi, Chee Y., Perrem, Lucy, Piper, Amanda, Prayle, Andrew, Ratjen, Felix, Rosenfeld, Margaret, Sanders, Don B., Schwarz, Carsten, Taccetti, Giovanni, Wainwright, Claire, West, Natalie E., Wilschanski, Michael, Bevan, Amanda, Castellani, Carlo, Drevinek, Pavel, Gartner, Silvia, Gramegna, Andrea, Lammertyn, Elise, Landau, Eddie (Edwina) C., Plant, Barry J., Smyth, Alan R., van Koningsbruggen-Rietschel, Silke, Middleton, Peter G., MMB, Infection & Immunity, Burgel, Pierre Régis, Southern, Kevin W., Addy, Charlotte, Battezzati, Alberto, Berry, Claire, Bouchara, Jean Philippe, Brokaar, Edwin, Brown, Whitney, Azevedo, Pilar, Durieu, Isabelle, Ekkelenkamp, Miquel, Finlayson, Felicity, Forton, Julian, Gardecki, Johanna, Hodkova, Pavla, Hong, Gina, Lowdon, Jacqueline, Madge, Su, Martin, Clémence, McKone, Edward, Munck, Anne, Ooi, Chee Y., Perrem, Lucy, Piper, Amanda, Prayle, Andrew, Ratjen, Felix, Rosenfeld, Margaret, Sanders, Don B., Schwarz, Carsten, Taccetti, Giovanni, Wainwright, Claire, West, Natalie E., Wilschanski, Michael, Bevan, Amanda, Castellani, Carlo, Drevinek, Pavel, Gartner, Silvia, Gramegna, Andrea, Lammertyn, Elise, Landau, Eddie (Edwina) C., Plant, Barry J., Smyth, Alan R., van Koningsbruggen-Rietschel, Silke, and Middleton, Peter G.
- Published
- 2024
40. Bronchiectasis and asthma:Data from the European Bronchiectasis Registry (EMBARC)
- Author
-
Polverino, Eva, Dimakou, Katerina, Traversi, Letizia, Bossios, Apostolos, Haworth, Charles S., Loebinger, Michael R., De Soyza, Anthony, Vendrell, Montserrat, Burgel, Pierre Régis, Mertsch, Pontus, McDonnell, Melissa, Škrgat, Sabina, Maiz Carro, Luis, Sibila, Oriol, van der Eerden, Menno, Kauppi, Paula, Hill, Adam T., Wilson, Robert, Milenkovic, Branislava, Menendez, Rosario, Murris, Marlene, Digalaki, Tonia, Crichton, Megan L., Borecki, Sermin, Obradovic, Dusanka, Nowinski, Adam, Amorim, Adelina, Torres, Antoni, Lorent, Natalie, Welte, Tobias, Blasi, Francesco, Van Braeckel, Eva, Altenburg, Josje, Shoemark, Amelia, Shteinberg, Michal, Boersma, Wim, Elborn, J. Stuart, Aliberti, Stefano, Ringshausen, Felix C., Chalmers, James D., Goeminne, Pieter C., Polverino, Eva, Dimakou, Katerina, Traversi, Letizia, Bossios, Apostolos, Haworth, Charles S., Loebinger, Michael R., De Soyza, Anthony, Vendrell, Montserrat, Burgel, Pierre Régis, Mertsch, Pontus, McDonnell, Melissa, Škrgat, Sabina, Maiz Carro, Luis, Sibila, Oriol, van der Eerden, Menno, Kauppi, Paula, Hill, Adam T., Wilson, Robert, Milenkovic, Branislava, Menendez, Rosario, Murris, Marlene, Digalaki, Tonia, Crichton, Megan L., Borecki, Sermin, Obradovic, Dusanka, Nowinski, Adam, Amorim, Adelina, Torres, Antoni, Lorent, Natalie, Welte, Tobias, Blasi, Francesco, Van Braeckel, Eva, Altenburg, Josje, Shoemark, Amelia, Shteinberg, Michal, Boersma, Wim, Elborn, J. Stuart, Aliberti, Stefano, Ringshausen, Felix C., Chalmers, James D., and Goeminne, Pieter C.
- Abstract
Background: Asthma is commonly reported in patients with a diagnosis of bronchiectasis. Objective: The aim of this study was to evaluate whether patients with bronchiectasis and asthma (BE+A) had a different clinical phenotype and different outcomes compared with patients with bronchiectasis without concomitant asthma. Methods: A prospective observational pan-European registry (European Multicentre Bronchiectasis Audit and Research Collaboration) enrolled patients across 28 countries. Adult patients with computed tomography–confirmed bronchiectasis were reviewed at baseline and annual follow-up visits using an electronic case report form. Asthma was diagnosed by the local investigator. Follow-up data were used to explore differences in exacerbation frequency between groups using a negative binomial regression model. Survival analysis used Cox proportional hazards regression. Results: Of 16,963 patients with bronchiectasis included for analysis, 5,267 (31.0%) had investigator-reported asthma. Patients with BE+A were younger, were more likely to be female and never smokers, and had a higher body mass index than patients with bronchiectasis without asthma. BE+A was associated with a higher prevalence of rhinosinusitis and nasal polyps as well as eosinophilia and Aspergillus sensitization. BE+A had similar microbiology but significantly lower severity of disease using the bronchiectasis severity index. Patients with BE+A were at increased risk of exacerbation after adjustment for disease severity and multiple confounders. Inhaled corticosteroid (ICS) use was associated with reduced mortality in patients with BE+A (adjusted hazard ratio 0.78, 95% CI 0.63-0.95) and reduced risk of hospitalization (rate ratio 0.67, 95% CI 0.67-0.86) compared with control subjects without asthma and not receiving ICSs. Conclusions: BE+A was common and was associated with an increased risk of exacerbations and improved outcomes with ICS use. Unexpectedly we identified significantly lower
- Published
- 2024
41. Considerations for the use of inhaled antibiotics for Pseudomonas aeruginosa in people with cystic fibrosis receiving CFTR modulator therapy
- Author
-
Longziekten, Child Health, Infection & Immunity, Burgel, Pierre-Régis, Ballmann, Manfred, Drevinek, Pavel, Heijerman, Harry, Jung, Andreas, Mainz, Jochen G, Peckham, Daniel, Plant, Barry J, Schwarz, Carsten, Taccetti, Giovanni, Smyth, Alan, Longziekten, Child Health, Infection & Immunity, Burgel, Pierre-Régis, Ballmann, Manfred, Drevinek, Pavel, Heijerman, Harry, Jung, Andreas, Mainz, Jochen G, Peckham, Daniel, Plant, Barry J, Schwarz, Carsten, Taccetti, Giovanni, and Smyth, Alan
- Published
- 2024
42. Standards for the care of people with cystic fibrosis; establishing and maintaining health
- Author
-
Southern, Kevin W; https://orcid.org/0000-0001-6516-9083, Addy, Charlotte, Bell, Scott C; https://orcid.org/0000-0001-8651-7139, Bevan, Amanda, Borawska, Urzula, Brown, Catherine, Burgel, Pierre-Régis, Button, Brenda, Castellani, Carlo, Chansard, Audrey, Chilvers, Mark, Davies, Gwyneth, Davies, Jane C, De Boeck, Kris, Declercq, Dimitri, Doumit, Michael, Drevinek, Pavel, Fajac, Isabelle, Gartner, Silvia, Georgiopoulos, Anna M, Gursli, Sandra, Gramegna, Andrea, Hansen, Carina M E, Hug, Martin J, Lammertyn, Elise, Landau, Edwina (Eddie) C, Langley, Ross, Mayer-Hamblett, Nicole, Middleton, Anna, Middleton, Peter G, Radtke, Thomas; https://orcid.org/0000-0002-1723-1070, et al, Southern, Kevin W; https://orcid.org/0000-0001-6516-9083, Addy, Charlotte, Bell, Scott C; https://orcid.org/0000-0001-8651-7139, Bevan, Amanda, Borawska, Urzula, Brown, Catherine, Burgel, Pierre-Régis, Button, Brenda, Castellani, Carlo, Chansard, Audrey, Chilvers, Mark, Davies, Gwyneth, Davies, Jane C, De Boeck, Kris, Declercq, Dimitri, Doumit, Michael, Drevinek, Pavel, Fajac, Isabelle, Gartner, Silvia, Georgiopoulos, Anna M, Gursli, Sandra, Gramegna, Andrea, Hansen, Carina M E, Hug, Martin J, Lammertyn, Elise, Landau, Edwina (Eddie) C, Langley, Ross, Mayer-Hamblett, Nicole, Middleton, Anna, Middleton, Peter G, Radtke, Thomas; https://orcid.org/0000-0002-1723-1070, and et al
- Published
- 2024
43. Ceftolozane/Tazobactam for the Treatment of Adults With Cystic Fibrosis: Results From a French Prospective Cohort Study.
- Author
-
Burgel, Pierre-Régis, Bourge, Xavier, Mackosso, Carole, and Parquin, Francois
- Subjects
- *
MULTIDRUG resistance in bacteria , *CYSTIC fibrosis , *SERRATIA marcescens , *GRAM-negative bacteria , *LUNG transplantation - Abstract
Background People with cystic fibrosis (pwCF) are particularly susceptible to respiratory infections, including those caused by multidrug-resistant (MDR) pathogens. Ceftolozane/tazobactam (C/T) is an antibacterial agent combination active against MDR gram-negative bacteria that has shown promising results in isolates from pwCF. This subanalysis is the first extensive observation of real-world C/T use in pwCF. Methods The multicenter observational CONDUCT study included consecutive patients, some with cystic fibrosis, who received ≥1 dose of C/T at 28 centers throughout France. Patients were treated according to hospital standards and followed up until the end of C/T treatment (EOT). Results Among 260 patients who had received ≥1 dose of C/T, 63 were pwCF, including 12 with previous lung transplant. The median age was 34 years and 55.6% of patients were female. Pseudomonas aeruginosa was the most frequently isolated pathogen (n = 40/41 [97.6%]). Most tested P aeruginosa strains (n = 65/73 [91.5%]) and all other isolated strains (Escherichia coli , Citrobacter koseri , Proteus mirabilis , and Serratia marcescens) were susceptible to C/T. Most patients completed the treatment duration, including those with historical β-lactam hypersensitivity. Reasons for stopping treatment were planned EOT and improvement in condition; overall, 88.9% of patients (n = 56/63) experienced improvement in condition. No new safety signals were identified. Mean forced expiratory volume in 1 second improved from 1.33 L to 1.47 L before and after C/T treatment, respectively (n = 52; P =.057). Conclusions C/T treatment was well tolerated and effective in pwCF, including those with previous β-lactam hypersensitivity. [ABSTRACT FROM AUTHOR]
- Published
- 2024
- Full Text
- View/download PDF
44. Brensocatib in non-cystic fibrosis bronchiectasis: ASPEN protocol and baseline characteristics.
- Author
-
Chalmers, James D., Burgel, Pierre-Régis, Daley, Charles L., De Soyza, Anthony, Haworth, Charles S., Mauger, David, Mange, Kevin, Teper, Ariel, Fernandez, Carlos, Conroy, Dan, and Metersky, Mark
- Published
- 2024
- Full Text
- View/download PDF
45. The Association between Bronchiectasis and Chronic Obstructive Pulmonary Disease: Data from the European Bronchiectasis Registry (EMBARC).
- Author
-
Polverino, Eva, De Soyza, Anthony, Dimakou, Katerina, Traversi, Letizia, Bossios, Apostolos, Crichton, Megan L., Ringshausen, Felix C., Vendrell, Montserrat, Burgel, Pierre-Régis, Haworth, Charles S., Loebinger, Michael R., Lorent, Natalie, Pink, Isabell, McDonnell, Melissa, Skrgat, Sabina, Carro, Luis M., Sibila, Oriol, van der Eerden, Menno, Kauppi, Paula, and Shoemark, Amelia
- Subjects
BRONCHIECTASIS ,CHRONIC obstructive pulmonary disease - Abstract
Rationale: COPD and bronchiectasis are commonly reported together. Studies report varying impacts of co-diagnosis on outcomes, which may be related to different definitions of disease used across studies. Objectives: To investigate the prevalence of chronic obstructive pulmonary disease (COPD) associated with bronchiectasis and its relationship with clinical outcomes. We further investigated the impact of implementing the standardized ROSE criteria (radiological bronchiectasis [R], obstruction [FEV
1 /FVC ratio <0.7; O], symptoms [S], and exposure [⩾10 pack-years of smoking; E]), an objective definition of the association of bronchiectasis with COPD. Methods: Analysis of the EMBARC (European Bronchiectasis Registry), a prospective observational study of patients with computed tomography–confirmed bronchiectasis from 28 countries. The ROSE criteria were used to objectively define the association of bronchiectasis with COPD. Key outcomes during a maximum of 5 years of follow-up were exacerbations, hospitalization, and mortality. Measurements and Main Results: A total of 16,730 patients with bronchiectasis were included; 4,336 had a clinician-assigned codiagnosis of COPD, and these patients had more exacerbations, worse quality of life, and higher severity scores. We observed marked overdiagnosis of COPD: 22.2% of patients with a diagnosis of COPD did not have airflow obstruction and 31.9% did not have a history of ⩾10 pack-years of smoking. Therefore, 2,157 patients (55.4%) met the ROSE criteria for COPD. Compared with patients without COPD, patients who met the ROSE criteria had increased risks of exacerbations and exacerbations resulting in hospitalization during follow-up (incidence rate ratio, 1.25; 95% confidence interval, 1.15–1.35; vs. incidence rate ratio, 1.69; 95% confidence interval, 1.51–1.90, respectively). Conclusions: The label of COPD is often applied to patients with bronchiectasis who do not have objective evidence of airflow obstruction or a smoking history. Patients with a clinical label of COPD have worse clinical outcomes. [ABSTRACT FROM AUTHOR]- Published
- 2024
- Full Text
- View/download PDF
46. Multisystemic Effects of Elexacaftor–Tezacaftor–Ivacaftor in Adults with Cystic Fibrosis and Advanced Lung Disease.
- Author
-
Burgel, Pierre-Régis, Paillasseur, Jean-Louis, Durieu, Isabelle, Reynaud-Gaubert, Martine, Hamidfar, Rebecca, Murris-Espin, Marlène, Danner-Boucher, Isabelle, Chiron, Raphaël, Leroy, Sylvie, Douvry, Benoit, Grenet, Dominique, Mely, Laurent, Ramel, Sophie, Montcouquiol, Sylvie, Burnet, Espérie, Ouaalaya, El Hassane, Sogni, Philippe, Da Silva, Jennifer, and Martin, Clémence
- Subjects
LUNGS ,CYSTIC fibrosis ,LUNG diseases ,PULMONARY fibrosis ,HEPATIC fibrosis ,FORCED expiratory volume - Abstract
Rationale: Limited data exist on the safety and effectiveness of elexacaftor-tezacaftor-ivacaftor (ETI) in people with cystic fibrosis (pwCF) and advanced lung disease. Objectives: To evaluate the effects of ETI in an unselected population of pwCF and advanced lung disease. Methods: A prospective observational study, including all adults aged 18 years and older with percentage predicted forced expiratory volume in 1 second (ppFEV
1 ) ⩽ 40 who initiated ETI from December 2019 to June 2021 in France, was conducted. PwCF were followed until August 8, 2022. Results: ETI was initiated in 434 pwCF with a median ppFEV1 of 30 (interquartile range, 25–35), including 27 with severe cystic fibrosis liver disease and 183 with diabetes. PwCF were followed for a median of 587 (interquartile range, 396–728) days after ETI initiation. Discontinuation of ETI occurred in 12 (2.8%) pwCF and was due mostly to lung transplantation (n = 5) or death (n = 4). Absolute increase in ppFEV1 by a mean of +14.2% (95% confidence interval, 13.1–15.4%) occurred at 1 month and persisted throughout the study. Increase in ppFEV1 in the youngest age quartile was almost twice that of the oldest quartile (P < 0.001); body mass index < 18.5 kg/m2 was found in 38.6% at initiation versus 11.3% at 12 months (P = 0.0001). Increases in serum concentrations of vitamins A and E, but not 25-hydroxy vitamin D3 , were observed. Significant reductions in the percentages of pwCF using oxygen therapy, noninvasive ventilation, nutritional support, and inhaled and systemic therapies (including antibiotics) were observed; insulin was discontinued in 12% of patients with diabetes. Conclusions: ETI is safe in pwCF and advanced lung disease, with multisystem pulmonary and extrapulmonary benefits. [ABSTRACT FROM AUTHOR]- Published
- 2024
- Full Text
- View/download PDF
47. Relationships between symptoms and lung function in asthma and/or chronic obstructive pulmonary disease in a real-life setting: the NOVEL observational longiTudinal studY.
- Author
-
Papi, Alberto, Hughes, Rod, del Olmo, Ricardo, Agusti, Alvar, Chipps, Bradley E., Make, Barry, Tomaszewski, Erin, Peres Da Costa, Keith, Srivastava, Divyansh, Vestbo, Jørgen, Janson, Christer, Burgel, Pierre-Régis, and Price, David
- Subjects
CHRONIC obstructive pulmonary disease ,COUGH ,WHEEZE ,LUNGS ,ASTHMA ,MEDICAL care ,SYMPTOMS - Abstract
Background: The relationships between spirometric assessment of lung function and symptoms (including exacerbations) in patients with asthma and/or chronic obstructive pulmonary disease (COPD) in a real-life setting are uncertain. Objectives: To assess the relationships between baseline post-bronchodilator (post-BD) spirometry measures of lung function and symptoms and exacerbations in patients with a physician-assigned diagnosis of asthma and/or COPD. Design: The NOVEL observational longiTudinal studY (NOVELTY) is a global, prospective, 3-year observational study. Methods: Logistic regression analysis was used to evaluate relationships. Spirometry measures were assessed as percent predicted (%pred). Symptoms were assessed at baseline, and exacerbations were assessed at baseline and Year 1. Results: A total of 11,181 patients in NOVELTY had spirometry data (asthma, n = 5903; COPD, n = 3881; asthma + COPD, n = 1397). A 10% lower post-BD %pred forced expiratory volume in 1 s (FEV
1 ) and forced vital capacity (FVC) – adjusted for age and sex – were significantly associated with dyspnea (modified Medical Research Council ⩾ grade 2), frequent breathlessness [St George's Respiratory Questionnaire (SGRQ)], frequent wheeze attacks (SGRQ), nocturnal awakening (Respiratory Symptoms Questionnaire; ⩾1 night/week), and frequent productive cough (SGRQ). Lower post-BD %pred FEV1 and, to a lesser extent, lower post-BD %pred FVC were significantly associated with ⩾1 physician-reported exacerbation at baseline or Year 1. This association was stronger in patients with COPD than in those with asthma. Conclusion: In a real-life setting, reduced lung function is consistently associated with symptoms in patients with asthma, COPD, or asthma + COPD. The relationship with exacerbations is stronger in COPD only than in asthma. Trail registration: clinicaltrials.gov identifier: NCT02760329 (www.clinicaltrials.gov). Plain Language Summary: Relationships between symptoms and lung function in asthma and/or chronic obstructive pulmonary disease in a study performed in a real-life setting: the NOVELTY study Background: Asthma and chronic obstructive pulmonary disease (COPD) have many symptoms in common. To confirm diagnosis, doctors use spirometry, a test to measure the amount of air that can be breathed out from the lungs and how fast it can be blown out. The relationship between these measurements and symptoms in asthma and COPD is not well understood. Objectives: The aim of this research is to describe the characteristics, treatment, and impact of asthma and/or COPD in patients who are receiving their usual medical care. Methods: NOVELTY is a large study of around 12,000 patients across 19 countries. This analysis of NOVELTY looked at the relationships between two spirometry measurements and the symptoms of asthma and/or COPD experienced by patients. The spirometry measurements were: - forced expiratory volume in 1 second (FEV1 ) – the amount of air that can be blown out of the lungs in 1 second - forced vital capacity (FVC) – the amount of air that can be forcibly breathed out from the lungs after taking the deepest breath possible Results: The lower the FEV1 and FVC, the more common the symptoms of breathlessness, wheeze attacks, night-time awakening, and coughing up of phlegm or mucus. These relationships were similar for FEV1 and FVC. Lower FEV1 was more strongly associated with worse symptoms in COPD than in asthma. Conclusion: These findings help to improve our understanding of the relationships between spirometry measures and symptoms in patients with asthma and/or COPD. [ABSTRACT FROM AUTHOR]- Published
- 2024
- Full Text
- View/download PDF
48. Sex differences between women and men with COPD: A new analysis of the 3CIA study
- Author
-
Perez, Tamara Alonso, Castillo, Elena García, Ancochea, Julio, Pastor Sanz, María Teresa, Almagro, Pere, Martínez-Camblor, Pablo, Miravitlles, Marc, Rodríguez-Carballeira, Mónica, Navarro, Annie, Lamprecht, Bernd, Ramírez-García Luna, Ana S., Kaiser, Bernhard, Alfageme, Inmaculada, Casanova, Ciro, Esteban, Cristóbal, Soler-Cataluña, Juan J., De-Torres, Juan P., Celli, Bartolomé R., Marin, Jose M., Lopez-Campos, Jose L., Riet, Gerben Ter, Sobradillo, Patricia, Lange, Peter, Garcia-Aymerich, Judith, Anto, Josep M., Turner, Alice M., Han, MeiLan K., Langhammer, Arnulf, Sternberg, Alice, Leivseth, Linda, Bakke, Per, Johannessen, Ane, Oga, Toru, Cosío, Borja, Echazarreta, Andres, Roche, Nicolas, Burgel, Pierre-Régis, Sin, Don D., Puhan, Milo A., and Soriano, Joan B.
- Published
- 2020
- Full Text
- View/download PDF
49. Antimicrobial resistance in cystic fibrosis: A Delphi approach to defining best practices
- Author
-
Zemanick, Edith, Burgel, Pierre–Régis, Taccetti, Giovanni, Holmes, Alison, Ratjen, Felix, Byrnes, Catherine A., Waters, Valerie J., Bell, Scott C., VanDevanter, Donald R., Stuart Elborn, J., and Flume, Patrick A.
- Published
- 2020
- Full Text
- View/download PDF
50. The future of cystic fibrosis care: a global perspective
- Author
-
Bell, Scott C, Mall, Marcus A, Gutierrez, Hector, Macek, Milan, Madge, Susan, Davies, Jane C, Burgel, Pierre-Régis, Tullis, Elizabeth, Castaños, Claudio, Castellani, Carlo, Byrnes, Catherine A, Cathcart, Fiona, Chotirmall, Sanjay H, Cosgriff, Rebecca, Eichler, Irmgard, Fajac, Isabelle, Goss, Christopher H, Drevinek, Pavel, Farrell, Philip M, Gravelle, Anna M, Havermans, Trudy, Mayer-Hamblett, Nicole, Kashirskaya, Nataliya, Kerem, Eitan, Mathew, Joseph L, McKone, Edward F, Naehrlich, Lutz, Nasr, Samya Z, Oates, Gabriela R, O'Neill, Ciaran, Pypops, Ulrike, Raraigh, Karen S, Rowe, Steven M, Southern, Kevin W, Sivam, Sheila, Stephenson, Anne L, Zampoli, Marco, and Ratjen, Felix
- Published
- 2020
- Full Text
- View/download PDF
Catalog
Discovery Service for Jio Institute Digital Library
For full access to our library's resources, please sign in.