Conference
Clinical outcomes after lung transplantation for fibrosis in telomerase related genes mutation carriers
| العنوان: | Clinical outcomes after lung transplantation for fibrosis in telomerase related genes mutation carriers |
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| المؤلفون: | Phillips Houlbracq, Mathilde, Mal, Hervé, Cottin, Vincent, Philit, Francois, Hirschi, Sandrine, Roux, Antoine, Wemeau-Stervinou, Lidwine, Le Pavec, Jérôme, Pison, Christophe, Marchand-Adam, Sylvain, Froidure, Antoine, Lazor, Romain, Naccache, Jean-Marc, Jouneau, Stephane, Nunes, Hilario, Reynaud-Gaubert, Martine, Le Borgne, Aurélie, Crestani, Bruno, Kannengiesser, Caroline, Borie, Raphaël |
| المساهمون: | Service de pneumologie B, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-AP-HP - Hôpital Bichat - Claude Bernard Paris, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Diderot - Paris 7 (UPD7), Department of Pneumology Lyon, Hospices Civils de Lyon (HCL), Centre de Référence des Maladies Pulmonaires Rares Hôpital Louis Pradel - HCL, Hôpital Louis Pradel CHU - HCL, Hospices Civils de Lyon (HCL)-Hospices Civils de Lyon (HCL), Service de pneumologie (Strasbourg), Centre Hospitalier Universitaire Strasbourg (CHU Strasbourg), Les Hôpitaux Universitaires de Strasbourg (HUS)-Les Hôpitaux Universitaires de Strasbourg (HUS)-Nouvel Hôpital Civil de Strasbourg, Les Hôpitaux Universitaires de Strasbourg (HUS), Service de pneumologie. Centre de compétence des maladies pulmonaires rares, Centre Hospitalier Régional Universitaire CHU Lille (CHRU Lille), Service de chirurgie thoracique et transplantation pulmonaire, Centre Chirurgical Marie Lannelongue (CCML), Laboratory of Fundamental and Applied Bioenergetics = Laboratoire de bioénergétique fondamentale et appliquée (LBFA), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Grenoble Alpes 2016-2019 (UGA 2016-2019 ), Cliniques Universitaires Saint-Luc Bruxelles, Centre Hospitalier Lyon Sud CHU - HCL (CHLS), CHU Tenon AP-HP, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Contaminants Chimiques, immunité et Inflammation, Institut de recherche en santé, environnement et travail (Irset), Université d'Angers (UA)-Université de Rennes (UR)-École des Hautes Études en Santé Publique EHESP (EHESP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Structure Fédérative de Recherche en Biologie et Santé de Rennes (Biosit : Biologie - Santé - Innovation Technologique)-Université d'Angers (UA)-Université de Rennes (UR)-École des Hautes Études en Santé Publique EHESP (EHESP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Structure Fédérative de Recherche en Biologie et Santé de Rennes (Biosit : Biologie - Santé - Innovation Technologique), Hypoxie et Poumon : pneumopathologies fibrosantes, modulations ventilatoires et circulatoires (H&P), Université Paris 13 (UP13)-UFR SMBH, Hôpital Nord CHU - APHM, Microbes évolution phylogénie et infections (MEPHI), Institut de Recherche pour le Développement (IRD)-Aix Marseille Université (AMU)-Centre National de la Recherche Scientifique (CNRS), Physiopathologie et Epidemiologie de l'Insuffisance Respiratoire, Université Paris Diderot - Paris 7 (UPD7)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre de recherche sur l'Inflammation (CRI (UMR_S_1149 / ERL_8252 / U1149)), Université Paris Diderot - Paris 7 (UPD7)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Université Paris Diderot, Sorbonne Paris Cité, Paris, France, Université Paris Diderot - Paris 7 (UPD7) |
| المصدر: | European-Respiratory-Society (ERS) International Congress ; https://amu.hal.science/hal-02546160 ; European-Respiratory-Society (ERS) International Congress, Sep 2019, madrid, Spain. pp.PA3365, ⟨10.1183/13993003.congress-2019.PA3365⟩ |
| بيانات النشر: | CCSD |
| سنة النشر: | 2019 |
| المجموعة: | Université Paris 13: HAL |
| مصطلحات موضوعية: | Lung transplantation, Idiopathic pulmonary fibrosis, Transplantation pulmonaire, Fibrose pulmonaire, [SDV.MP.BAC]Life Sciences [q-bio]/Microbiology and Parasitology/Bacteriology, [SDV.MP.VIR]Life Sciences [q-bio]/Microbiology and Parasitology/Virology, [SDV.MP.PAR]Life Sciences [q-bio]/Microbiology and Parasitology/Parasitology, [SDV.MHEP.CSC]Life Sciences [q-bio]/Human health and pathology/Cardiology and cardiovascular system, [SDV.MHEP.ME]Life Sciences [q-bio]/Human health and pathology/Emerging diseases, [SDV.MHEP.MI]Life Sciences [q-bio]/Human health and pathology/Infectious diseases |
| جغرافية الموضوع: | Spain |
| Time: | madrid, Spain |
| الوصف: | International audience ; Carriers of telomerase related genes (TRG) mutation seem to present a worst prognosis with more common hematological complications after lung transplantation (LT) for pulmonary fibrosis. The aim of this study was to describe the outcomes after LT and identify pre-LT prognosis factors in a multicenter cohort of lung transplant recipients with TRG mutation.We retrospectively reviewed all identified patients with pathogenic TRG mutation (n=38; TERT, n=22, TERC, n=10, RTEL1, n=6) who received LT in France, Switzerland and Belgium between 2009 and 2018. The median age at LT was 54 years (46-59), 70% were male, and 60% had idiopathic pulmonary fibrosis (IPF). At diagnosis of pulmonary fibrosis, 84% had a hematological disease, including 8 with myelodysplasia, and 45% had a liver disease. After a median follow up of 2.2 years (1,2-3,7), 16 received a single LT, 22 a double LT and 2 a combined liver-LT.The overall post-LT median survival was 3.75 years (1.8-NA). Patients with myelodysplasia before LT had an increased risk of death after LT (HR= 4.12 (1.47-11.53) p=0.007). After LT, all patients showed anemia, 70% thrombocytopenia, and 60% neutropenia. Four patients showed severe liver disease: portal hypertension, cirrhosis. Sixteen patients (42%) experienced acute renal failure and 18 (47%) developed chronic renal insufficiency during follow up. Four developed chronic lung allograft dysfunction.Overall survival after LT supports LT in TRG mutation carriers. Careful evaluation at diagnosis, might limit LT indication for patients with established myelodysplasia. |
| نوع الوثيقة: | conference object |
| اللغة: | English |
| DOI: | 10.1183/13993003.congress-2019.PA3365 |
| الاتاحة: | https://amu.hal.science/hal-02546160 https://doi.org/10.1183/13993003.congress-2019.PA3365 |
| رقم الانضمام: | edsbas.9D7A0D73 |
| قاعدة البيانات: | BASE |
| DOI: | 10.1183/13993003.congress-2019.PA3365 |
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