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3. 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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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
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5. 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. 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
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10. 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
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11. 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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12. Insufficient Pulmonary Rehabilitation Uptake After Severe Exacerbation of COPD: A Multicentre Study in the South West Region of France

Author

Gueçamburu,Marina, Verdy,Guillaume, Cuadros,Julie, Nocent-Ejnaini,Cécilia, Macey,Julie, Portel,Laurent, Rapin,Amandine, Zysman,Maéva, Gueçamburu,Marina, Verdy,Guillaume, Cuadros,Julie, Nocent-Ejnaini,Cécilia, Macey,Julie, Portel,Laurent, Rapin,Amandine, and Zysman,Maéva

Abstract

Marina Gueçamburu,1– 3 Guillaume Verdy,4 Julie Cuadros,1 Cécilia Nocent-Ejnaini,2 Julie Macey,1 Laurent Portel,3 Amandine Rapin,5,6 Maéva Zysman1,7 1Service des Maladies Respiratoires, CHU Bordeaux, Pessac, 33604, France; 2Service de pneumologie, Centre Hospitalier de la Côte Basque, Bayonne, France; 3Service de pneumologie, Centre Hospitalier Robert Boulin, Libourne, France; 4Unité d’Informatique et d’Archivistique Médicales, Service d’Information Médicale, CHU Bordeaux, Pessac, 33604, France; 5Université de Reims Champagne-Ardenne, VieFra, Reims, F-51100, France; 6CHU de Reims, Unité de Médecine Physique et de Réadaptation, Reims, F-51100, France; 7Univ-Bordeaux, Centre de Recherche cardio-thoracique de Bordeaux, U1045, CIC 1401, Pessac, F-33604, FranceCorrespondence: Marina Gueçamburu, Service des Maladies Respiratoires, CHU Bordeaux, Pessac, 33604, France, Email marina.guecamburu@live.frPurpose: Pulmonary rehabilitation (PR) is a type of multidisciplinary care strongly recommended after severe exacerbation of chronic obstructive pulmonary disease (COPD). Recently, a national French study reported a very low rate of PR uptake (8.6%); however, important clinical data were missing. Here, we aimed to identify the main factors associated with insufficient PR uptake after hospitalisation for COPD exacerbation.Patients and Methods: This multicentre retrospective study included patients hospitalised with COPD exacerbation between 1 January 2017 and 31 December 2018, as identified by both coding and a detailed review of medical records. PR was defined as inpatient care in a specialised centre or unit within 90 days of discharge. Multivariate logistic regression was used to identify associations between PR uptake and patient characteristics, such as comorbidities, non-invasive ventilation (NIV), inhaled treatment, and forced expiratory volume in 1 second (FEV1).Results: Among the 325 patients admitted for severe COPD exacerbation, 92 (28.3%) underwent

Published
2024

14. Longitudinal Evaluation of Bronchial Changes in Cystic Fibrosis Patients Undergoing Elexacaftor/Tezacaftor/Ivacaftor Therapy Using Lung MRI With Ultrashort Echo‐Times.

Author

David, Mathieu, Benlala, Ilyes, Bui, Stephanie, Benkert, Thomas, Berger, Patrick, Laurent, François, Macey, Julie, and Dournes, Gael

Subjects
CYSTIC fibrosis, FORCED expiratory volume, MAGNETIC resonance imaging, LUNGS, INTRACLASS correlation, ECHO-planar imaging
Abstract

Background: Lung magnetic resonance imaging (MRI) with ultrashort echo‐times (UTE‐MRI) allows high‐resolution and radiation‐free imaging of the lung structure in cystic fibrosis (CF). In addition, the combination of elexacaftor/tezacaftor/ivacaftor (ETI) has improved CF clinical outcomes such as need for hospitalization. However, the effect on structural disease still needs longitudinal evaluation at high resolution. Purpose: To analyze the effects of ETI on lung structural alterations using UTE‐MRI, with a focus on bronchiectasis reversibility. Study Type: Retrospective. Population: Fifty CF patients (mean age 24.3 ± 9.2; 23 males). Field Strength/Sequence: 1.5 T, UTE‐MRI. Assessment: All subjects completed both UTE‐MRI and pulmonary function tests (PFTs) during two annual visits (M0 and M12), and 30 of them completed a CT scan. They initiated ETI treatment after M0 within a maximum of 3 months from the annual examinations. Three observers scored a clinical MRI Bhalla score on UTE‐MRI. Bronchiectasis reversibility was defined as a reduction in both outer and inner bronchial dimensions. Correlations were searched between the Bhalla score and PFT such as the forced expiratory volume in 1 second percentage predicted (FEV1%p). Statistical Tests: Comparison was assessed using the paired t‐test, correlation using the Spearman correlation test with a significance level of 0.05. Concordance and reproducibility were assessed using intraclass correlation coefficient (ICC). Results: There was a significant improvement in MRI Bhalla score after ETI treatment. UTE‐MRI demonstrated bronchiectasis reversibility in a subgroup of 18 out of 50 CF patients (36%). These patients with bronchiectasis reversibility were significantly younger, with lower severity of wall thickening but no difference in mucus plugging extent (P = 0.39) was found. The reproducibility of UTE‐MRI evaluations was excellent (ICC ≥ 0.95), was concordant with CT scan (N = 30; ICC ≥ 0.90) and significantly correlated to FEV1% at PFT at M0 (N = 50; r = 0.71) and M12 (N = 50; r = 0.72). Data Conclusion: UTE‐MRI is a reproducible tool for the longitudinal follow‐up of CF patients, allowing to quantify the response to ETI and demonstrating the reversibility of some structural alterations such as bronchiectasis in a substantial fraction of this study population. Level of Evidence: 4 Technical Efficacy: Stage 2 [ABSTRACT FROM AUTHOR]

Published
2024
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15. 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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18. Similarities and differences of interstitial lung disease associated with pathogenic variants in SFTPC and ABCA3 in adults.

Author

Diesler, Rémi, Legendre, Marie, Si‐Mohamed, Salim, Brillet, Pierre‐Yves, Wemeau, Lidwine, Manali, Effrosyni D., Gagnadoux, Frédéric, Hirschi, Sandrine, Lorillon, Gwenaël, Reynaud‐Gaubert, Martine, Bironneau, Vanessa, Blanchard, Elodie, Bourdin, Arnaud, Dominique, Stéphane, Justet, Aurélien, Macey, Julie, Marchand‐Adam, Sylvain, Morisse‐Pradier, Hélène, Nunes, Hilario, and Papiris, Spyros A.

Subjects
INTERSTITIAL lung diseases, IDIOPATHIC pulmonary fibrosis, PULMONARY fibrosis, ADULTS, COMPUTED tomography, LUNG transplantation
Abstract

Background and Objective: Variants in surfactant genes SFTPC or ABCA3 are responsible for interstitial lung disease (ILD) in children and adults, with few studies in adults. Methods: We conducted a multicentre retrospective study of all consecutive adult patients diagnosed with ILD associated with variants in SFTPC or ABCA3 in the French rare pulmonary diseases network, OrphaLung. Variants and chest computed tomography (CT) features were centrally reviewed. Results: We included 36 patients (median age: 34 years, 20 males), 22 in the SFTPC group and 14 in the ABCA3 group. Clinical characteristics were similar between groups. Baseline median FVC was 59% ([52–72]) and DLco was 44% ([35–50]). An unclassifiable pattern of fibrosing ILD was the most frequent on chest CT, found in 85% of patients, however with a distinct phenotype with ground‐glass opacities and/or cysts. Nonspecific interstitial pneumonia and usual interstitial pneumonia were the most common histological patterns in the ABCA3 group and in the SFTPC group, respectively. Annually, FVC and DLCO declined by 1.87% and 2.43% in the SFTPC group, respectively, and by 0.72% and 0.95% in the ABCA3 group, respectively (FVC, p = 0.014 and DLCO, p = 0.004 for comparison between groups). Median time to death or lung transplantation was 10 years in the SFTPC group and was not reached at the end of follow‐up in the ABCA3 group. Conclusion: SFTPC and ABCA3‐associated ILD present with a distinct phenotype and prognosis. A radiologic pattern of fibrosing ILD with ground‐glass opacities and/or cysts is frequently found in these rare conditions. [ABSTRACT FROM AUTHOR]

Published
2024
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19. Non‐Contrast‐Enhanced Functional Lung MRI to Evaluate Treatment Response of Allergic Bronchopulmonary Aspergillosis in Patients With Cystic Fibrosis: A Pilot Study.

Author

Benlala, Ilyes, Klaar, Rabea, Gaass, Thomas, Macey, Julie, Bui, Stéphanie, Senneville, Baudouin Denis De, Berger, Patrick, Laurent, François, Dournes, Gael, and Dinkel, Julien

Subjects
PULMONARY aspergillosis, FUNCTIONAL magnetic resonance imaging, CYSTIC fibrosis, PULMONARY function tests, INTRACLASS correlation
Abstract

Background: Allergic bronchopulmonary aspergillosis (ABPA) in cystic fibrosis (CF) patients is associated with severe lung damage and requires specific therapeutic management. Repeated imaging is recommended to both diagnose and follow‐up response to treatment of ABPA in CF. However, high risk of cumulative radiation exposure requires evaluation of free‐radiation techniques in the follow‐up of CF patients with ABPA. Purpose: To evaluate whether Fourier decomposition (FD) functional lung MRI can detect response to treatment of ABPA in CF patients. Study Type: Retrospective longitudinal. Population: Twelve patients (7M, median‐age:14 years) with CF and ABPA with pre‐ and post‐treatment MRI. Field Strength/Sequence: 2D‐balanced‐steady‐state free‐precession (bSSFP) sequence with FD at 1.5T. Assessment: Ventilation‐weighted (V) and perfusion‐weighted (Q) maps were obtained after FD processing of 2D‐coronal bSSFP time‐resolved images acquired before and 3–9 months after treatment. Defects extent was assessed on the functional maps using a qualitative semi‐quantitative score (0 = absence/negligible, 1 = <50%, 2 = >50%). Mean and coefficient of variation (CV) of the ventilation signal‐intensity (VSI) and the perfusion signal‐intensity (QSI) were calculated. Measurements were performed independently by three readers and averaged. Inter‐reader reproducibility of the measurements was assessed. Pulmonary function tests (PFTs) were performed within 1 week of both MRI studies as markers of the airflow‐limitation severity. Statistical Tests: Comparisons of medians were performed using the paired Wilcoxon‐test. Reproducibility was assessed using intraclass correlation coefficient (ICC). Correlations between MRI and PFT parameters were assessed using the Spearman‐test (rho correlation‐coefficient). A P‐value <0.05 was considered as significant. Results: Defects extent on both V and Q maps showed a significant reduction after ABPA treatment (4.25 vs. 1.92 for V‐defect‐score and 5 vs. 2.75 for Q‐defect‐score). VSI_mean was significantly increased after treatment (280 vs. 167). Qualitative analyses reproducibility showed an ICC > 0.90, while the ICCs of the quantitative measurements was almost perfect (>0.99). Changes in VSI_cv and QSI_cv before and after treatment correlated inversely with changes of FEV1%p (rho = −0.68 for both). Data Conclusion: Non‐contrast‐enhanced FD lung MRI has potential to reproducibly assess response to treatment of ABPA in CF patients and correlates with PFT obstructive parameters. Evidence Level: 4 Technical Efficacy: Stage 3 [ABSTRACT FROM AUTHOR]

Published
2024
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21. Theratyping cystic fibrosis patients to guide elexacaftor/tezacaftor/ivacaftor out-of-label prescription

Author

Dreano, Elise, primary, Burgel, Pierre Régis, additional, Hatton, Aurelie, additional, Bouazza, Naim, additional, Chevalier, Benoit, additional, Macey, Julie, additional, Leroy, Sylvie, additional, Durieu, Isabelle, additional, Weiss, Laurence, additional, Grenet, Dominique, additional, Stremler, Nathalie, additional, Ohlmann, Camille, additional, Reix, Philippe, additional, Porzio, Michele, additional, Roux Claude, Pauline, additional, Rémus, Natacha, additional, Douvry, Benoit, additional, Montcouquiol, Sylvie, additional, Cosson, Laure, additional, Mankikian, Julie, additional, Languepin, Jeanne, additional, Houdouin, Veronique, additional, Le Clainche, Laurence, additional, Guillaumot, Anne, additional, Pouradier, Delphine, additional, Tissot, Adrien, additional, Priou, Pascaline, additional, Mély, Laurent, additional, Chedevergne, Frederique, additional, Lebourgeois, Muriel, additional, Lebihan, Jean, additional, Martin, Clémence, additional, Zavala, Flora, additional, Da Silva, Jennifer, additional, Lemonnier, Lydie, additional, Kelly-Aubert, Mairead, additional, Golec, Anita, additional, Foucaud, Pierre, additional, Marguet, Christophe, additional, Edelman, Aleksander, additional, Hinzpeter, Alexandre, additional, de Carli, Paola, additional, Girodon, Emmanuelle, additional, Sermet-Gaudelus, Isabelle, additional, and Pranke, Iwona, additional

Published
2023
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22. Phenotypic characterization of interstitial lung disease associated with mutations in SFTPC and ABCA3 in adults

Author

Diesler, Rémi, primary, Legendre, Marie, additional, Si-Mohamed, Salim, additional, Brillet, Pierre-Yves, additional, Wemeau, Lidwine, additional, Manali, Effrosyni D, additional, Gagnadoux, Frédéric, additional, Gondouin, Anne, additional, Hirschi, Sandrine, additional, Lorillon, Gwenael, additional, Reynaud-Gaubert, Martine, additional, Bironneau, Vanessa, additional, Blanchard, Elodie, additional, Bourdin, Arnaud, additional, Dominique, Stéphane, additional, Justet, Aurélien, additional, Macey, Julie, additional, Marchand-Adam, Sylvain, additional, Morisse-Pradier, Hélène, additional, Nunes, Hilario, additional, Papiris, Spyros, additional, Traclet, Julie, additional, Crestani, Bruno, additional, Amsellem, Serge, additional, Nathan, Nadia, additional, Borie, Raphaël, additional, and Cottin, Vincent, additional

Published
2023
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23. Functional pattern of baseline lung allograft dysfunction after lung transplantation.

Author

Bon, Martin, primary, Verdy, Guillaume, additional, Bon, Claire, additional, Blanchard, Elodie, additional, Macey, Julie, additional, Golhen, Hadrien, additional, Boisselier, Clement, additional, Repusseau, Benjamin, additional, Seramondi, Régis, additional, Reynier, Patrick, additional, Bonnardel, Eline, additional, Pellerin, Christelle, additional, Ridolfo, Jerome, additional, Gallo, Eloise, additional, Khan, Pierre, additional, Roze, Hadrien, additional, Rodriguez, Arnaud, additional, Belaroussi, Yaniss, additional, Chevalier, Benjamin, additional, Delcambre, Frederic, additional, Thumerel, Matthieu, additional, Jougon, Jacques, additional, and Demant, Xavier, additional

Published
2023
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24. Non‐Contrast‐Enhanced Functional Lung MRI to Evaluate Treatment Response of Allergic Bronchopulmonary Aspergillosis in Patients With Cystic Fibrosis: A Pilot Study

Author

Benlala, Ilyes, primary, Klaar, Rabea, additional, Gaass, Thomas, additional, Macey, Julie, additional, Bui, Stéphanie, additional, Senneville, Baudouin Denis De, additional, Berger, Patrick, additional, Laurent, François, additional, Dournes, Gael, additional, and Dinkel, Julien, additional

Published
2023
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29. The French Compassionate Program of elexacaftor-tezacaftor-ivacaftor in people with cystic fibrosis with advanced lung disease and no F508delCFTRvariant

Author

Burgel, Pierre-Régis, primary, Sermet-Gaudelus, Isabelle, additional, Durieu, Isabelle, additional, Kanaan, Reem, additional, Macey, Julie, additional, Grenet, Dominique, additional, Porzio, Michele, additional, Coolen-Allou, Nathalie, additional, Chiron, Raphael, additional, Marguet, Christophe, additional, Douvry, Benoit, additional, Dufeu, Nadine, additional, Danner-Boucher, Isabelle, additional, Foucaud, Pierre, additional, Lemonnier, Lydie, additional, Girodon, Emmanuelle, additional, Da Silva, Jennifer, additional, and Martin, Clémence, additional

Published
2023
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32. ROHHAD syndrome without rapid-onset obesity: A diagnosis challenge

Author

Desse, Blandine, primary, Tran, Antoine, additional, Butori, Mathilde, additional, Marchal, Sarah, additional, Afanetti, Michael, additional, Barthélemy, Sébastien, additional, Bérard, Etienne, additional, Baechler, Elisabeth, additional, Debelleix, Stéphane, additional, Lampin, Marie-Emilie, additional, Macey, Julie, additional, Massenavette, Bruno, additional, Harvengt, Julie, additional, Trang, Ha, additional, and Giovannini-Chami, Lisa, additional

Published
2022
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33. Chronic lung allograft dysfunction is associated with an early increase of circulating cytotoxic CD4+CD57+ILT2+ T cells, selectively inhibited by the immune check-point HLA-G

Author

Brugière, Olivier, primary, Mouren, Domitille, additional, Trichereau, Julie, additional, Vallée, Alexandre, additional, Kuzniak, Isabelle, additional, Hirschi, Sandrine, additional, Renaud-Picard, Benjamin, additional, Reynaud-Gaubert, Martine, additional, Nieves, Ana, additional, Bunel, Vincent, additional, Messika, Jonathan, additional, Demant, Xavier, additional, Macey, Julie, additional, Le Pavec, Jérôme, additional, Dauriat, Gaëlle, additional, Saint-Raymond, Christel, additional, Falque, Loic, additional, Mornex, Jean-François, additional, Tissot, Adrien, additional, Foureau, Aurore, additional, Borgne Krams, Aurélie Le, additional, Bousseau, Véronique, additional, Magnan, Antoine, additional, Picard, Clément, additional, Roux, Antoine, additional, Carosella, Edgardo, additional, LeMaoult, Joel, additional, and Rouas-Freiss, Nathalie, additional

Published
2022
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34. Safety and pharmacokinetics of Roscovitine (Seliciclib) in cystic fibrosis patients chronically infected with Pseudomonas aeruginosa, a randomized, placebo-controlled study

Author

Meijer, Laurent, primary, Hery-Arnaud, Geneviève, additional, Leven, Cyril, additional, Nowak, Emmanuel, additional, Hillion, Sophie, additional, Renaudineau, Yves, additional, Durieu, Isabelle, additional, Chiron, Raphaël, additional, Prevotat, Anne, additional, Fajac, Isabelle, additional, Hubert, Dominique, additional, Murris-Espin, Marlène, additional, Huge, Sandrine, additional, Danner-Boucher, Isabelle, additional, Ravoninjatovo, Bruno, additional, Leroy, Sylvie, additional, Macey, Julie, additional, Urban, Thierry, additional, Rault, Gilles, additional, Mottier, Dominique, additional, and Berre, Rozenn Le, additional

Published
2022
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35. Artificial intelligence in CT for quantifying lung changes in the era of CFTR modulators

Author

Dournes, Gael, Hall, Chase, Willmering, Matthew, Brody, Alan, Macey, Julie, BUI, Stephanie, Denis De Senneville, Baudouin, Berger, Patrick, Laurent, François, Benlala, Ilyes, Woods, Jason, Centre de recherche Cardio-Thoracique de Bordeaux [Bordeaux] (CRCTB), Université Bordeaux Segalen - Bordeaux 2-CHU Bordeaux [Bordeaux]-Institut National de la Santé et de la Recherche Médicale (INSERM), CIC Bordeaux, Université Bordeaux Segalen - Bordeaux 2-Institut National de la Santé et de la Recherche Médicale (INSERM), CHU Bordeaux [Bordeaux], University of Kansas [Kansas City], Cincinnati Children's Hospital Medical Center, Hôpital Pellegrin, CHU Bordeaux [Bordeaux]-Groupe hospitalier Pellegrin, Biothérapies des maladies génétiques et cancers, Institut de Mathématiques de Bordeaux (IMB), Université Bordeaux Segalen - Bordeaux 2-Université Sciences et Technologies - Bordeaux 1 (UB)-Université de Bordeaux (UB)-Institut Polytechnique de Bordeaux (Bordeaux INP)-Centre National de la Recherche Scientifique (CNRS), Modélisation Mathématique pour l'Oncologie (MONC), Université Bordeaux Segalen - Bordeaux 2-Université Sciences et Technologies - Bordeaux 1 (UB)-Université de Bordeaux (UB)-Institut Polytechnique de Bordeaux (Bordeaux INP)-Centre National de la Recherche Scientifique (CNRS)-Université Bordeaux Segalen - Bordeaux 2-Université Sciences et Technologies - Bordeaux 1 (UB)-Université de Bordeaux (UB)-Institut Polytechnique de Bordeaux (Bordeaux INP)-Centre National de la Recherche Scientifique (CNRS)-Institut Bergonié [Bordeaux], UNICANCER-UNICANCER-Inria Bordeaux - Sud-Ouest, Institut National de Recherche en Informatique et en Automatique (Inria)-Institut National de Recherche en Informatique et en Automatique (Inria), University of Cincinnati (UC), ANR-10-IDEX-03-02, ANR-10-LABX-0057,TRAIL,Translational Research and Advanced Imaging Laboratory(2010), Université Bordeaux Segalen - Bordeaux 2-Université Sciences et Technologies - Bordeaux 1-Université de Bordeaux (UB)-Institut Polytechnique de Bordeaux (Bordeaux INP)-Centre National de la Recherche Scientifique (CNRS), Université Bordeaux Segalen - Bordeaux 2-Université Sciences et Technologies - Bordeaux 1-Université de Bordeaux (UB)-Institut Polytechnique de Bordeaux (Bordeaux INP)-Centre National de la Recherche Scientifique (CNRS)-Université Bordeaux Segalen - Bordeaux 2-Université Sciences et Technologies - Bordeaux 1-Université de Bordeaux (UB)-Institut Polytechnique de Bordeaux (Bordeaux INP)-Centre National de la Recherche Scientifique (CNRS)-Institut Bergonié [Bordeaux], and Denis De Senneville, Baudouin

Subjects
[SDV.IB.IMA] Life Sciences [q-bio]/Bioengineering/Imaging, [SDV.IB.IMA]Life Sciences [q-bio]/Bioengineering/Imaging, [SDV.MHEP.PSR] Life Sciences [q-bio]/Human health and pathology/Pulmonology and respiratory tract, [SPI.SIGNAL]Engineering Sciences [physics]/Signal and Image processing, [SDV.MHEP.PSR]Life Sciences [q-bio]/Human health and pathology/Pulmonology and respiratory tract
Abstract

International audience; Background: Chest computed tomography (CT) remains the imaging standard for demonstrating cystic fibrosis (CF) airway structural disease in vivo. However, visual scoring systems as an outcome measure are time consuming, require training and lack high reproducibility. Our objective was to validate a fully automated artificial intelligence (AI)-driven scoring system of CF lung disease severity.Methods: Data were retrospectively collected in three CF reference centres, between 2008 and 2020, in 184 patients aged 4-54 years. An algorithm using three 2D convolutional neural networks was trained with 78 patients' CT scans (23 530 CT slices) for the semantic labelling of bronchiectasis, peribronchial thickening, bronchial mucus, bronchiolar mucus and collapse/consolidation. 36 patients' CT scans (11 435 CT slices) were used for testing versus ground-truth labels. The method's clinical validity was assessed in an independent group of 70 patients with or without lumacaftor/ivacaftor treatment (n=10 and n=60, respectively) with repeat examinations. Similarity and reproducibility were assessed using the Dice coefficient, correlations using the Spearman test, and paired comparisons using the Wilcoxon rank test.Results: The overall pixelwise similarity of AI-driven versus ground-truth labels was good (Dice 0.71). All AI-driven volumetric quantifications had moderate to very good correlations to a visual imaging scoring (p0.99).Conclusion: AI allows fully automated volumetric quantification of CF-related modifications over an entire lung. The novel scoring system could provide a robust disease outcome in the era of effective CF transmembrane conductance regulator modulator therapy.Trial registration: ClinicalTrials.gov NCT04760548.

Published
2022

37. Cumulative Incidence and Risk Factors for Severe Coronavirus Disease 2019 in French People With Cystic Fibrosis.

Author

Corvol, Harriet, Miranda, Sandra de, Dehillotte, Clémence, Lemonnier, Lydie, Chiron, Raphael, Danner-Boucher, Isabelle, Hamidfar, Rebecca, Houdouin, Véronique, Macey, Julie, Marguet, Christophe, Murris-Espin, Marlène, Reynaud, Quitterie, Reix, Philippe, Gaubert, Martine Reynaud, Kemgang, Astrid, Burgel, Pierre-Régis, and Group, French Cystic Fibrosis Reference Network Study

Subjects
LUNG physiology, COVID-19, CONFIDENCE intervals, AGE distribution, PATIENTS, DIABETES, CYSTIC fibrosis, SEVERITY of illness index, SEX distribution, DESCRIPTIVE statistics, HOSPITAL care, FORCED expiratory volume, ODDS ratio, TRANSPLANTATION of organs, tissues, etc., DISEASE complications
Abstract

Background Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infections are closely monitored in people with cystic fibrosis (pwCF), especially severe cases. Previous studies used hospitalization rates as proxy for severity. Methods We evaluated data from coronavirus disease 2019 (COVID-19) cases diagnosed in French pwCF over the first pandemic year. Objective criteria were applied for defining severity (eg, respiratory failure and/or death). Data were compared to all French pwCF using the National Registry. Results As of 30 April 2021, 223 pwCF were diagnosed with COVID-19, with higher risks in adults (odds ratio [OR], 2.52 [95% confidence interval {CI}, 1.82−3.48]) and transplant recipients (OR, 2.68 [95% CI, 1.98–3.63]). Sixty (26.9%) patients were hospitalized, with increased risk in transplant recipients (OR, 4.74 [95% CI, 2.49–9.02]). In 34 (15%) cases, COVID-19 was considered severe; 28 (46.7%) hospitalizations occurred without objective criteria of severity. Severe cases occurred mostly in adult (85.3%) and posttransplant pwCF (61.8%; OR, 6.02 [95% CI, 2.77–13.06]). In nontransplanted pwCF, risk factors for severity included low lung function (median percentage of predicted forced expiratory volume in 1 second, 54.6% vs 75.1%; OR, 1.04 [95% CI, 1.01–1.08]) and CF-related diabetes (OR, 3.26 [95% CI, 1.02–10.4]). While 204 cases fully recovered, 16 were followed for possible sequelae, and 3 posttransplant females died. Conclusions Severe COVID-19 occurred infrequently during the first pandemic year in French pwCF. Nontransplanted adults with severe respiratory disease or diabetes and posttransplant individuals were at risk for severe COVID-19. Thus, specific preventive measures should be proposed. [ABSTRACT FROM AUTHOR]

Published
2022
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38. Identification and analysis of clinical phenotypes in COPD patients: PALOMB Cohort

Author

Ouaalaya, El-hassane, primary, Zysman, Maéva, additional, Berteaud, Émilie, additional, Falque, Laurent, additional, Monge, Emmanuel, additional, Nguyen, Laurent, additional, Ozier, Annaig, additional, Dupis, Jean Michel, additional, Sabatini, Marielle, additional, Nocent-Ejnaini, Cécilia, additional, Petrov, Laura, additional, Bernady, Alain, additional, Roy, Christophe, additional, Le Guillou, Frédéric, additional, Aliati, Mohammed, additional, Prudhomme, Anne, additional, Quinquenel, Marie Line, additional, Staali, Mohammed, additional, Pilard, Frederic, additional, Iglesias, Esther, additional, Sapène, Marc, additional, Casteigt, Julien, additional, Moinard, Jean, additional, Daoudi, Yannick, additional, Blanchard, Élodie, additional, Macey, Julie, additional, Veillon, Rémi, additional, Demant, Xavier, additional, Bon, Claire, additional, Grassion, Leo, additional, Molimard, Mathieu, additional, and Raherison-Semjen, Chantal, additional

Published
2021
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39. Late Breaking Abstract - Good outcome for lung transplantation for adults with interstitial lung disease associated with genetic disorders of surfactant metabolism

Author

Bermudez, Julien, primary, Nathan, Nadia, additional, Coiffard, Benjamin, additional, Roux, Antoine, additional, Hirschi, Sandrine, additional, Degot, Tristan, additional, Bunel, Vincent, additional, Le Pavec, Jérôme, additional, Macey, Julie, additional, Leborgne-Krams, Aurélie, additional, Cottin, Vincent, additional, Borie, Raphaël, additional, and Reynaud-Gaubert, Martine, additional

Published
2021
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41. 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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42. The Clinical Use of Lung MRI in Cystic Fibrosis

Author

Dournes, Gaël, primary, Walkup, Laura L., additional, Benlala, Ilyes, additional, Willmering, Matthew M., additional, Macey, Julie, additional, Bui, Stephanie, additional, Laurent, François, additional, and Woods, Jason C., additional

Published
2021
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43. The Clinical Use of Lung MRI in Cystic Fibrosis: What, Now, How?

Author

Dournes, Gael, Walkup, Laura L., Benlala, Ilyes, Willmering, Matthew M., Macey, Julie, Bui, Stephanie, Laurent, Francois, Woods, Jason C., Admin, Oskar, Centre de recherche Cardio-Thoracique de Bordeaux [Bordeaux] (CRCTB), Université Bordeaux Segalen - Bordeaux 2-CHU Bordeaux [Bordeaux]-Institut National de la Santé et de la Recherche Médicale (INSERM), CIC Bordeaux, Université Bordeaux Segalen - Bordeaux 2-Institut National de la Santé et de la Recherche Médicale (INSERM), CHU Bordeaux [Bordeaux], Cincinnati Children's Hospital Medical Center, University of Cincinnati (UC), Hôpital Pellegrin, and CHU Bordeaux [Bordeaux]-Groupe hospitalier Pellegrin

Subjects
CF, cystic fibrosis, TE, echo time, UTE, ultrashort echo time, Cystic Fibrosis, T1w, T1 weighted, [SPI] Engineering Sciences [physics], ZTE, zero echo time, Reproducibility of Results, imaging, Magnetic Resonance Imaging, T2w, T2 weighted, TR, repetition time, lung, PFT, pulmonary function test, magnetic resonance, [SPI]Engineering Sciences [physics], ABPA, allergic bronchopulmonary aspergillosis, HAM, high-attenuation mucus, Treatment Outcome, Humans, Chest Infections: CHEST Reviews, IMIS, inverted mucus impaction signal
Abstract

International audience; To assess airway and lung parenchymal damage noninvasively in cystic fibrosis (CF), chest MRI has been historically out of the scope of routine clinical imaging because of technical difficulties such as low proton density and respiratory and cardiac motion. However, technological breakthroughs have emerged that dramatically improve lung MRI quality (including signal-to-noise ratio, resolution, speed, and contrast). At the same time, novel treatments have changed the landscape of CF clinical care. In this contemporary context, there is now consensus that lung MRI can be used clinically to assess CF in a radiation-free manner and to enable quantification of lung disease severity. MRI can now achieve three-dimensional, high-resolution morphologic imaging, and beyond this morphologic information, MRI may offer the ability to sensitively differentiate active inflammation vs scarring tissue. MRI could also characterize various forms of inflammation for early guidance of treatment. Moreover, functional information from MRI can be used to assess regional, small-airway disease with sensitivity to detect small changes even in patients with mild CF. Finally, automated quantification methods have emerged to support conventional visual analyses for more objective and reproducible assessment of disease severity. This article aims to review the most recent developments of lung MRI, with a focus on practical application and clinical value in CF, and the perspectives on how these modern techniques may converge and impact patient care soon.

Published
2020

46. COPD phenotypes and 5-years mortality risk prediction: PALOMB Cohort

Author

Ouaalaya, El-hassane, primary, Zysman, Maéva, additional, Berteaud, Émilie, additional, Falque, Laurent, additional, Nguyen, Laurent, additional, Dupis, Jean Michel, additional, Sabatini, Marielle, additional, Bernady, Alain, additional, Ozier, Annaig, additional, Nocent-Ejnaini, Cécilia, additional, Roy, Christophe, additional, Le Guillou, Frédéric, additional, Monge, Emmanuel, additional, Aliati, Mohammed, additional, Prudhomme, Anne, additional, Petrov, Laura, additional, Quinquenel, Marie Line, additional, Staali, Mohammed, additional, Pilard, Frederic, additional, Sapène, Marc, additional, Casteigt, Julien, additional, Moinard, Jean, additional, Daoudi, Yannick, additional, Iglesias, Esther, additional, Blanchard, Élodie, additional, Macey, Julie, additional, Veillon, Rémi, additional, Demant, Xavier, additional, Bon, Claire, additional, Grassion, Leo, additional, Molimard, Mathieu, additional, and Raherison-Semjen, Chantal, additional

Published
2020
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49. 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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