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1. Lumacaftor/Ivacaftor Population Pharmacokinetics in Pediatric Patients with Cystic Fibrosis: A First Step Toward Personalized Therapy

Author

Bouazza, Naïm, Urien, Saïk, Foissac, Frantz, Choupeaux, Laure, Lui, Gabrielle, Froelicher Bournaud, Léo, Rouillon, Steeve, Zheng, Yi, Bardin, Emmanuelle, Stremler, Nathalie, Bessaci, Katia, Bihouee, Tiphaine, Coirier-Duet, Emmanuelle, Marguet, Christophe, Deneuville, Eric, Laurans, Muriel, Reix, Philippe, Gerardin, Michèle, Mittaine, Marie, Epaud, Ralph, Thumerelle, Caroline, Weiss, Laurence, Berthaud, Romain, Semeraro, Michaela, Treluyer, Jean-Marc, Benaboud, Sihem, and Sermet-Gaudelus, Isabelle

Published
2024
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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
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3. 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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4. Epidemiology of childhood interstitial lung disease in France: the RespiRare cohort.

Author

Fletcher, Camille, Hadchouel, Alice, Thumerelle, Caroline, Mazenq, Julie, Fleury, Manon, Corvol, Harriet, Jedidi, Nouha, Benhamida, Myriam, Bessaci, Katia, Bilhouee, Tiphaine, Borie, Raphael, Brouard, Jacques, Cantais, Aurélie, Clement, Annick, Coutier, Laurianne, Cisterne, Camille, Cros, Pierrick, Dalphin, Marie-Laure, Delacourt, Christophe, and Deneuville, Eric

Subjects
PULMONARY alveolar proteinosis, PRIMARY immunodeficiency diseases, NUCLEOTIDE sequencing, PULMONARY eosinophilia, INTERSTITIAL lung diseases, CHILD patients, EPIDEMIOLOGY
Published
2024
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5. 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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6. Can we decondition TRIKAFTA® tablets for the younger ones?

Author

Gabsi, Asma, primary, Benaboud, Sihem, additional, Pouradier, Delphine, additional, Reix, Philippe, additional, L'Excellent, Sophie, additional, Weiss, Laurence, additional, Le-Clainche, Laurence, additional, Dalphin, Marie-Laure, additional, Perisson, Caroline, additional, Deneuville, Eric, additional, Bonnel, Anne Sophie, additional, and Sermet-Gaudelus, Isabelle, additional

Published
2023
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7. Behavioural and sleep issues after initiation of elexacaftor–tezacaftor–ivacaftor in preschool-age children with cystic fibrosis

Author

Barboura, Mahassen, Barzic, Audrey, Benhalla, Lilia, Bennour, Khadidja, Bessaci, Katia, Bessou, Antoine, Bihouee, Thiphaine, Bonnel, Anne-Sophie, Bouleghem, Nesrine, Bui, Stéphanie, Chedevergne, Frédérique, Corvol, Harriet, Cosson, Laure, Couderc, Laure, Dalphin, Marie-Laure, De carli, Paola, Deneuville, Eric, Foucaud, Pierre, Gabsi, Asma, Gachelin, Elsa, Hassani, Fatiha, Houdouin, Veronique, Huet, Frédéric, Jamin, Marie, Kaba, Kadiatou, Labbe, Guillaume, Languepin, Jane, Laurans, Muriel, Lerena, Cathy, Letierce, Alexia, Livrozet, Clotilde, Marguet, Christophe, Mely, Laurent, Messaoudi, Rania, Mittaine, Marie, Perisson, Caroline, Piccini-bailly, Carole, Reix, Philippe, Remus, Natascha, Ronayette, Anna, Sahki, Djouher, Scalbert, Manuela, Sermet-Gaudelus, Isabelle, Socchi, Floriane, Stremler, Nathalie, Tatopoulos, Aurélie, Thouvenin, Guillaume, Troussier, Françoise, Weiss, Laurence, Wizla, Nathalie, Sermet-Gaudelus, I, Benaboud, Sihem, Bihouée, Tiphaine, and Gautier, Sophie

Published
2024
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8. Pneumocystis jirovecii and Cystic Fibrosis in Brittany, France

Author

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

Published
2017
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11. 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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13. Chest physiotherapy enhances detection of Pseudomonas aeruginosa in nonexpectorating children with cystic fibrosis

Author

Marguet, Christophe, primary, Houdouin, Véronique, additional, Pin, Isabelle, additional, Reix, Philippe, additional, Huet, Frédéric, additional, Mittaine, Marie, additional, Ramel, Sophie, additional, Wizla-Derambure, Nathalie, additional, Abely, Michel, additional, Dalphin, Marie-Laure, additional, Fayon, Michael, additional, Bihouée, Tiphaine, additional, Le Bourgeois, Muriel, additional, Deneuville, Eric, additional, Corvol, Harriet, additional, Laurans, Muriel, additional, Couderc, Laure, additional, Leroux, Evelyne, additional, and Lémée, Ludovic, additional

Published
2021
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14. 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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15. Clinical Phenotype and Genotype of Children with Borderline Sweat Test and Abnormal Nasal Epithelial Chloride Transport

Author

Sermet-Gaudelus, Isabelle, Girodon, Emanuelle, Sands, Dorota, Stremmler, Nathalie, Vavrova, Vera, Deneuville, Eric, Reix, Philippe, Bui, Stéphanie, Huet, Frédéric, Lebourgeois, Muriel, Munck, Anne, Iron, Albert, Skalicka, Veronika, Bienvenu, Thierry, Roussel, Delphine, Lenoir, Gérard, Bellon, Gabriel, Sarles, Jacques, Macek, Milan, Roussey, Michel, Fajac, Isabelle, and Edelman, Aleksander

Published
2010
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16. Measurement of nasal potential difference in young children with an equivocal sweat test following newborn screening for cystic fibrosis

Author

Sermet-Gaudelus, Isabelle, Girodon, Emmanuelle, Roussel, Delphine, Deneuville, Eric, Bui, Stéphanie, Huet, Frédéric, Guillot, Marcel, Aboutaam, Rola, Renouil, Michel, Munck, Anne, des Georges, Marie, Iron, Albert, Thauvin-Robinet, Christel, Fajac, Isabelle, Lenoir, Gerard, Roussey, Michel, and Edelman, Aleksander

Published
2010
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20. The CF-CIRC study: a French collaborative study to assess the accuracy of Cystic Fibrosis diagnosis in neonatal screening

Author

Bellon Gabriel, Reix Philippe, Huet Frédéric, Deneuville Eric, Bui Stéphanie, Roussel Delphine, Sermet-Gaudelus Isabelle, Lenoir Gérard, and Edelman Aleksander

Subjects
Pediatrics, RJ1-570
Abstract

Abstract Background Cystic fibrosis (CF) is caused by mutations in the gene encoding for the CF transmembrane conductance regulator (CFTR) protein, which acts as a chloride channel after activation by cyclic AMP (cAMP). Newborn screening programs for CF usually consist of an immunoreactive trypsinogen (IRT) assay, followed when IRT is elevated by testing for a panel of CF-causing mutations. Some children, however, may have persistent hypertrypsinogenemia, only one or no identified CFTR gene mutation, and sweat chloride concentrations close to normal values. In vivo demonstration of abnormal CFTR protein function would be an important diagnostic aid in this situation. Measurements of transepithelial nasal potential differences (NPD) in adults accurately characterize CFTR-related ion transport. The aim of the present study is to establish reference values for NPD measurements for healthy children and those with CF aged 3 months to 3 years, the age range of most difficult-to-diagnose patients with suspected CF. The ultimate goal of our study is to validate NPD testing as a diagnostic tool for children with borderline results in neonatal screening. Methods/Design We adapted the standard NPD protocol for young children, designed a special catheter for them, used a slower perfusion rate, and shortened the protocol to include only measurement of basal PD, transepithelial sodium (Na+) transport in response to the Na+ channel inhibitor amiloride, and CFTR-mediated chloride (Cl-) secretion in response to isoproterenol, a β-agonist in a Cl- free solution. The study will include 20 children with CF and 20 healthy control children. CF children will be included only if they carry 2 CF-causing mutations in the CFTR gene or have sweat chloride concentrations > 60 mEq/L or both. The healthy children will be recruited among the siblings of the CF patients, after verification that they do not carry the familial mutation. Discussion A preliminary study of 3 adult control subjects and 4 children older than 12 years with CF verified that the new protocol was well tolerated and produced NPD measurements that did not differ significantly from those obtained with the standard protocol. This preliminary study will provide a basis for interpreting NPD measurements in patients with suspected CF after neonatal screening. Earlier definitive diagnosis should alleviate parental distress and allow earlier therapeutic intervention and genetic counseling.

Published
2006
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23. Behavioural and sleep issues after initiation of elexacaftor–tezacaftor–ivacaftor in preschool-age children with cystic fibrosis

Author

Sermet-Gaudelus, I, Benaboud, Sihem, Bui, Stéphanie, Bihouée, Tiphaine, Gautier, Sophie, Barboura, Mahassen, Barzic, Audrey, Benhalla, Lilia, Bennour, Khadidja, Bessaci, Katia, Bessou, Antoine, Bihouee, Thiphaine, Bonnel, Anne-Sophie, Bouleghem, Nesrine, Bui, Stéphanie, Chedevergne, Frédérique, Corvol, Harriet, Cosson, Laure, Couderc, Laure, Dalphin, Marie-Laure, De carli, Paola, Deneuville, Eric, Foucaud, Pierre, Gabsi, Asma, Gachelin, Elsa, Hassani, Fatiha, Houdouin, Veronique, Huet, Frédéric, Jamin, Marie, Kaba, Kadiatou, Labbe, Guillaume, Languepin, Jane, Laurans, Muriel, Lerena, Cathy, Letierce, Alexia, Livrozet, Clotilde, Marguet, Christophe, Mely, Laurent, Messaoudi, Rania, Mittaine, Marie, Perisson, Caroline, Piccini-bailly, Carole, Reix, Philippe, Remus, Natascha, Ronayette, Anna, Sahki, Djouher, Scalbert, Manuela, Sermet-Gaudelus, Isabelle, Socchi, Floriane, Stremler, Nathalie, Tatopoulos, Aurélie, Thouvenin, Guillaume, Troussier, Françoise, Weiss, Laurence, and Wizla, Nathalie

Published
2024
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24. Lessons from a French collaborative case–control study in cystic fibrosis patients during the 2009 A/H1N1 influenza pandemy

Author

Bucher, Julie, primary, Boelle, Pierre-Yves, additional, Hubert, Dominique, additional, Lebourgeois, Muriel, additional, Stremler, Nathalie, additional, Durieu, Isabelle, additional, Bremont, François, additional, Deneuville, Eric, additional, Delaisi, Bertrand, additional, Corvol, Harriet, additional, Bassinet, Laurence, additional, Grenet, Dominique, additional, Remus, Natacha, additional, Vodoff, Marie Véronique, additional, Boussaud, Véronique, additional, Troussier, Françoise, additional, Leruez-Ville, Marianne, additional, Treluyer, Jean-Marc, additional, Launay, Odile, additional, and Sermet-Gaudelus, Isabelle, additional

Published
2015
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25. Pneumocystis jirovecii and Cystic Fibrosis in Brittany, France.

Author

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

Abstract

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

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

Author

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

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

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

Published
2010

28. Loss-of-Function Mutations in RSPH1 Cause Primary Ciliary Dyskinesia with Central-Complex and Radial-Spoke Defects

Author

Kott, Esther, primary, Legendre, Marie, additional, Copin, Bruno, additional, Papon, Jean-François, additional, Dastot-Le Moal, Florence, additional, Montantin, Guy, additional, Duquesnoy, Philippe, additional, Piterboth, William, additional, Amram, Daniel, additional, Bassinet, Laurence, additional, Beucher, Julie, additional, Beydon, Nicole, additional, Deneuville, Eric, additional, Houdouin, Véronique, additional, Journel, Hubert, additional, Just, Jocelyne, additional, Nathan, Nadia, additional, Tamalet, Aline, additional, Collot, Nathalie, additional, Jeanson, Ludovic, additional, Le Gouez, Morgane, additional, Vallette, Benoit, additional, Vojtek, Anne-Marie, additional, Epaud, Ralph, additional, Coste, André, additional, Clement, Annick, additional, Housset, Bruno, additional, Louis, Bruno, additional, Escudier, Estelle, additional, and Amselem, Serge, additional

Published
2013
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29. Lessons from a French collaborative case-control study in cystic fibrosis patients during the 2009 A/H1N1 influenza pandemy.

Author

Bucher, Julie, Boelle, Pierre-Yves, Hubert, Dominique, Lebourgeois, Muriel, Stremler, Nathalie, Durieu, Isabelle, Bremont, François, Deneuville, Eric, Delaisi, Bertrand, Corvol, Harriet, Bassinet, Laurence, Grenet, Dominique, Remus, Natacha, Vodoff, Marie Véronique, Boussaud, Véronique, Troussier, Françoise, Leruez-Ville, Marianne, Treluyer, Jean-Marc, Launay, Odile, and Sermet-Gaudelus, Isabelle

Subjects
H1N1 influenza, CYSTIC fibrosis, PSEUDOMONAS aeruginosa infections, SYMPTOMS, LUNG transplantation, BRONCHOPULMONARY dysplasia, PATIENTS, ANTIBIOTICS, INFLUENZA complications, SPUTUM microbiology, ANTIVIRAL agents, INFLUENZA epidemiology, OSELTAMIVIR, COMPARATIVE studies, EPIDEMICS, INFLUENZA, LONGITUDINAL method, RESEARCH methodology, MEDICAL cooperation, MEMBRANE proteins, GENETIC mutation, PSEUDOMONAS, PSEUDOMONAS diseases, RESEARCH, EVALUATION research, CASE-control method, INFLUENZA A virus, H1N1 subtype, DISEASE complications, THERAPEUTICS
Abstract

Background: Viral infections such as influenza are thought to impact respiratory parameters and to promote infection with Pseudomonas aeruginosa in patients with cystic fibrosis (CF). However, the real morbidity of the influenza virus in CF needs to be further investigated because previous studies were only observational.Methods: CF patients were included in a case-control study (n = 44 cases and n = 371 controls) during the 2009 pandemic A/H1N1 influenza. Cases were patients with polymerase reaction chain-confirmed influenza A/H1N1 infection. Controls did not report any influenza symptoms during the same period. Sputum colonization and lung function were monitored during 1 year after inclusion.Results: Cases were significantly younger than controls (mean(SD) 14.9 years(11) versus 20.1 years (13.2) and significantly less frequently colonized with P. aeruginosa (34 % versus 53 %). During influenza infection, 74 % of cases had pulmonary exacerbation, 92 % had antibiotics adapted to their usual sputum colonization and 82 % were treated with oseltamivir. Two cases required lung transplantation after A/H1N1 infection (one had not received oseltamivir and the other one had been treated late). The cases received a mean number of antibiotic treatments significantly higher during the year after the influenza infection (mean(SD) 2.8 (2.4) for cases versus 1.8(2.1) for controls; p = 0.002). An age-matched comparison did not demonstrate any significant modification of bronchopulmonary bacterial colonization during the year after influenza infection nor any significant change in FEV1 at months 1, 3 and 12 after A/H1N1 infection.Conclusions: Our results do not demonstrate any change in sputum colonization nor significant lung disease progression after pandemic A/H1N1 influenza.Trial Registration: Clinical Trials.gov registration number: NCT01499914. [ABSTRACT FROM AUTHOR]

Published
2016
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30. Continuous versus Intermittent Infusions of Ceftazidime for Treating Exacerbation of Cystic Fibrosis

Author

Hubert, Dominique, primary, Le Roux, Evelyne, additional, Lavrut, Thibaud, additional, Wallaert, Benoit, additional, Scheid, Philippe, additional, Manach, Dominique, additional, Grenet, Dominique, additional, Sermet-Gaudelus, Isabelle, additional, Ramel, Sophie, additional, Cracowski, Claire, additional, Sardet, Anne, additional, Wizla, Nathalie, additional, Deneuville, Eric, additional, and Garraffo, Rodolphe, additional

Published
2009
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32. [Diagnosis and neonatal screening of cystic fibrosis].

Author

Roussey M, Deneuville E, and Dabadie A

Subjects
Cystic Fibrosis genetics, France, Government Programs, Humans, Infant, Infant, Newborn, Cystic Fibrosis diagnosis, Neonatal Screening methods
Abstract

The circumstances leading to the diagnosis of cystic fibrosis are sensibly going to be modified in France, with the generalisation of systematic neonatal screening. Indeed, close to 95% of patients will be diagnosed in the neonatal period. Those who are missed by this screening often have a form of disease that is said to be moderate, with specific mutations, and without pancreatic insufficiency. However be the mode of revelation, the diagnosis should be confirmed by a positive sweat test with an elevated level of chlorine, and (or) the presence of 2 mutations in the gene causing the illness. Early diagnosis by screening only makes sense if the patients are followed in specialised centres of care, accredited by the professional bodies. It is in all likelihood to be at this price that the results, in terms of median survival, will improve in our country.

Published
2003

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