Phenotypes and outcome of diffuse pulmonary non-amyloid light chain deposition disease

Autor: François Lestelle, Catherine Beigelman, David Rotzinger, Salim Si-Mohamed, Mouhamad Nasser, Lidwine Wemeau, Sandrine Hirschi, Grégoire Prevot, Antoine Roux, Vincent Bunel, Emmanuel Gomez, Laurent Sohier, Helene Morisse Pradier, Martine Reynaud Gaubert, Anne Gondouin, Romain Lazor, Jean-Charles Glerant, Françoise Thivolet Bejui, Magali Colombat, Vincent Cottin, the OrphaLung network

Publikováno v: Respiratory Research, Vol 25, Iss 1, Pp 1-12 (2024)

Abstract Background Light chain deposition disease (LCDD) is a very rare entity. Clinical manifestations of LCDD vary according to the organs involved. Data on pulmonary LCDD are scarce and limited to small series or case reports. This study aimed to

Externí odkaz: https://doaj.org/article/fba51a8eb5a4463791e31e0e1411e6c3

Pilot experience of multidisciplinary team discussion dedicated to inherited pulmonary fibrosis

Autor: Raphael Borie, Caroline Kannengiesser, Laurent Gouya, Clairelyne Dupin, Serge Amselem, Ibrahima Ba, Vincent Bunel, Philippe Bonniaud, Diane Bouvry, Aurélie Cazes, Annick Clement, Marie Pierre Debray, Philippe Dieude, Ralph Epaud, Pascale Fanen, Elodie Lainey, Marie Legendre, Aurélie Plessier, Flore Sicre de Fontbrune, Lidwine Wemeau-Stervinou, Vincent Cottin, Nadia Nathan, Bruno Crestani

Publikováno v: Orphanet Journal of Rare Diseases, Vol 14, Iss 1, Pp 1-10 (2019)

Abstract Background Genetic testing is proposed for suspected cases of monogenic pulmonary fibrosis, but clinicians and patients need specific information and recommendation about the related diagnosis and management issues. Because multidisciplinary

Externí odkaz: https://doaj.org/article/8636ea4a4a5f4fc7add9b69396ed22b1

Long-term evaluation of home-based pulmonary rehabilitation in patients with fibrotic idiopathic interstitial pneumonias

Autor: Benoit Wallaert, Louise Duthoit, Elodie Drumez, Hélène Behal, Lidwine Wemeau, Cécile Chenivesse, Jean-Marie Grosbois

Publikováno v: ERJ Open Research, Vol 5, Iss 2 (2019)

Background Few studies have examined the benefits of pulmonary rehabilitation in patients with fibrotic idiopathic pulmonary pneumonia (f-IIP). Here, we report the results of an observational study in routine clinical practice of home-based pulmonary

Externí odkaz: https://doaj.org/article/8184a9dc71aa439185f20758f7ca4333

Cyclophosphamide added to glucocorticoids in acute exacerbation of idiopathic pulmonary fibrosis (EXAFIP): a randomised, double-blind, placebo-controlled, phase 3 trial

Autor: Abdellatif Tazi, Aurélie Le Borgne-Krams, Marine Cachanado, Tabassome Simon, Sylvain Marchand-Adam, Morgane Didier, Lidwine Wemeau-Stervinou, Sandrine Hirschi, Frédéric Rivière, Arnaud Bourdin, François Lebargy, Stéphane Dominique, Aude Gibelin, Alexandre Chabrol, Tristan Dégot, Jacques Cadranel, Martine Reynaud-Gaubert, Marie-Pierre Debray, Sylvie Leroy, Frédéric Gagnadoux, Emmanuel Bergot, François-Xavier Blanc, Alexandra Rousseau, Raphael Borie, Pierre Yves Brillet, Guillaume Beltramo, Mallorie Kerjouan, Hilario Nunes, Olivia Freynet, Julie Traclet, Bruno Crestani, Anne-Sophie Gamez, Grégoire Prévot, Jean Pastré, Dominique Israel-Biet, Marie-Christine Dombret, Laurent Plantier, Cécile Chenivesse, Laurence Berard, Ana Nieves, Emmanuel Gomez, Dominique Valeyre, Stéphane Jouneau, Anne Gondouin, Elodie Blanchard, C. Launois, Nathalie Bautin, Jean-Marc Naccache, Vincent Cottin, Antoine Parrot, Philippe Bonniaud

Publikováno v: The Lancet Respiratory Medicine
The Lancet Respiratory Medicine, 2022, 10 (1), pp.26-34. ⟨10.1016/S2213-2600(21)00354-4⟩
The Lancet Respiratory Medicine, Elsevier, 2021, ⟨10.1016/S2213-2600(21)00354-4⟩

Summary Background The use of cyclophosphamide in patients with acute exacerbation of idiopathic pulmonary fibrosis (IPF) is unknown. Our study was designed to evaluate the efficacy and safety of four cyclophosphamide pulses in addition to high-dose

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::691aabc7559d1112b47b6efd1428d864
https://doi.org/10.1016/s2213-2600(21)00354-4

Filamin A Mutations

Autor: Victor Valentin, Thomas Smol, J.F. Bervar, Olivier Le Rouzic, Martine Remy, Cécile Chenivesse, Lidwine Wemeau, Catherine Vincent-Delorme

Publikováno v: Chest. 159:e131-e135

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 al

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_________::7114427d7804ed3998f571dfd8dd3e50
https://doi.org/10.1016/j.chest.2020.10.003