35 results on '"Wemeau, Lidwine"'
Search Results
2. Anti–IL-4R versus anti–IL-5/5R after anti–IL-5/5R failure in asthma: An emulated target trial
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Devouassoux, G., Taillé, C., Chanez, P., Bonniaud, P., Bourdin, A., Saint Raymond, C., Maurer, C., Beurnier, A., Roux, P., Margelidon, V., Boudjemaa, A., Mangiapan, G., Freymond, N., Didi, T., Russier, M., Garcia, G., Meyer, E. Popin, Dupin, C., Fouquet, F., Jouveshomme, S., Gaspard, W., Dury, S., Maillard, S. Habib, Izadifar, A., Cuvillon, E., Deslée, G., Barnig, C., Perotin, J.M., Gamez, A.S., Oster, J.P., Khayat, N., Chenivesse, C., Li, X., Appere de Vecchi, C., Gicquello, A., Rami, H., Vignal, G., Just, N., Blanc, X., Leroyer, C., Wemeau, L., Achkar, A., Sattler, C., Catherinot, E., Guilleminault, L., Gaillot-Drevon, M., Rochefort-Morel, C., Couturaud, F., Martin, P., Chabrol, A., Pegliasco, H., Sése, L., Romanet, S., Caverstri, B., Tcherakian, C., Magnan, A., Ahmed, E., Allibe, F., Beltramo, G., Michaux, K., Paleiron, N., Martinez, S., Begne, C., Tummino, C., Givel, C., Mourin, G., Salvator, H., Volpato, M., Drucbert, M., Rossignoli, N., Keddache, S., Justet, A., Andrejak, C., Valcke, J., Perrin, J., Mercy, M., Jouvenot, M., Soumagne, T., Elharrar, X., Douvry, B., Godbert, B., Maitre, B., Goyard, C., Didier, A., Cadet, E., Chabot, F., Gonzalez, J., Mattei, L., Gouitaa, M., Chauveau, S., Raymond, S., Dirou, S., Fry, S., Briault, A., Moui, A., Paris, A., NoelSavina, E., Olivier, C., Caradec, E., Roche, N., Picart, G., Belmont, L., Portel, L., Serra, M. Rocca, Guibert, N., Jean, R., Hadjadj, S., Guillo, S., Gauquelin, L., Estellat, C., Prigent, A., Larrousse, M., Jaffuel, D., Bourayou, Karima, Klising, Eve, Yelles, Nessima, Pochon, Sarra, Gouider, Amal, Medina, Hadj Kaci, Yasmine, Sellali, Djouher, Dahmani, Ndao, Diakhou, Vacher, Yannick, Achkar, Antoine, Ahmed, Engi, Alain, Didier, Allibe, Flora, Andrejak, Claire, De Vecchi, Corinne Appere, Barnig, Cindy, Begne, Camille, Belmont, Laure, Beltramo, Guillaume, Blanc, Xavier, Briault, Amandine, Cadet, Emmanuelle, Caradec, Emmanuela, Catherinot, Émilie, Cavestri, Beatrice, Chabrol, Alexandre, Chanez, Pascal, Chauveau, Simon, Couturaud, Francis, Cuvillon, Édouard, Deslee, Gaëtan, Didi, Toufik, Dirou, Stéphanie, Douvry, benoît, Drucbert, Mélanie, Dupin, Clairelyne, Dury, Sandra, Elharrar, Xavier, Fouquet, Helen, Freymond, Nathalie, Fry, Stéphanie, Gaillot-Drevon, Maud, Gamez, Anne Sophie, Garcia, Gilles, Gaspard, Wanda, Gicquello, Alice, Givel, Claire, Godbert, benoit, Gonzalez, Jésus, Gouitaa, Marion, Goupil, François, Goyard, Céline, Guibert, Nicolas, Guilleminault, Laurent, Habib-Maillard, Stéphanie, Hadjadj, Samra, Izadifar, Armine, Jean, Romain, Jouvenot, marie, Jouveshomme, Stéphane, Just, Nicolas, Justet, Aurélien, Keddache, Sophia, Khayath, Naji, Lemaire, Bertrand, Leroyer, Christophe, Li, Xing, Magnan, Antoine, Maitre, Bernard, Mangiapan, Gilles, Margelidon, Victor, Martin, Pascale, Martinez, Stéphanie, Mattei, Laura, Maurer, Cyril, Mercy, Magalie, Michaux, Karine, Moui, Antoine, Mourin, Gisèle, Noel-Savina, Elisa, Olivier, Cécile, Oster, Jean-Philippe, Paleiron, Nicolas, Paris, Audrey, Pegliasco, Hervé, Perotin Collard, Jeanne-Marie, Perrin, Julie, Picart, Gaël, Pison, Christophe, Popin-Meyer, Élisabeth, Portel, Laurent, Rami, Hassina, Raymond, Stéphane, Serra, Mireille Rocca, Rochefort-Morel, Cécile, Romanet, Stéphanie, Rossignoli, Nadine, Roux, Pauline, Russier, Maud, Saint-Raymond, Christel, Salmeron, Sergio, Salvator, Helene, Sattler, Caroline, Sese, Lucile, Soumagne, Thibaud, Tcherakian, Colas, Tiotiu, Angélica, Tummino, Céline, Valcke-Brossollet, Judith, Vignal, Guillaume, Volpato, Mathilde, Wemeau, Lidwine, Valery, Solène, Simon-Tillaux, Noémie, Devouassoux, Gilles, Bonniaud, Philippe, Beurnier, Antoine, Boudjemaa, Amel, Chenivesse, Cécile, Bourdin, Arnaud, Gauquelin, Lisa, Guillo, Sylvie, Taillé, Camille, and Estellat, Candice
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- 2024
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3. French recommendations for the diagnosis and management of lymphangioleiomyomatosis
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Cottin, Vincent, Blanchard, Elodie, Kerjouan, Mallorie, Lazor, Romain, Reynaud-Gaubert, Martine, Taille, Camille, Uzunhan, Yurdagül, Wemeau, Lidwine, Andrejak, Claire, Baud, Dany, Bonniaud, Philippe, Brillet, Pierre-Yves, Calender, Alain, Chalabreysse, Lara, Court-Fortune, Isabelle, Desbaillets, Nicolas Pierre, Ferretti, Gilbert, Guillemot, Anne, Hardelin, Laurane, Kambouchner, Marianne, Leclerc, Violette, Lederlin, Mathieu, Malinge, Marie-Claire, Mancel, Alain, Marchand-Adam, Sylvain, Maury, Jean-Michel, Naccache, Jean-Marc, Nasser, Mouhamad, Nunes, Hilario, Pagnoux, Gaële, Prévot, Grégoire, Rousset-Jablonski, Christine, Rouviere, Olivier, Si-Mohamed, Salim, Touraine, Renaud, Traclet, Julie, Turquier, Ségolène, Vagnarelli, Stéphane, and Ahmad, Kaïs
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- 2023
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4. Treatment of Idiopathic Pulmonary Fibrosis with Capsule or Tablet Formulations of Pirfenidone in the Real-Life French RaDiCo-ILD Cohort
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Cottin, Vincent, Guéguen, Sonia, Nunes, Hilario, Jouneau, Stéphane, Crestani, Bruno, Bonniaud, Philippe, Wemeau, Lidwine, Israël-Biet, Dominique, Reynaud-Gaubert, Martine, Gondouin, Anne, Cadranel, Jacques, Marchand-Adam, Sylvain, Chevereau, Marie, Dufaure-Garé, Isabelle, Amselem, Serge, and Clément, Annick
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- 2022
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5. Interstitial pneumonia with autoimmune features: Evaluation of connective tissue disease incidence during follow-up
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Decker, Paul, Sobanski, Vincent, Moulinet, Thomas, Launay, David, Hachulla, Eric, Valentin, Victor, Godbert, Benoit, Revuz, Sabine, Guillaumot, Anne, Gomez, Emmanuel, Chabot, François, Wémeau, Lidwine, and Jaussaud, Roland
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- 2022
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6. Frequency and characteristics of refractory dyspnea in idiopathic fibrosing interstitial pneumonia
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Bautin, Nathalie, primary, Ferlin, Juliette, additional, Terce, Gaelle, additional, Ternynck, Camille, additional, Valentin, Victor, additional, Wemeau, Lidwine, additional, and Chenivesse, Cecile, additional
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- 2024
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7. Efficacy and safety of rituximab in patients with chronic hypersensitivity pneumonitis (cHP): A retrospective, multicentric, observational study
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Ferreira, Marion, Borie, Raphaël, Crestani, Bruno, Rigaud, Pierre, Wemeau, Lidwine, Israel-Biet, Dominique, Leroy, Sylvie, Quétant, Sébastien, Plantier, Laurent, Dalphin, Jean-Charles, Cottin, Vincent, and Marchand-Adam, Sylvain
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- 2020
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8. Similarities and differences of interstitial lung disease associated with pathogenic variants in SFTPC and ABCA3 in adults.
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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.
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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]
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- 2024
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9. Liver disease in germline mutations of telomere-related genes: Prevalence, clinical, radiological, pathological features, outcome, and risk factors
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Sidali, Sabrina, primary, Borie, Raphaël, additional, de Fontbrune, Flore Sicre, additional, El Husseini, Kinan, additional, Rautou, Pierre-Emmanuel, additional, Lainey, Elodie, additional, Goria, Odile, additional, Crestani, Bruno, additional, Cadrane, Jacques, additional, Cottin, Vincent, additional, Bune, Vincent, additional, Dumortier, Jérôme, additional, Jacquemin, Emmanuel, additional, Reboux, Noémi, additional, Hirschi, Sandrine, additional, Bourdin, Arnaud, additional, Meszaros, Magdalena, additional, Dharancy, Sebastien, additional, Hilaire, Sophie, additional, Mallet, Vincent, additional, Reynaud-Gaubert, Martine, additional, Terriou, Louis, additional, Gottrand, Frédéric, additional, Abou Chahla, Wadih, additional, Khan, Jean-Emmanuel, additional, Carrier, Paul, additional, Saliba, Faouzi, additional, Rubbia-Brandt, Laura, additional, Aubert, John-David, additional, Elkrief, Laure, additional, de Lédinghen, Victor, additional, Abergel, Armand, additional, Olivier, Tournilhac, additional, Houssel, Pauline, additional, Jouneau, Stephane, additional, Wemeau, Lidwine, additional, Bergeron, Anne, additional, Leblanc, Thierry, additional, Ollivier-Hourmand, Isabelle, additional, Nguyen Khac, Eric, additional, Morisse-Pradier, Hélène, additional, Ba, Ibrahima, additional, Boileau, Catherine, additional, Roudot-Thoraval, Françoise, additional, Vilgrain, Valérie, additional, Bureau, Christophe, additional, Nunes, Hilario, additional, Naccache, Jean-Marc, additional, Durand, François, additional, Francoz, Claire, additional, Roulot, Dominique, additional, Valla, Dominique, additional, Paradis, Valérie, additional, Kannengiesser, Caroline, additional, and Plessier, Aurélie, additional
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- 2023
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10. Phenotypic characterization of interstitial lung disease associated with mutations in SFTPC and ABCA3 in adults
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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
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- 2023
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11. Increased mortality in patients with RA-associated interstitial lung disease: data from a French administrative healthcare database.
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Juge, Pierre-Antoine, Wemeau, Lidwine, Ottaviani, Sebastien, Desjeux, Guillaume, Joe Zhuo, Vannier-Moreau, Virginie, Flipo, René-Marc, Crestani, Bruno, and Dieudé, Philippe
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- 2023
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12. Idiopathic chronic obliterative bronchiolitis: a multicentric retrospective cohort
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Nasser, Mouhamad, primary, Uzunhan, Yurdagül, additional, Hirschi, Sandrine, additional, Jouneau, Stéphane, additional, Lebargy, François, additional, Justet, Aurélien, additional, Taillé, Camille, additional, Wemeau, Lidwine, additional, Valentin, Victor, additional, Borie, Raphael, additional, Godbert, Benoit, additional, Gagnadoux, Frédéric, additional, Guilleminault, Laurent, additional, Nunes, Hilario, additional, Marchand-Adam, Sylvain, additional, Cottin, Vincent, additional, and Nasser, Mouhamad, additional
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- 2021
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13. Diffuse Pulmonary Non-Amyloid Light-Chain Deposition Disease: Phenotypes and Outcome
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Lestelle, François, primary, Nasser, Mouham, additional, Roux, Antoine, additional, Hirschi, Sandrine, additional, Wemeau, Lidwine, additional, Prévot, Gregoire, additional, Rotzinger, David, additional, Si-Mohamed, Salim, additional, Bunel, Vincent, additional, Gomez, Emmanuel, additional, Sohier, Laurent, additional, Morisse Pradier, Helene, additional, Reynaud Gaubert, Martine, additional, Gondouin, Anne, additional, Lazor, Romain, additional, Uzunhan, Yurdagul, additional, Rigaud, Pierre, additional, Colombat, Magali, additional, Beigelman, Catherine, additional, and Cottin, Vincent, additional
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- 2021
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14. Seronegative polyarthritis revealing antisynthetase syndrome: a multicentre study of 40 patients
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Lefèvre, Guillaume, Meyer, Alain, Launay, David, Machelart, Irène, DeBandt, Michel, Michaud, Julien, Tournadre, Anne, Godmer, Pascal, Kahn, Jean-Emmanuel, Behra-Marsac, Aurélie, Timsit, Marie-Agnès, Schleinitz, Nicolas, Wendling, Daniel, Melac-Ducamp, Sylvie, Boyer, Philippe, Peretz, Anne, Lequerré, Thierry, Richez, Christophe, Stervinou-Wemeau, Lidwine, Morell-Dubois, Sandrine, Lambert, Marc, Dubucquoi, Sylvain, Wallaert, Benoit, Benveniste, Olivier, Flipo, René-Marc, Hatron, Pierre-Yves, Sibilia, Jean, Hachulla, Eric, and Hervier, Baptiste
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- 2015
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15. Safety and efficacy of pirfenidone and nintedanib in patients with idiopathic pulmonary fibrosis and carrying a telomere-related gene mutation
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Justet, Aurelien Klay, Dymph Porcher, Raphael Cottin, Vincent Ahmad, Kais Molina, Maria Molina Nunes, Hilario and Reynaud-Gaubert, Martine Naccache, Jean Marc Manali, Effrosyni and Froidure, Antoine Jouneau, Stephane Wemeau, Lidwine and Andrejak, Claire Gondouin, Anne Hirschi, Sandrine Blanchard, Elodie Bondue, Benjamin Bonniaud, Philippe Tromeur, Cecile and Prevot, Gregoire Marchand-Adam, Sylvain Funke-Chambour, Manuela Gamez, Anne Sophie Ba, Ibrahima Papiris, Spyridon and Grutters, Jan Crestani, Bruno van Moorsel, Coline and Kannengiesser, Caroline Borie, Raphael OrphaLung Network
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- 2021
16. Treatment of Idiopathic Pulmonary Fibrosis with Capsule or Tablet Formulations of Pirfenidone in the Real-Life French RaDiCo-ILD Cohort
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Cottin, Vincent, Guéguen, Sonia, Nunes, Hilario, Jouneau, Stéphane, Crestani, Bruno, Bonniaud, Philippe, Wemeau, Lidwine, Israël-Biet, Dominique, Reynaud-Gaubert, Martine, Gondouin, Anne, Cadranel, Jacques, Marchand-Adam, Sylvain, Chevereau, Marie, Dufaure-Garé, Isabelle, Amselem, Serge, Clément, Annick, Bergot, Emmanuel, Bourdin, Arnaud, Chenivesse, Cécile, Dalphin, Jean-Charles, Dromer, Claire, Gomez, Emmanuel, Hirschi, Sandrine, Montani, David, Prévot, Grégoire, Quetant, Sébastien, Valeyre, Dominique, Physiologie & médecine expérimentale du Cœur et des Muscles [U 1046] (PhyMedExp), Université de Montpellier (UM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Hôpital Louis Pradel [CHU - HCL], Hospices Civils de Lyon (HCL), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon, Maladies génétiques d'expression pédiatrique [CHU Trousseau] (Inserm U933), Institut National de la Santé et de la Recherche Médicale (INSERM)-CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Hôpital Avicenne [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), CHU Pontchaillou [Rennes], AP-HP - Hôpital Bichat - Claude Bernard [Paris], CHU Dijon, Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), CHU Lille, Hôpital Européen Georges Pompidou [APHP] (HEGP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO), CHU Marseille, Centre Hospitalier Régional Universitaire de Besançon (CHRU Besançon), CHU Tenon [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Centre Hospitalier Régional Universitaire de Tours (CHRU Tours), UF de Génétique moléculaire [CHU Trousseau], CHU Trousseau [APHP], Service de Pneumologie pédiatrique [CHU Trousseau], Centre de référence national pour les maladies respiratoires rares de l’enfant RespiRare [CHU Trousseau], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-CHU Trousseau [APHP], and the RaDiCo team: Emmanuel Bergot, Philippe Bonniaud, Arnaud Bourdin, Jacques Cadranel, Cécile Chenivesse, Vincent Cottin, Bruno Crestani, Jean-Charles Dalphin, Claire Dromer, Emmanuel Gomez, Sandrine Hirschi, Dominique Israël-Biet, Stéphane Jouneau, Sylvain Marchand-Adam, David Montani, Hilario Nunes, Grégoire Prévot, Sébastien Quetant, Martine Reynaud-Gaubert, Dominique Valeyre, Lidwine Wemeau, and MORNET, Dominique
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medicine.medical_specialty ,Pyridones ,[SDV]Life Sciences [q-bio] ,Population ,Interstitial lung disease ,Pirfenidone ,[SDV.MHEP.PSR]Life Sciences [q-bio]/Human health and pathology/Pulmonology and respiratory tract ,Cohort Studies ,03 medical and health sciences ,Idiopathic pulmonary fibrosis ,0302 clinical medicine ,Internal medicine ,medicine ,Humans ,Pharmacology (medical) ,030212 general & internal medicine ,Adverse effect ,education ,Capsule ,ComputingMilieux_MISCELLANEOUS ,education.field_of_study ,business.industry ,General Medicine ,medicine.disease ,Rash ,Idiopathic Pulmonary Fibrosis ,3. Good health ,Clinical trial ,Treatment Outcome ,030228 respiratory system ,Cohort ,[SDV.MHEP.PSR] Life Sciences [q-bio]/Human health and pathology/Pulmonology and respiratory tract ,France ,Antifibrotic ,medicine.symptom ,Tablet ,Lung Diseases, Interstitial ,business ,Tablets ,Cohort study ,medicine.drug - Abstract
Auteur groupe collaboratif RaDiCo team; International audience; Introduction: Pirfenidone, an antifibrotic medication for idiopathic pulmonary fibrosis (IPF), is now available in France in two formulations: tablets since April 2018, and the initial capsules form. We conducted a cohort study to describe tolerance and acceptability of capsules and/or tablets of pirfenidone in patients with IPF.Methods: This study was nested within the French, non-randomized, multicenter RaDiCo-ILD (Rare Disease Cohort-Interstitial Lung Diseases). Included patients with IPF received at least one dose of pirfenidone tablets or capsules from July 2017 to June 2019 in three populations: the inclusion population (patients treated at least once with pirfenidone during the study period, n = 288); the potential switch population (patients treated with pirfenidone during the switch period starting April 2018, n = 256); the newly treated population (patients who initiated pirfenidone during the study period, n = 162). Each of those last two populations included three subgroups (tablets, capsules, and substitution).Results: In 288 patients treated, 162 newly initiated pirfenidone during the study period: there were no meaningful differences in the baseline characteristics with the 256 patients treated during the potential switch period. In the newly treated population, 30.3% started pirfenidone treatment with tablet formulation. In the potential switch population, 44.9% of patients shifted from capsule to tablet. Half of the patients shifted to tablet formulation within the first 10 months. The mean treatment duration was 21.5 months with a mean dose of 2106.7 mg/day; 46.5% of patients discontinued treatment, mainly because of adverse events. There were fewer discontinuations in the tablets and substitution subgroups than in the capsules-only subgroup. The most reported adverse event was skin rash (11.5%). No new adverse event was identified.Conclusions: This real-life cohort assessing the characteristics of the prescription of pirfenidone tablets and capsules suggests a good acceptability of the tablet formulation by patients with IPF.Trial registration: Clinical trial registered with www.clinicaltrials.gov (NCT04238871).
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- 2021
17. Filamin A Mutations
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Valentin, Victor, primary, Bervar, Jean-François, additional, Vincent-Delorme, Catherine, additional, Smol, Thomas, additional, Wemeau, Lidwine, additional, Remy, Martine, additional, Le Rouzic, Olivier, additional, and Chenivesse, Cécile, additional
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- 2021
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18. Interstitial lung diseases associated with mutations of poly(A)-specific ribonuclease: a multicentric retrospective cohort study
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Philippot, Quentin Kannengiesser, Caroline Ba, Ibrahima and Gondouin, Anne Naccache, Jean-Marc Reynaud-Gobert, Martine and Uzunhan, Yurdagul Bondue, Benjamin Biet, Dominique Israel and Manali, Effrosyne Papiris, Spyros Wemeau, Lidwine Hirschi, Sandrine Mal, Herve Nunes, Hilario Schlemmer, Frederic and Rigaud, Matthieu Cottin, Vincent Crestani, Bruno Borie, Raphael
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- 2020
19. Safety and efficacy of pirfenidone and nintedanib in patients with idiopathic pulmonary fibrosis and carrying a telomere-related gene mutation
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Justet, Aurélien, primary, Klay, Dymph, additional, Porcher, Raphaël, additional, Cottin, Vincent, additional, Ahmad, Kais, additional, Molina Molina, Maria, additional, Nunes, Hilario, additional, Reynaud-Gaubert, Martine, additional, Naccache, Jean Marc, additional, Manali, Effrosyni, additional, Froidure, Antoine, additional, Jouneau, Stéphane, additional, Wemeau, Lidwine, additional, Andrejak, Claire, additional, Gondouin, Anne, additional, Hirschi, Sandrine, additional, Blanchard, Elodie, additional, Bondue, Benjamin, additional, Bonniaud, Philippe, additional, Tromeur, Cécile, additional, Prévot, Grégoire, additional, Marchand-Adam, Sylvain, additional, Funke-Chambour, Manuela, additional, Gamez, Anne Sophie, additional, Ba, Ibrahima, additional, Papiris, Spyridon, additional, Grutters, Jan, additional, Crestani, Bruno, additional, van Moorsel, Coline, additional, Kannengiesser, Caroline, additional, and Borie, Raphaël, additional
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- 2020
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20. Fatal Covid‐19 vasoplegic shock in a recipient few hours before double lung transplantation in high emergency
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Mercier, Olaf, primary, Laverdure, Florent, additional, Filaire, Laura, additional, Mal, Hervé, additional, Bunel, Vincent, additional, Deblauwe, Delphine, additional, Wemeau, Lidwine, additional, Dauriat, Gaëlle, additional, Fadel, Elie, additional, and Vincentelli, André, additional
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- 2020
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21. Interstitial lung diseases associated with mutations of poly(A)‐specific ribonuclease: A multicentre retrospective study.
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Philippot, Quentin, Kannengiesser, Caroline, Debray, Marie Pierre, Gauvain, Clément, Ba, Ibrahima, Vieri, Margherita, Gondouin, Anne, Naccache, Jean‐Marc, Reynaud‐Gaubert, Martine, Uzunhan, Yurdagul, Bondue, Benjamin, Israël‐Biet, Dominique, Dieudé, Philippe, Fourrage, Cécile, Lainey, Elodie, Manali, Effrosyne, Papiris, Spyros, Wemeau, Lidwine, Hirschi, Sandrine, and Mal, Hervé
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TELOMERASE reverse transcriptase ,RIBONUCLEASES ,INTERSTITIAL lung diseases ,IDIOPATHIC pulmonary fibrosis ,VITAL capacity (Respiration) ,PULMONARY fibrosis ,GENETIC mutation - Abstract
Background and objective: Poly(A)‐specific ribonuclease (PARN) mutations have been associated with familial pulmonary fibrosis. This study aims to describe the phenotype of patients with interstitial lung disease (ILD) and heterozygous PARN mutations. Methods: We performed a retrospective, observational, non‐interventional study of patients with an ILD diagnosis and a pathogenic heterozygous PARN mutation followed up in a centre of the OrphaLung network. Results: We included 31 patients (29 from 16 kindreds and two sporadic patients). The median age at ILD diagnosis was 59 years (range 54 to 63). In total, 23 (74%) patients had a smoking history and/or fibrogenic exposure. The pulmonary phenotypes were heterogenous, but the most frequent diagnosis was idiopathic pulmonary fibrosis (n = 12, 39%). Haematological abnormalities were identified in three patients and liver disease in two. In total, 21 patients received a specific treatment for ILD: steroids (n = 13), antifibrotic agents (n = 11), immunosuppressants (n = 5) and N‐acetyl cysteine (n = 2). The median forced vital capacity decline for the whole sample was 256 ml/year (range −363 to −148). After a median follow‐up of 32 months (range 18 to 66), 10 patients had died and six had undergone lung transplantation. The median transplantation‐free survival was 54 months (95% CI 29 to ∞). Extra‐pulmonary features were less frequent with PARN mutation than telomerase reverse transcriptase (TERT) or telomerase RNA component (TERC) mutation. Conclusion: IPF is common among individuals with PARN mutation, but other ILD subtypes may be observed. The pulmonary phenotypes of 31 patients with interstitial lung disease (ILD) and heterozygous poly(A)‐specific ribonuclease (PARN) mutations in this cohort were heterogeneous, but idiopathic pulmonary fibrosis was the most frequent diagnosis. Haematological and hepatic features were less frequent than in patients affected with telomerase reverse transcriptase (TERT)‐ or telomerase RNA component (TERC)‐associated ILDs. [ABSTRACT FROM AUTHOR]
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- 2022
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22. Characteristics and management of patients with idiopathic pulmonary fibrosis treated with pirfenidone capsule or tablet formulation
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Cottin, Vincent, primary, Guéguen, Sonia, additional, Jouneau, Stéphane, additional, Nunes, Hilario, additional, Crestani, Bruno, additional, Bonniaud, Philippe, additional, Wemeau, Lidwine, additional, Israel-Biet, Dominique, additional, Chevereau, Marie, additional, Dufaure-Garé, Isabelle, additional, Amselem, Serge, additional, and Clément, Annick, additional
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- 2020
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23. Interstitial lung diseases associated with mutations of poly(A)-specific ribonuclease: a multicentric retrospective cohort study
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Philippot, Quentin, primary, Kannengiesser, Caroline, additional, Ba, Ibrahima, additional, Gondouin, Anne, additional, Naccache, Jean-Marc, additional, Reynaud-Gobert, Martine, additional, Uzunhan, Yurdagul, additional, Bondue, Benjamin, additional, Israel Biet, Dominique, additional, Manali, Effrosyne, additional, Papiris, Spyros, additional, Wemeau, Lidwine, additional, Hirschi, Sandrine, additional, Mal, Hervé, additional, Nunes, Hilario, additional, Schlemmer, Frédéric, additional, Rigaud, Matthieu, additional, Cottin, Vincent, additional, Crestani, Bruno, additional, and Borie, Raphaël, additional
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- 2020
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24. Real-Life Safety and Effectiveness of Lumacaftor–Ivacaftor in Patients with Cystic Fibrosis
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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
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- 2020
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25. Safety and efficacy of pirfenidone in patients carrying telomerase complex mutation
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Justet, Aurelien Thabut, Gabriel Manali, Effrosyni Molina Molina, Maria Kannengiesser, Caroline Cadranel, Jacques and Cottin, Vincent Gondouin, Anne Nunes, Hilario Magois, Eline and Tromeur, Cecile Prevot, Gregoire Papiris, Spyros and Marchand-Adam, Sylvain Gamez, Anne Sophie Reynaud-Gaubert, Martine Wemeau, Lidwine Crestani, Bruno Borie, Raphael
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- 2018
26. Characterisation of a new clinical presentation of chronic lymphocytic leukaemia: symptomatic bronchial involvement, a study from the FILO group
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Nudel, Morgane, primary, Baran‐Marszak, Fanny, additional, Bossard, Jean‐Baptiste, additional, Dubois, Romain, additional, Dapvril, Héloïse, additional, Dupuis, Jehan, additional, Laribi, Kamel, additional, Bay, Jacques‐Olivier, additional, Tomowiak, Cécile, additional, Dreyfus, Brigitte, additional, Lepretre, Stéphane, additional, Demarquette, Hélène, additional, Wallyn, Frédéric, additional, Wemeau, Lidwine, additional, Wemeau, Mathieu, additional, Poulain, Stéphanie, additional, Morschhauser, Franck, additional, Cymbalista, Florence, additional, and Herbaux, Charles, additional
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- 2019
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27. Long-term evaluation of home-based pulmonary rehabilitation in patients with fibrotic idiopathic interstitial pneumonias
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Wallaert, Benoit, primary, Duthoit, Louise, additional, Drumez, Elodie, additional, Behal, Hélène, additional, Wemeau, Lidwine, additional, Chenivesse, Cécile, additional, and Grosbois, Jean-Marie, additional
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- 2019
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28. Daily life physical activity in patients with chronic stage IV sarcoidosis: A multicenter cohort study
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Froidure, Sarah, primary, Kyheng, Maeva, additional, Grosbois, Jean Marie, additional, Lhuissier, Francois, additional, Stelianides, Sandrine, additional, Wemeau, Lidwine, additional, and Wallaert, Benoit, additional
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- 2019
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29. Filamin A Mutations: A New Cause of Unexplained Emphysema in Adults?
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Valentin, Victor, Bervar, Jean-François, Vincent-Delorme, Catherine, Smol, Thomas, Wemeau, Lidwine, Remy, Martine, Le Rouzic, Olivier, and Chenivesse, Cécile
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NERVOUS system abnormalities ,RESEARCH ,LUNGS ,RESEARCH methodology ,GENETIC testing ,MEDICAL cooperation ,EVALUATION research ,COMPARATIVE studies ,MEDICAL history taking ,DISEASE susceptibility ,GENETIC techniques ,COMPUTED tomography ,PULMONARY emphysema ,CARRIER proteins ,GENEALOGY - Abstract
Emphysema is a chronic respiratory disorder characterized by destruction of alveoli, usually due to cigarette smoking or exposure to noxious particles or gases. Dysfunction of proteins that are involved in lung development and maintenance, such as alpha-1 antitrypsin, also contributes to emphysema. Filamin A (FLNA) is an actin-binding protein involved in cytoskeleton reorganization. Mutations in the FLNA gene classically lead to abnormal neuronal migration and connective and vascular tissue anomalies. Pulmonary manifestations consist of a wide range of pulmonary disorders that occur during infancy. We report the first familial case of emphysema in non- and very low-smoking adults who carry a loss-of-function mutation of the FLNA gene. The identification of this new risk factor for emphysema encourages (1) screening, prevention and monitoring of pulmonary disorders in patients with FLNA mutation and (2) screening for FLNA mutation in patients with early-onset emphysema that is associated with low-smoking or vascular or connective tissue anomalies. [ABSTRACT FROM AUTHOR]
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- 2021
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30. Safety and efficacy of pirfenidone in patients carrying telomerase complex mutation
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Justet, Aurélien, primary, Thabut, Gabriel, additional, Manali, Effrosyni, additional, Molina Molina, Maria, additional, Kannengiesser, Caroline, additional, Cadranel, Jacques, additional, Cottin, Vincent, additional, Gondouin, Anne, additional, Nunes, Hilario, additional, Magois, Eline, additional, Tromeur, Cécile, additional, Prevot, Grégoire, additional, Papiris, Spyros, additional, Marchand-Adam, Sylvain, additional, Gamez, Anne Sophie, additional, Reynaud-Gaubert, Martine, additional, Wemeau, Lidwine, additional, Crestani, Bruno, additional, and Borie, Raphael, additional
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- 2018
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31. Seronegative polyarthritis revealing antisynthetase syndrome: a multicentre study of 40 patients
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Lefèvre, Guillaume, primary, Meyer, Alain, additional, Launay, David, additional, Machelart, Irène, additional, DeBandt, Michel, additional, Michaud, Julien, additional, Tournadre, Anne, additional, Godmer, Pascal, additional, Kahn, Jean-Emmanuel, additional, Behra-Marsac, Aurélie, additional, Timsit, Marie-Agnès, additional, Schleinitz, Nicolas, additional, Wendling, Daniel, additional, Melac-Ducamp, Sylvie, additional, Boyer, Philippe, additional, Peretz, Anne, additional, Lequerré, Thierry, additional, Richez, Christophe, additional, Stervinou-Wemeau, Lidwine, additional, Morell-Dubois, Sandrine, additional, Lambert, Marc, additional, Dubucquoi, Sylvain, additional, Wallaert, Benoit, additional, Benveniste, Olivier, additional, Flipo, René-Marc, additional, Hatron, Pierre-Yves, additional, Sibilia, Jean, additional, Hachulla, Eric, additional, and Hervier, Baptiste, additional
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- 2014
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32. Fatal Covid‐19 vasoplegic shock in a recipient few hours before double lung transplantation in high emergency.
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Mercier, Olaf, Laverdure, Florent, Filaire, Laura, Mal, Hervé, Bunel, Vincent, Deblauwe, Delphine, Wemeau, Lidwine, Dauriat, Gaëlle, Fadel, Elie, and Vincentelli, André
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COVID-19 ,LUNG transplantation ,BK virus - Abstract
SARS-Cov2 outbreak has deeply impacted French lung transplant programs by the decreased number of lung donors, the scarcity of intensive care unit (ICU) beds, and most importantly, the assumed worse prognosis of Covid-19 in recipients during perioperative period. Despite no evidence has been published to suggest that lung transplant recipients were at high risk of acquiring the virus or developing severe Covid-19, immunosuppression may worsen the prognosis of such infection in patient transplanted for end-staged lung diseases. It seems mandatory to benefit from recipient SARS Cov2 PCR results as close as possible to acceptance of donor lungs for transplantation. [Extracted from the article]
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- 2021
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33. Liver disease in germline mutations of telomere-related genes: Prevalence, clinical, radiological, pathological features, outcome, and risk factors.
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Sidali S, Borie R, Sicre de Fontbrune F, El Husseini K, Rautou PE, Lainey E, Goria O, Crestani B, Cadranel J, Cottin V, Bunel V, Dumortier J, Jacquemin E, Reboux N, Hirschi S, Bourdin A, Meszaros M, Dharancy S, Hilaire S, Mallet V, Reynaud-Gaubert M, Terriou L, Gottrand F, Abou Chahla W, Khan JE, Carrier P, Saliba F, Rubbia-Brandt L, Aubert JD, Elkrief L, de Lédinghen V, Abergel A, Olivier T, Houssel P, Jouneau S, Wemeau L, Bergeron A, Leblanc T, Ollivier-Hourmand I, Nguyen Khac E, Morisse-Pradier H, Ba I, Boileau C, Roudot-Thoraval F, Vilgrain V, Bureau C, Nunes H, Naccache JM, Durand F, Francoz C, Roulot D, Valla D, Paradis V, Kannengiesser C, and Plessier A
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- Humans, Male, Female, Retrospective Studies, Middle Aged, Adult, Risk Factors, Prevalence, Aged, Young Adult, Telomere genetics, Adolescent, DNA Helicases, Liver Diseases genetics, Liver Diseases epidemiology, Liver Diseases pathology, Germ-Line Mutation, Telomerase genetics
- Abstract
Background and Aim: Germline mutations of telomere-related genes (TRG) induce multiorgan dysfunction, and liver-specific manifestations have not been clearly outlined. We aimed to describe TRG mutations-associated liver diseases., Approach and Results: Retrospective multicenter analysis of liver disease (transaminases > 30 IU/L and/or abnormal liver imaging) in patients with TRG mutations. Main measurements were characteristics, outcomes, and risk factors of liver disease in a TRG mutations cohort. The prevalence of liver disease was compared to a community-based control group (n = 1190) stratified for age and matched 1:3 for known risk factors of liver disease. Among 132 patients with TRG mutations, 95 (72%) had liver disease, with associated lung, blood, skin, rheumatological, and ophthalmological TRG diseases in 82%, 77%, 55%, 39%, and 30% of cases, respectively. Liver biopsy was performed in 52/95 patients, identifying porto-sinusoidal vascular disease in 48% and advanced fibrosis/cirrhosis in 15%. After a follow-up of 21 months (12-54), ascites, hepato-pulmonary syndrome, variceal bleeding, and HCC occurred in 14%, 13%, 13%, and 2% of cases, respectively. Five-year liver transplantation-free survival was 69%. A FIB-4 score ≥ 3·25 and ≥1 risk factor for cirrhosis were associated with poor liver transplantation-free survival. Liver disease was more frequent in patients with TRG mutations than in the paired control group [80/396, (20%)], OR 12.9 (CI 95%: 7.8-21.3, p < 0.001)., Conclusions: TRG mutations significantly increase the risk of developing liver disease. Although symptoms may be mild, they may be associated with severe disease. Porto-sinusoidal vascular disease and cirrhosis were the most frequent lesions, suggesting that the mechanism of action is multifactorial., (Copyright © 2023 American Association for the Study of Liver Diseases.)
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- 2024
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34. High risk of lung cancer in surfactant-related gene variant carriers.
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Brudon A, Legendre M, Mageau A, Bermudez J, Bonniaud P, Bouvry D, Cadranel J, Cazes A, Crestani B, Dégot T, Delestrain C, Diesler R, Epaud R, Philippot Q, Théou-Anton N, Kannengiesser C, Ba I, Debray MP, Fanen P, Manali E, Papiris S, Nathan N, Amselem S, Gondouin A, Guillaumot A, Andréjak C, Jouneau S, Beltramo G, Uzunhan Y, Galodé F, Westeel V, Mehdaoui A, Hirschi S, Leroy S, Marchand-Adam S, Nunes H, Picard C, Prévot G, Reynaud-Gaubert M, De Vuyst P, Wemeau L, Defossez G, Zalcman G, Cottin V, and Borie R
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- Humans, Male, Female, Middle Aged, Aged, Cross-Sectional Studies, Adult, Thyroid Nuclear Factor 1 genetics, ATP-Binding Cassette Transporters genetics, Risk Factors, Genetic Predisposition to Disease, Lung Diseases, Interstitial genetics, Heterozygote, Pulmonary Surfactant-Associated Proteins genetics, Lung Neoplasms genetics, Lung Neoplasms pathology, Pulmonary Surfactant-Associated Protein C genetics, Pulmonary Surfactant-Associated Protein A genetics
- Abstract
Background: Several rare surfactant-related gene (SRG) variants associated with interstitial lung disease are suspected to be associated with lung cancer, but data are missing. We aimed to study the epidemiology and phenotype of lung cancer in an international cohort of SRG variant carriers., Methods: We conducted a cross-sectional study of all adults with SRG variants in the OrphaLung network and compared lung cancer risk with telomere-related gene (TRG) variant carriers., Results: We identified 99 SRG adult variant carriers ( SFTPA1 (n=18), SFTPA2 (n=31), SFTPC (n=24), ABCA3 (n=14) and NKX2-1 (n=12)), including 20 (20.2%) with lung cancer ( SFTPA1 (n=7), SFTPA2 (n=8), SFTPC (n=3), NKX2-1 (n=2) and ABCA3 (n=0)). Among SRG variant carriers, the odds of lung cancer was associated with age (OR 1.04, 95% CI 1.01-1.08), smoking (OR 20.7, 95% CI 6.60-76.2) and SFTPA1 / SFTPA2 variants (OR 3.97, 95% CI 1.39-13.2). Adenocarcinoma was the only histological type reported, with programmed death ligand-1 expression ≥1% in tumour cells in three samples. Cancer staging was localised (I/II) in eight (40%) individuals, locally advanced (III) in two (10%) and metastatic (IV) in 10 (50%). We found no somatic variant eligible for targeted therapy. Seven cancers were surgically removed, 10 received systemic therapy, and three received the best supportive care according to their stage and performance status. The median overall survival was 24 months, with stage I/II cancers showing better survival. We identified 233 TRG variant carriers. The comparative risk (subdistribution hazard ratio) for lung cancer in SRG patients versus TRG patients was 18.1 (95% CI 7.1-44.7)., Conclusions: The high risk of lung cancer among SRG variant carriers suggests specific screening and diagnostic and therapeutic challenges. The benefit of regular computed tomography scan follow-up should be evaluated., Competing Interests: Conflict of interest: P. Bonniaud reports grants from AstraZeneca, lecture honoraria from Sanofi and AstraZeneca, travel support from AstraZeneca, Novartis, Sanofi, Boehringer and Stallergenes, and advisory board membership with AstraZeneca, Novartis, Sanofi, GSK and Boehringer. J. Cadranel had a patent planned, received consulting fees and participated on a data safety monitoring board or advisory board for AbbVie, Amgen, AstraZeneca, Boehringer Ingelheim, BMS, Daichi, Lilly, Pfizer, Novartis, MSD, Roche and Takeda. A. Cazes reports lecture honoraria and travel support from Boehringer Ingelheim. B. Crestani reports grants from Boehringer Ingelheim, consulting fees from Apellis, BMS, Boehringer Ingelheim and Sanofi, lecture honoraria from Apellis, AstraZeneca, BMS, Boehringer Ingelheim, Novartis and Sanofi, support for attending meetings or travel from AstraZeneca, BMS, Boehringer Ingelheim and Sanofi, participated on a data safety monitoring board or advisory board for Apellis, BMS, Boehringer Ingelheim and Sanofi, and had a leadership role as President of the Board of Trustees of the Fondation du Souffle. R. Epaud reports consulting fees from AstraZeneca, lecture honoraria from GSK, AstraZeneca and Menarini, travel support from GSK and AstraZeneca, and advisory board membership with AstraZeneca and Novartis. M-P. Debray reports lecture honoraria and travel support from Boehringer Ingelheim. E. Manali reports lecture honoraria from Boehringer Ingelheim, CLS Behring and Hoffman-La Roche, support for attending meetings or travel from Boehringer Ingelheim, CLS Behring, Hoffman-La Roche and Elpen, and had a leadership role as a Chair in the ERS Task Force for transition of chILD to adult care. S. Papiris reports lecture honoraria from Boehringer Ingelheim and Hoffmann-La Roche, and travel support from Boehringer Ingelheim and Elpen. N. Nathan reports grants from Legs poix de la Chancellerie des Universités 2022 (number 2022000594). C. Andréjak participated on a data safety monitoring board or advisory board for the EVER-ILD2 study (rituximab in diffuse interstitial pneumonia) and received funding via a grant from the French Research Ministry. S. Jouneau reports grants from AIRB, Boehringer Ingelheim and Roche, lecture honoraria from AIRB, AstraZeneca, Bristol Myers Squibb, Boehringer Ingelheim, Chiesi, Genzyme, GSK, LVL, Novartis, Pfizer, Roche and Sanofi, travel support from Boehringer Ingelheim, Roche and AIRB, and advisory board participation for Boehringer Ingelheim, GSK and Sanofi. G. Beltramo reports lecture honoraria from Bristol Myers Squibb, and support for attending meetings or travel from Sanofi Aventis France and Boehringer Ingelheim France. S. Hirschi reports research grants from Agence de la Biomedécine, CSL Behring and Adiral medical assistance, lecture honoraria from Boehringer Ingelheim, travel support from CSL Behring, Boehringer Ingelheim and ISIS Medical, and received medical equipment from ISIS Medical. C. Picard reports lecture honoraria and consulting fees from Boehringer Ingelheim. G. Prévot reports honoraria for presentations and educational event from Boehringer Ingelheim, Sanofi, Jansen and MSD. G. Zalcman reports consulting fees from AstraZeneca, BMS, Pfizer and Sanofi, lecture honoraria from BMS, AstraZeneca and Sanofi, support for attending meetings or travel from AstraZeneca and BMS, and participated on a data safety monitoring board or advisory board for AstraZeneca and BMS. V. Cottin reports grants from Boehringer Ingelheim, consulting fees from AstraZeneca, Boehringer Ingelheim, Celgene/BMS, CSL Behring, Ferrer/United Therapeutics, GSK, Pliant, Pure Tech, RedX, Roche, Sanofi and Shionogi, lecture honoraria from Boehringer Ingelheim, Ferrer/United Therapeutics and Roche, support for attending meetings or travel from Boehringer Ingelheim and Roche, participated on a data safety monitoring board or advisory board for Galapagos, Galecto and GSK, and had a leadership role in an adjudication committee for Fibrogen. R. Borie reports consulting fees from Boehringer Ingelheim, Ferrer and Sanofi, lecture honoraria from Boehringer Ingelheim and Roche, travel support from Boehringer Ingelheim, Roche and Chiesi, and advisory board participation for Savara. The remaining authors have no potential conflicts of interest to disclose., (Copyright ©The authors 2024.)
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- 2024
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35. Safety and efficacy of pirfenidone and nintedanib in patients with idiopathic pulmonary fibrosis and carrying a telomere-related gene mutation.
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Justet A, Klay D, Porcher R, Cottin V, Ahmad K, Molina Molina M, Nunes H, Reynaud-Gaubert M, Naccache JM, Manali E, Froidure A, Jouneau S, Wemeau L, Andrejak C, Gondouin A, Hirschi S, Blanchard E, Bondue B, Bonniaud P, Tromeur C, Prévot G, Marchand-Adam S, Funke-Chambour M, Gamez AS, Ba I, Papiris S, Grutters J, Crestani B, van Moorsel C, Kannengiesser C, and Borie R
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- Humans, Indoles, Mutation, Pyridones therapeutic use, Treatment Outcome, Idiopathic Pulmonary Fibrosis drug therapy, Idiopathic Pulmonary Fibrosis genetics, Telomere
- Abstract
Competing Interests: Conflict of interest: A. Justet reports grants from Roche, personal fees from Boeringher Ingelheim, outside the submitted work. Conflict of interest: D. Klay has nothing to disclose. Conflict of interest: R. Porcher has nothing to disclose. Conflict of interest: V. Cottin reports personal fees for advisory board work and lectures, and non-financial support for meeting attendance from Actelion, grants, personal fees for consultancy and lectures, and non-financial support for meeting attendance from Boehringer Ingelheim, personal fees for advisory board and data monitoring committee work from Bayer/MSD and Galapagos, personal fees for advisory board work and lectures from Novartis, personal fees for consultancy, lectures, steering committee and data monitoring committee work, and non-financial support for meeting attendance from Roche/Promedior, personal fees for lectures from Sanofi and AstraZeneca, personal fees for data monitoring committee work from Celgene and Galecto, personal fees for advisory board work from Shionogi, outside the submitted work. Conflict of interest: K. Ahmad reports personal fees from Roche and Boeringher Ingelheim, outside the submitted work. Conflict of interest: M. Molina-Molina reports grants and personal fees from Roche, Boehringer Ingelheim and Esteve-Teijin, personal fees from Chiesi, Pfizer and Galapagos, outside the submitted work. Conflict of interest: H. Nunes reports personal fees from Intermune, Roche, Boehringer Ingelheim and Sanofi, outside the submitted work. Conflict of interest: M. Reynaud-Gaubert has nothing to disclose. Conflict of interest: J.M. Naccache has nothing to disclose. Conflict of interest: E. Manali reports grants and personal fees from Roche and Boehringer Ingelheim, during the conduct of the study. Conflict of interest: A. Froidure reports grants, personal fees and non-financial support from Roche and Boehringer Ingelheim, personal fees and non-financial support from AstraZeneca, personal fees from GlaxoSmithKline, outside the submitted work. Conflict of interest: S. Jouneau reports fees, funding or reimbursement for national and international conferences, boards, expert or opinion groups, research projects over the past 3 years from AIRB, Bellorophon Therapeutics, Biogen, Boehringer, Chiesi, Fibrogen, Galecto Biotech, Genzyme, Gilead, LVL, Novartis, Olam Pharm, Pfizer, Pliant Therapeutics, Roche, Sanofi and Savara-Serendex. Conflict of interest: L. Wemeau has nothing to disclose. Conflict of interest: C. Andrejak has nothing to disclose. Conflict of interest: A. Gondouin has nothing to disclose. Conflict of interest: S. Hirschi has nothing to disclose. Conflict of interest: E. Blanchard has nothing to disclose. Conflict of interest: B. Bondue reports grants and personal fees from Boeringher Ingleheim and Hoffman La Roche, outside the submitted work. Conflict of interest: P. Bonniaud reports personal fees from Roche, Novartis, Boeringher, TEVA and AstraZeneca, outside the submitted work. Conflict of interest: C. Tromeur has nothing to disclose. Conflict of interest: G. Prevot reports personal fees from Actelion, Bayer, Boehringer Ingelheim and Roche, outside the submitted work. Conflict of interest: S. Marchand-Adam has nothing to disclose. Conflict of interest: M. Funke-Chambour reports grants from Roche and Boehringer Ingelheim, during the conduct of the study. Conflict of interest: A.S. Gamez has nothing to disclose. Conflict of interest: I. Ba has nothing to disclose. Conflict of interest: S. Papiris reports grants and personal fees from Roche and Boehringer Ingelheim, during the conduct of the study. Conflict of interest: J. Grutters has nothing to disclose. Conflict of interest: B. Crestani reports personal fees from AstraZeneca and Sanofi, grants and personal fees from Boeringher Ingelheim and Roche, personal fees and non-financial support from BMS, outside the submitted work. Conflict of interest: C. van Moorsel has nothing to disclose. Conflict of interest: C. Kannengiesser has nothing to disclose. Conflict of interest: R. Borie reports grants and personal fees from Boeringher Ingelheim and Roche, personal fees from Savapharma, outside the submitted work.
- Published
- 2021
- Full Text
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