Complex lung diseases require multidisciplinary care along with psycho-social support. This complexity can be due to the underlying genetic mechanism of the disease, the secondary changes and damage done to other organ systems. Early diagnosis and access to specialist care can improve outcomes for many of these conditions.

ERN-LUNG addresses a number of rare and complex pulmonary conditions, including idiopathic pulmonary fibrosis, cystic fibrosis, non-cystic fibrosis bronchiectasis, pulmonary hypertension, PCD, AATD, mesothelioma, chronic lung allograft dysfunction, and ORLD.

The network seeks to improve expertise across Europe to advance standards of care, quality of life and prognosis across the spectrum of rare pulmonary diseases. Members are: developing and disseminating care guidelines; promoting common treatment approaches; enhancing cross-border access to diagnosis and treatment; initiating and supporting registries; and assembling sufficiently large cohorts for clinical studies, drug development and natural history studies.

ERN-LUNG provides patients with access to the interdisciplinary teams, providing online second opinions on complex cases without requiring patients to travel. This will see the expansion of an online expert advice system established through the EU-funded pilot project, ECORN-CF.

Contact information


Below an overview is given of scientific publications related to ERN LUNG. Publications were obtained from the PubMed database. All publications where one of the authors has an affiliation link where "ERN LUNG" is mentioned, are added to the publication overview automatically. Updates are performed once a week.

Filter list publications

TitleAuthorsDateKeywordsIssue name
Initial combination therapy with macitentan and tadalafil in patients with pulmonary arterial hypertension, with and without cardiac comorbidities.McLaughlin VV, Sitbon O, Chin KM, Galiè N, Hoeper MM, Kiely DG, MacDonald G, Martin N, Mathai SC, Peacock A, Tawakol A, Torbicki A, Noordegraaf AV, Rosenkranz S2024-07-05Cardiac comorbidities, Initial double combination therapy, Macitentan, Pulmonary arterial hypertension, Risk stratification, TadalafilEuropean journal of heart failure
Phenotypes in Pulmonary Hypertension.Weatherald J, Hemnes AR, Maron BA, Mielniczuk LM, Gerges C, Price LC, Hoeper MM, Humbert M2024-07-04The European respiratory journal
Pulmonary Arterial Wedge Pressure in Healthy Subjects - a Meta-Analysis.Zeder K, Avian A, Mak S, Giannakoulas G, Kawut SM, Maron BA, Humbert M, Olschewski H, Kovacs G2024-07-04The European respiratory journal
EMBARCing on a new era for bronchiectasis: a review series for the Seventh World Bronchiectasis Conference.Chalmers JD, Goeminne PC, Ringshausen FC2024-07-01European respiratory review : an official journal of the European Respiratory Society
Growing from common ground: nontuberculous mycobacteria and bronchiectasis.Van Braeckel E, Bosteels C2024-07-01European respiratory review : an official journal of the European Respiratory Society
Multisystemic Effects of Elexacaftor-Tezacaftor-Ivacaftor in Adults with Cystic Fibrosis and Advanced Lung Disease.Burgel PR, Paillasseur JL, Durieu I, Reynaud-Gaubert M, Hamidfar R, Murris-Espin M, Danner-Boucher I, Chiron R, Leroy S, Douvry B, Grenet D, Mely L, Ramel S, Montcouquiol S, Burnet E, Ouaalaya EH, Sogni P, Da Silva J, Martin C2024-07-01cystic fibrosis transmembrane conductance regulator modulators, real-word evidence, severe cystic fibrosis liver diseaseAnnals of the American Thoracic Society
Anxiety and Depression in Patients with Pulmonary Arterial Hypertension and Chronic Thromboembolic Pulmonary Hypertension after the Removal of COVID-19 Pandemic Restrictions.Wieteska-Miłek M, Witowicz A, Szmit S, Florczyk M, Peller M, Dzienisiewicz M, Kurzyna M2024-06-17HADS scale, anxiety, chronic thromboembolic pulmonary hypertension, depression, fear of COVID-19, pandemic, pulmonary arterial hypertensionJournal of clinical medicine
Primary Ciliary Dyskinesia in Adult Bronchiectasis - Data from the German Bronchiectasis Registry PROGNOSIS.Ewen R, Pink I, Sutharsan S, Aries SP, Grünewaldt A, Shoemark A, Sommerwerck U, Staar BO, Wege S, Mertsch P, Rademacher J, Ringshausen FC2024-06-14Bronchiectasis, Kartagener syndrome, phenotype, primary ciliary dyskinesia, registriesChest
Correlation Between Tacrolimus Levels in Blood Samples Obtained from Central Catheter and Peripheral Venipuncture in Lung Transplant Patients (Ven-Cat Study).Diego-Fernández V, García-Saiz MDM, Llorente-Cantalapiedra A, Arquero-González JA, Bermúdez-García MV, Catalán-Ramírez MM, Cornejo-Callejo P, Nuria de Pedro-Simón M, Díez-Pérez MJ, Gandarillas-Ruiz P, Hernández-González F, Herranz-Arenillas P, Laso-Boada MJ, Medina-Gonzalo G, Rodríguez-López A, Ruiz-Antolín M, Álamo-Ibañez M, Cos-Cossio MLÁ, Lavín-Alconero L, Mora-Cuesta VM2024-06-11Therapeutic drug monitoring
Phenotypic and pathomechanistic overlap between tapasin and TAP deficiencies.Elsayed A, von Hardenberg S, Atschekzei F, Graalmann T, Jänke C, Witte T, Ringshausen FC, Sogkas G2024-06-10MHC class I, MHC class I deficiency, TAPBP, bronchiectasis, immunodeficiency, inborn errors of immunity, tapasinThe Journal of allergy and clinical immunology
A randomised phase II study of extended pleurectomy/decortication (e-PD) preceded or followed by chemotherapy in patients (pts) with early-stage pleural mesothelioma (PM): EORTC 1205.Raskin J, Surmont V, Maat APWM, Yahia M, Burgers SA, Pretzenbacher Y, De Ryck F, Cornelissen R, Klomp HM, Oliveira A, Aerts J, Baas P, Marreaud S, Van Meerbeeck JP, Van Schil PEY2024-06-06The European respiratory journal
Chronic Thromboembolic Pulmonary Hypertension: When Thrombus Mandates Definite Diagnosis to Occur in the Operating Room.Martín de Miguel I, López-Gude MJ, Martínez Meñaca A, Revilla Ostolaza Y, Arribas Ynsaurriaga F, Escribano Subías P2024-06-05acenocumarol, endarterectomy, magnetic resonance imaging, pulmonary hypertension thrombosis, tomographyCirculation. Cardiovascular imaging
[Primary ciliary dyskinesia].Raidt J, Staar BO, Omran H, Ringshausen FC2024-06-01Hydrocephalus, Infertility, Kartagener syndrome, Mucociliary clearance, Situs inversusInnere Medizin (Heidelberg, Germany)
Primum non nocere, secundum cavere, tertium sanare: lessons on the management of pulmonary hypertension associated with COPD.Kovacs G, Humbert M2024-06-01The European respiratory journal
Centralized intestinal organoid generation is a feasible and safe approach for personalized medicine as demonstrated in the HIT-CF Europe Organoid Study.Bierlaagh MC, van Mourik P, Vonk AM, Pott J, Muilwijk D, Berkers G, Aalbers BL, Vleggaar FP, Michel S, Boj SF, Vries RGJ, Beekman JM, van der Ent CK2024-05-18Forceps biopsy, Intestinal organoids, Personalized medicine, Rectal biopsy, Suction biopsyJournal of cystic fibrosis : official journal of the European Cystic Fibrosis Society
The impact of time from ILD diagnosis to referral to the transplant center on the probability of inclusion in the transplant waiting list.Mora-Cuesta VM, Martínez-Meñaca A, González-Fernández Á, Iturbe-Fernández D, Tello-Mena S, Izquierdo-Cuervo S, Fernández-Rozas S, Alonso-Lecue P, Cifrián-Martínez JM2024-05-11Interstitial lung disease, Lung transplantation, Rehabilitation, Transplant centerHeart & lung : the journal of critical care
Interstitial Lung Disease Associated with Systemic Sclerosis.Mismetti V, Si-Mohamed S, Cottin V2024-05-07Seminars in respiratory and critical care medicine
Considerations for the use of inhaled antibiotics for Pseudomonas aeruginosa in people with cystic fibrosis receiving CFTR modulator therapy.Burgel PR, Ballmann M, Drevinek P, Heijerman H, Jung A, Mainz JG, Peckham D, Plant BJ, Schwarz C, Taccetti G, Smyth A2024-05-03Bacterial Infection, Bronchiectasis, Cystic Fibrosis, Respiratory InfectionBMJ open respiratory research
Editing Physicians' Responses Using GPT-4 for Academic Research.Weber MT, Schaaf J, Storf H, Wagner TOF, Berger A, Noll R2024-04-26Artificial Intelligence, Data Anonymisation, Medical Informatics, Natural Language ProcessingStudies in health technology and informatics
Prime editing functionally corrects cystic fibrosis-causing CFTR mutations in human organoids and airway epithelial cells.Bulcaen M, Kortleven P, Liu RB, Maule G, Dreano E, Kelly M, Ensinck MM, Thierie S, Smits M, Ciciani M, Hatton A, Chevalier B, Ramalho AS, Casadevall I Solvas X, Debyser Z, Vermeulen F, Gijsbers R, Sermet-Gaudelus I, Cereseto A, Carlon MS2024-04-24CRISPR, DETECTOR, cystic fibrosis, gene editing, human nasal epithelial cells, machine learning, patient-derived organoids, prime editingCell reports. Medicine
Safety and Outcomes of Inferior Vena Cava Filter Placement in Oncology Patients: A Single-Centre Experience.Kurzyna P, Banaszkiewicz M, Florczyk M, Kępski J, Piłka M, Kędzierski P, Mańczak R, Szwed P, Kasperowicz K, Wrona K, Doroszewski G, Torbicki A, Kurzyna M, Szmit S, Darocha S2024-04-19cancer-associated thrombosis, deep venous thrombosis, inferior vena cava filter, oncology, pulmonary embolism, venous thromboembolismCancers
Ultra-low dose chest CT for the diagnosis of pulmonary arteriovenous malformation in patients with hereditary hemorrhagic telangiectasia.Delpon JE, Greffier J, Lacombe H, Barbe A, Bouin M, De Oliveira F, Mansuy A, Delagrange L, Fargeton AE, Beregi JP, Cottin V, Dupuis-Girod S, Si-Mohamed SA2024-04-10Hereditary hemorrhagic, Image enhancement, Multidetector computed tomography, Pulmonary arteriovenous malformation, Telangiectasia, Ultra-low dose protocolDiagnostic and interventional imaging
Phenotypes and outcome of diffuse pulmonary non-amyloid light chain deposition disease.Lestelle F, Beigelman C, Rotzinger D, Si-Mohamed S, Nasser M, Wemeau L, Hirschi S, Prevot G, Roux A, Bunel V, Gomez E, Sohier L, Pradier HM, Gaubert MR, Gondouin A, Lazor R, Glerant JC, Bejui FT, Colombat M, Cottin V2024-04-10Bronchiectasis, Light chain deposition disease, Lung cysts, Lung transplantationRespiratory research
High risk of lung cancer in surfactant-related gene variant carriers.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, Theou-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, Prevot G, Reynaud-Gaubert M, De Vuyst P, Wemeau L, Defossez G, Zalcman G, Cottin V, Borie R2024-04-04The European respiratory journal
Combined pulmonary fibrosis and emphysema syndrome: the age of majority.Cottin V2024-04-01The European respiratory journal
PREFUL MRI for Monitoring Perfusion and Ventilation Changes after Elexacaftor-Tezacaftor-Ivacaftor Therapy for Cystic Fibrosis: A Feasibility Study.Dohna M, Voskrebenzev A, Klimeš F, Kaireit TF, Glandorf J, Pallenberg ST, Ringshausen FC, Hansen G, Renz DM, Wacker F, Dittrich AM, Vogel-Claussen J2024-04-01CFTR, Comparative Studies, Cystic Fibrosis, Elexacaftor-Tezacaftor-Ivacaftor Therapy, Fourier Decomposition, Free‐Breathing Proton MRI, Functional MRI, Kaftrio, Lung, MR–Functional Imaging, Modulator Therapy, PREFUL, Pediatrics, Perfusion, Pulmonary, Pulmonary MRIRadiology. Cardiothoracic imaging
MERIT reloaded-what has changed in CTEPH management since 2017?Torbicki A, Kurzyna M2024-04-01The Lancet. Respiratory medicine
Pulmonary vascular phenotype identified in patients with GDF2 (BMP9) or BMP10 variants: an international multicentre study.Grynblat J, Bogaard HJ, Eyries M, Meyrignac O, Savale L, Jaïs X, Ghigna MR, Celant L, Meijboom L, Houweling AC, Levy M, Antigny F, Chaouat A, Cottin V, Guignabert C, Coulet F, Sitbon O, Bonnet D, Humbert M, Montani D2024-04-01The European respiratory journal
Quality of Life and the Cardiopulmonary Exercise Test in Pulmonary Arterial Hypertension Patients.Martínez-Meñaca A, Mora-Cuesta VM, Iturbe-Fernández D, Sáinz-Ezquerra Belmonte B, Fernández-Cavia G, Gallardo-Ruiz MJ2024-04-01Archivos de bronconeumologia
Similarities and differences of interstitial lung disease associated with pathogenic variants in SFTPC and ABCA3 in adults.Diesler R, Legendre M, Si-Mohamed S, Brillet PY, Wemeau L, Manali ED, Gagnadoux F, Hirschi S, Lorillon G, Reynaud-Gaubert M, Bironneau V, Blanchard E, Bourdin A, Dominique S, Justet A, Macey J, Marchand-Adam S, Morisse-Pradier H, Nunes H, Papiris SA, Traclet J, Traore I, Crestani B, Amselem S, Nathan N, Borie R, Cottin V2024-04-01ABCA3, SFTPC, adults, fibrosing interstitial lung disease, interstitial lung disease, surfactantRespirology (Carlton, Vic.)
Comment on the article titled "Impact of sex on outcome after pulmonary endarterectomy for chronic thromboembolic pulmonary hypertension" published by J. Chan et al.Cruz-Utrilla A, López-Gude MJ, Velázquez M, Arribas-Ynsaurriaga F, Escribano-Subias P2024-04-01The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation
Clinical course of suspected familial and sporadic idiopathic pulmonary fibrosis: Data from the PROOF-Next registry.Froidure A, Bondue B, Dahlqvist C, Guiot J, Gusbin N, Wirtz G, Brusselle G, Strens D, Slabbynck H, Wuyts WA2024-04-01clinical respiratory medicine, interstitial lung disease, pulmonary fibrosisRespirology (Carlton, Vic.)
The Initial Experience of Balloon Pulmonary Angioplasty for Chronic Thromboembolic Pulmonary Hypertension in Latvia.Krigere A, Kalejs VR, Kaulins R, Rudzitis A, Bondare L, Sablinskis M, Lejnieks A, Kigitovica D, Kurzyna M, Skride A2024-03-30Latvia, balloon pulmonary angioplasty, chronic thromboembolic pulmonary hypertensionMedicina (Kaunas, Lithuania)
Pulmonary Hypertension in Connective Tissue Diseases Other than Systemic Sclerosis.Budhram B, Weatherald J, Humbert M2024-03-18Seminars in respiratory and critical care medicine
Chronic interstitial lung disease associated with systemic lupus erythematosus: A multicentric study of 89 cases.Deneuville L, Mageau A, Debray MP, Sacre K, Costedoat-Chalumeau N, Hachulla E, Uzunhan Y, Le Tallec E, Cadranel J, Marchand Adam S, Montani D, Rémi-Jardin M, Reynaud-Gaubert M, Prevot G, Beltramo G, Crestani B, Cottin V, Borie R2024-03-17connective-tissue disease, disease progression, interstitial lung disease, lupus, non-specific interstitial pneumonia, pulmonary fibrosis, survivalRespirology (Carlton, Vic.)
Pulmonary fibrosis is uncommon in primary Sjögren disease.Si-Mohamed SA, Cottin V2024-03-15Computed tomography, Diffuse parenchymal lung disease, Interstitial lung disease, Primary Sjögren disease, Pulmonary fibrosisDiagnostic and interventional imaging
Health care resource utilization preceding death or lung transplantation in people with cystic fibrosis: HCRU before transplant or death in cystic fibrosis.Guyot E, Reynaud Q, Belhassen M, Bérard M, Dehillotte C, Lemonnier L, Viprey M, Van Ganse E, Burgel PR, Durieu I2024-03-12Claims data, Costs, Cystic fibrosis, Healthcare resource utilization, National registry, TransplantationJournal of cystic fibrosis : official journal of the European Cystic Fibrosis Society
Invasive Pulmonary Aspergillosis with Exclusive Pleural Involvement in a Lung Transplant Recipient.García-Martín S, Mora-Cuesta VM, Naranjo-Gozalo S, Jiménez-Velasco JJ, Roiz-Mesones MP, Fernández-Lobo V2024-03-11Aspergilllus fumigatus, clinical outcomes, infection, invasive pulmonary aspergillosis, lung transplantation, pneumologyProgress in transplantation (Aliso Viejo, Calif.)
Treatments for pulmonary arterial hypertension: navigating through a network of choices.Pitre T, Weatherald J, Humbert M2024-03-05European heart journal
Corrigendum to "Expert management of congenital portosystemic shunts and their complications" [JHEP Reports 6 (2024)].McLin VA, Franchi-Abella S, Brütsch T, Bahadori A, Casotti V, de Ville de Goyet J, Dumery G, Gonzales E, Guérin F, Hascoet S, Heaton N, Kuhlmann B, Lador F, Lambert V, Marra P, Plessier DA, Quaglia A, Rougemont AL, Savale L, Sarma MS, Sitbon O, Superina RA, Uchida H, van Albada M, Johannes van der Doef HP, Vilgrain V, Wacker J, Zwaveling-Soonawala N, Debray D, Wildhaber BE2024-03-01JHEP reports : innovation in hepatology
Lumacaftor/Ivacaftor Population Pharmacokinetics in Pediatric Patients with Cystic Fibrosis: A First Step Toward Personalized Therapy.Bouazza N, Urien S, Foissac F, Choupeaux L, Lui G, Froelicher Bournaud L, Rouillon S, Zheng Y, Bardin E, Stremler N, Bessaci K, Bihouee T, Coirier-Duet E, Marguet C, Deneuville E, Laurans M, Reix P, Gerardin M, Mittaine M, Epaud R, Thumerelle C, Weiss L, Berthaud R, Semeraro M, Treluyer JM, Benaboud S, Sermet-Gaudelus I2024-03-01Clinical pharmacokinetics
C-reactive protein as a candidate biomarker in fibrotic interstitial lung disease.Cottin V, Valenzuela C2024-03-01C-reactive protein, blood biomarker, idiopathic pulmonary fibrosis, interstitial lung diseases, progressive pulmonary fibrosis, treatable traitRespirology (Carlton, Vic.)
Effect of Macitentan in Pulmonary Arterial Hypertension and the Relationship Between Echocardiography and cMRI Variables: REPAIR Echocardiography Sub-study Results.Torbicki A, Channick R, Galiè N, Kiely DG, Moceri P, Peacock A, Swift AJ, Tawakol A, Vonk Noordegraaf A, Flores D, Martin N, Rosenkranz S2024-03-01Agreement, Cardiac magnetic resonance imaging, Correlation, Echocardiography, Macitentan, Pulmonary arterial hypertensionCardiology and therapy
Impact of Air Pollution and MUC5B Genotype on Survival in Idiopathic Pulmonary Fibrosis.Sesé L, Borie R, Kannengiesser C, Cottin V, Israel-Biet D, Crestani B, Cadranel J, Chenivesse C, Boubaya M, Valeyre D, Annesi-Maesano I, Nunes H2024-03-01Annals of the American Thoracic Society
Risk Stratification in Pulmonary Veno-Occlusive Disease.Cruz-Utrilla A, Pérez-Olivares C, Luna-López R, Jiménez López-Guarch C, Bedate P, Martínez Meñaca A, López Meseguer M, Escribano-Subias P2024-02-24Archivos de bronconeumologia
Zinpentraxin Alfa for Idiopathic Pulmonary Fibrosis: The Randomized Phase III STARSCAPE Trial.Richeldi L, Schiffman C, Behr J, Inoue Y, Corte T, Cottin V, Jenkins RG, Nathan SD, Raghu G, Walsh SLF, Jayia PK, Kamath N, Martinez FJ2024-02-14Antifibrotic medication, Forced vital capacity (FVC), Futility analysis, Interstitial lung disease, Linear mixed-effects modelAmerican journal of respiratory and critical care medicine
Reversal of cylindrical bronchial dilatations in a subset of adults with cystic fibrosis treated with elexacaftor-tezacaftor-ivacaftor.Cazier P, Chassagnon G, Dhote T, Da Silva J, Kanaan R, Honore I, Carlier N, Revel MP, Canniff E, Martin C, Burgel PR2024-02-08The European respiratory journal
Role of Oxygen Starvation in Right Ventricular Decompensation and Failure in Pulmonary Arterial Hypertension.Oknińska M, Zajda K, Zambrowska Z, Grzanka M, Paterek A, Mackiewicz U, Szczylik C, Kurzyna M, Piekiełko-Witkowska A, Torbicki A, Kieda C, Mączewski M2024-02-01angiogenesis, hypertrophy, oxygen deficiency, pulmonary arterial hypertension, right ventricular failureJACC. Heart failure
A formula for predicting emphysema extent in combined idiopathic pulmonary fibrosis and emphysema.Wells AU, Jacob J, Sverzellati N, Cross G, Barnett J, De Lauretis A, Antoniou K, Weycker D, Atwood M, Kirchgaessler KU, Cottin V2024-01-18Clinical trial cohort, Interstitial lung disease, Pulmonary function test, Radiology, Real-world cohortRespiratory research
Publisher Correction: Pulmonary hypertension.Mocumbi A, Humbert M, Saxena A, Jing ZC, Sliwa K, Thienemann F, Archer SL, Stewart S2024-01-17Nature reviews. Disease primers
Standards for the care of people with cystic fibrosis (CF); recognising and addressing CF health issues.Burgel PR, Southern KW, Addy C, Battezzati A, Berry C, Bouchara JP, Brokaar E, Brown W, Azevedo P, Durieu I, Ekkelenkamp M, Finlayson F, Forton J, Gardecki J, Hodkova P, Hong G, Lowdon J, Madge S, Martin C, McKone E, Munck A, Ooi CY, Perrem L, Piper A, Prayle A, Ratjen F, Rosenfeld M, Sanders DB, Schwarz C, Taccetti G, Wainwright C, West NE, Wilschanski M, Bevan A, Castellani C, Drevinek P, Gartner S, Gramegna A, Lammertyn E, Landau EEC, Plant BJ, Smyth AR, van Koningsbruggen-Rietschel S, Middleton PG2024-01-16Breathing, CFRD, Complications, Cystic fibrosis, End of life, Liver, Lung infection, Mental health, Pulmonary, TransplantJournal of cystic fibrosis : official journal of the European Cystic Fibrosis Society
Pulmonary hypertension.Mocumbi A, Humbert M, Saxena A, Jing ZC, Sliwa K, Thienemann F, Archer SL, Stewart S2024-01-04Nature reviews. Disease primers
Mepolizumab and benralizumab in patients with severe asthma and a history of eosinophilic granulomatosis with polyangiitis.Desaintjean C, Ahmad K, Traclet J, Gerfaud-Valentin M, Durel CA, Glerant JC, Hot A, Lestelle F, Mainbourg S, Nasser M, Seve P, Turquier S, Devouassoux G, Cottin V2024-01-01Interleukin-5, asthma, corticosteroids, eosinophil, granulomatosis, vasculitisFrontiers in medicine
Abnormal functional lymphoid tolerance and enhanced myeloid exocytosis are characteristics of resting and stimulated PBMCs in cystic fibrosis patients.Gaudin C, Ghinnagow R, Lemaire F, Villeret B, Sermet-Gaudelus I, Sallenave JM2024-01-01PBMCs, Pseudomonas aeruginosa, cystic fibrosis, low-density neutrophils, lymphocyte, proteases, toleranceFrontiers in immunology
Simplified risk stratification based on cardiopulmonary exercise test: A Spanish two-center experience.Martínez-Meñaca A, Cruz-Utrilla A, Mora-Cuesta VM, Luna-López R, Segura-de la Cal T, Flox-Camacho Á, Alonso-Lecue P, Escribano-Subias P, Cifrián-Martínez JM2024-01-016 min walking test, cardiopulmonary exercise test, pulmonary arterial hypertension, risk assessmentPulmonary circulation
Gathering real-world compassionate data to expand eligibility for elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis with N1303K or other rare CFTR variants: a viewpoint.Burgel PR, Sermet-Gaudelus I, Girodon E, Kanaan R, Le Bihan J, Remus N, Ravoninjatovo B, Grenet D, Porzio M, Houdouin V, Le Clainche-Viala L, Durieu I, Nove-Josserand R, Languepin J, Coltey B, Guillaumot A, Audousset C, Chiron R, Weiss L, Fajac I, Da Silva J, Martin C2024-01-01The European respiratory journal
Expert management of congenital portosystemic shunts and their complications.McLin VA, Franchi-Abella S, Brütsch T, Bahadori A, Casotti V, de Ville de Goyet J, Dumery G, Gonzales E, Guérin F, Hascoet S, Heaton N, Kuhlmann B, Lador F, Lambert V, Marra P, Plessier A, Quaglia A, Rougemont AL, Savale L, Sarma MS, Sitbon O, Superina RA, Uchida H, van Albada M, Johannes van der Doef HP, Vilgrain V, Wacker J, Zwaveling N, Debray D, Wildhaber BE2024-01-01adenoma, congenital portosystemic shunt, focal nodular hyperplasia, hepatocellular carcinoma, hyperandrogenism, hyperinsulinism, hypoglycemia, occlusion test, portal pressure, puberty, pulmonary hypertension, β-cateninJHEP reports : innovation in hepatology
A BEAT-PCD consensus statement: a core outcome set for pulmonary disease interventions in primary ciliary dyskinesia.Kos R, Goutaki M, Kobbernagel HE, Rubbo B, Shoemark A, Aliberti S, Altenburg J, Anagnostopoulou P, Athanazio RA, Beydon N, Dell SD, Emiralioglu N, Ferkol TW, Loebinger MR, Lorent N, Maître B, Marthin J, Morgan LC, Nielsen KG, Ringshausen FC, Shteinberg M, Tiddens HAWM, Maitland-Van der Zee AH, Chalmers JD, Lucas JSA, Haarman EG2024-01-01ERJ open research
[Genetic diffuse cystic lung disease in adults].Diesler R, Ahmad K, Chalabreysse L, Glérant JC, Harzallah I, Touraine R, Si-Mohamed S, Cottin V2024-01-01Birt-Hogg-Dubé, Birt-Hogg-Dubé syndrome, Diffuse cystic lung disease, Genetic, Génétique, Interstitial lung disease, Lymphangioleiomyomatosis, Lymphangioléiomyomatose, Maladie kystique pulmonaire, Pneumopathie interstitielle diffuse, Sclérose tubéreuse de Bourneville, Tuberous sclerosis complexRevue des maladies respiratoires
Infections in autoimmune pulmonary alveolar proteinosis: a large retrospective cohort.Mabo A, Borie R, Wemeau-Stervinou L, Uzunhan Y, Gomez E, Prevot G, Reynaud-Gaubert M, Traclet J, Bergot E, Cadranel J, Marchand-Adam S, Bergeron A, Blanchard E, Bondue B, Bonniaud P, Bourdin A, Burgel PR, Hirschi S, Marquette CH, Quétant S, Nunes H, Chenivesse C, Crestani B, Guirriec Y, Monnier D, Ménard C, Tattevin P, Cottin V, Luque Paz D, Jouneau S2023-12-15GM-CSF autoantibody, bacterial Infection, opportunist lung infections, pulmonary alveolar proteinosisThorax
The Utility of a Resting Electrocardiogram (ECG-PH Index) in Evaluating the Efficacy of Pulmonary Endarterectomy in Chronic Thromboembolic Pulmonary Hypertension.Piłka M, Darocha S, Florczyk M, Mańczak R, Banaszkiewicz M, Kędzierski P, Zieliński D, Wróbel K, Torbicki A, Kurzyna M2023-12-11chronic thromboembolic pulmonary hypertension, electrocardiography, pulmonary endarterectomy, pulmonary hypertensionJournal of clinical medicine
Rituximab to treat prolidase deficiency due to a novel pathogenic copy number variation in PEPD.Atschekzei F, Fedchenko M, Elsayed A, Dubrowinskaja N, Graalmann T, Ringshausen FC, Witte T, Sogkas G2023-12-07Autoimmune Diseases, Immune System Diseases, Rituximab, Sjogren's SyndromeRMD open
European pathways of care in Alpha-1 Antitrypsin deficiency.Chorostowska-Wynimko J, Koczulla AR, Sucena M2023-12-01Alpha-1 antitrypsin deficiency, Diagnosis, European, SurveyRespiratory medicine
Long-term safety and efficacy of elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis and at least one F508del allele: 144-week interim results from a 192-week open-label extension study.Daines CL, Tullis E, Costa S, Linnemann RW, Mall MA, McKone EF, Polineni D, Quon BS, Ringshausen FC, Rowe SM, Selvadurai H, Taylor-Cousar JL, Withers NJ, Ahluwalia N, Moskowitz SM, Prieto-Centurion V, Tan YV, Tian S, Weinstock T, Xuan F, Zhang Y, Ramsey B, Griese M2023-12-01The European respiratory journal
Monographic Issue on Pulmonary Hypertension.Barberà JA, Humbert M2023-12-01Seminars in respiratory and critical care medicine
Extracorporeal Life Support in Pulmonary Hypertension: Practical Aspects.Hoeper MM2023-12-01Seminars in respiratory and critical care medicine
Pulmonary Hypertension Associated with Left Heart Disease.Baratto C, Caravita S, Vachiéry JL2023-12-01Seminars in respiratory and critical care medicine
Updated Hemodynamic Definition and Classification of Pulmonary Hypertension.Lechartier B, Kularatne M, Jaïs X, Humbert M, Montani D2023-12-01Seminars in respiratory and critical care medicine
Monographic Issue on Pulmonary Hypertension: Medical and Interventional Treatment for Chronic Thromboembolic Pulmonary Hypertension.Delcroix M, Belge C, Maleux G, Godinas L2023-12-01Seminars in respiratory and critical care medicine
The Right Ventricle in Pulmonary Hypertension.Wessels JN, Celant LR, de Man FS, Vonk Noordegraaf A2023-12-01Seminars in respiratory and critical care medicine
Management of Pulmonary Hypertension Associated with Chronic Lung Disease.Blanco I, Hernández-González F, García A, Torres-Castro R, Barberà JA2023-12-01Seminars in respiratory and critical care medicine
Management of Acutely Decompensated Pulmonary Hypertension.Savale L, Kularatne M, Roche A, Pichon J, Baron A, Boucly A, Sitbon O, Humbert M2023-12-01Seminars in respiratory and critical care medicine
Ciliary Ultrastructure Assessed by Transmission Electron Microscopy in Adults with Bronchiectasis and Suspected Primary Ciliary Dyskinesia but Inconclusive Genotype.Staar BO, Hegermann J, Auber B, Ewen R, von Hardenberg S, Olmer R, Pink I, Rademacher J, Wetzke M, Ringshausen FC2023-11-18bronchiectasis, genotype–phenotype correlation, primary ciliary dyskinesia, transmission electron microscopy, ultrastructure, whole-exome sequencingCells
Monitoring of ECFS quality standards for the clinical management of adults with cystic fibrosis.Gramegna A, Aliberti S, Amorim A, Blasi F, Bourke S, Burgel PR, Diamantea F, Durieu I, Fila L, Moreno RMG, Messore B, Pokojová E, Taccetti G, Verhulst S, Dugac AV, Wege S, Duff A, Southern KW, Castellani C2023-11-08Adulthood, Cystic fibrosis, Cystic fibrosis centers, Quality standards, Standards of careJournal of cystic fibrosis : official journal of the European Cystic Fibrosis Society
Leukocyte telomere length: the dawn of a new era of personalised medicine in fibrotic interstitial lung diseases?Cottin V, Kolb M2023-11-01The European respiratory journal
Outcome of lung transplantation for adults with interstitial lung disease associated with genetic disorders of the surfactant system.Bermudez J, Nathan N, Coiffard B, Roux A, Hirschi S, Degot T, Bunel V, Le Pavec J, Macey J, Le Borgne A, Legendre M, Cottin V, Thomas PA, Borie R, Reynaud-Gaubert M2023-11-01ERJ open research
Idiopathic pulmonary fibrosis with benign SFTPC variant and pathogenic MARS1 mutations: can't see the forest for the trees!Castaldo A, Delestrain C, Diesler R, Merveilleux du Vignaux C, Onnee M, Touraine R, Chalabreysse L, Fanen P, Epaud R, Cottin V, De Becdelièvre A2023-11-01ERJ open research
Viewpoint: activin signalling inhibitors for the treatment of pulmonary arterial hypertension.Humbert M2023-11-01The European respiratory journal
Defining the clinical validity of genes reported to cause pulmonary arterial hypertension.Welch CL, Aldred MA, Balachandar S, Dooijes D, Eichstaedt CA, Gräf S, Houweling AC, Machado RD, Pandya D, Prapa M, Shaukat M, Southgate L, Tenorio-Castano J, Chung WK2023-11-01Genetics, Genomic medicine, Molecular diagnosis, Pulmonary arterial hypertensionGenetics in medicine : official journal of the American College of Medical Genetics
Nonsense mutations accelerate lung disease and decrease survival of cystic fibrosis children.Orenti A, Pranke I, Faucon C, Varilh J, Hatton A, Golec A, Dehillotte C, Durieu I, Reix P, Burgel PR, Grenet D, Tasset C, Gachelin E, Perisson C, Lepissier A, Dreano E, Tondelier D, Chevalier B, Weiss L, Kiefer S, Laurans M, Chiron R, Lemonnier L, Marguet C, Jung A, Edelman A, Kerem BS, Girodon E, Taulan-Cadars M, Hinzpeter A, Kerem E, Naehrlich L, Sermet-Gaudelus I2023-11-01Cystic fibrosis (MeSH: DO008550), Cystic fibrosis transmembrane conductance regulator (MeSH: DO19005), Premature termination codon (MeSH: DO:18389)Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society
Bronchial Stenosis After Lung Transplantation From cDCD Donors Using Simultaneous Abdominal Normothermic Regional Perfusion: A Single-center Experience.Mora-Cuesta VM, Tello-Mena S, Izquierdo-Cuervo S, Iturbe-Fernández D, Sánchez-Moreno L, Ballesteros MA, Alonso-Lecue P, Ortíz-Portal F, Ferrer-Pargada D, Miñambres-García E, Cifrián-Martínez JM, Naranjo-Gozalo S2023-11-01Transplantation
Exploring intrinsic variability between cultured nasal and bronchial epithelia in cystic fibrosis.Rodenburg LW, Metzemaekers M, van der Windt IS, Smits SMA, den Hertog-Oosterhoff LA, Kruisselbrink E, Brunsveld JE, Michel S, de Winter-de Groot KM, van der Ent CK, Stadhouders R, Beekman JM, Amatngalim GD2023-10-30Scientific reports
Seven Additional Patients with SOX17 Related Pulmonary Arterial Hypertension and Review of the Literature.Gallego-Zazo N, Miranda-Alcaraz L, Cruz-Utrilla A, Del Cerro Marín MJ, Álvarez-Fuente M, Del Mar Rodríguez Vázquez Del Rey M, Guillén Rodríguez I, Becerra-Munoz VM, Moya-Bonora A, Ochoa Parra N, Parra A, Pascual P, Cazalla M, Silván C, Arias P, Valverde D, de Jesús-Pérez V, Lapunzina P, Escribano-Subías P, Tenorio-Castano J2023-10-20SOX17, cardiovascular disorders, congenital heart disease, genomic medicine, personalized medicine, pulmonary arterial hypertension, whole exome sequencingGenes
Theratyping cystic fibrosis patients to guide elexacaftor/tezacaftor/ivacaftor out-of-label prescription.Dreano E, Burgel PR, Hatton A, Bouazza N, Chevalier B, Macey J, Leroy S, Durieu I, Weiss L, Grenet D, Stremler N, Ohlmann C, Reix P, Porzio M, Roux Claude P, Rémus N, Douvry B, Montcouquiol S, Cosson L, Mankikian J, Languepin J, Houdouin V, Le Clainche L, Guillaumot A, Pouradier D, Tissot A, Priou P, Mély L, Chedevergne F, Lebourgeois M, Lebihan J, Martin C, Zavala F, Da Silva J, Lemonnier L, Kelly-Aubert M, Golec A, Foucaud P, Marguet C, Edelman A, Hinzpeter A, de Carli P, Girodon E, Sermet-Gaudelus I, Pranke I2023-10-01The European respiratory journal
Prognostic Stratification and Treatment for Pulmonary Arterial Hypertension. Gaps and Promising Remedies.Del Pozo R, Cruz-Utrilla A, Escribano-Subias P2023-10-01Archivos de bronconeumologia
Protocol for generating airway organoids from 2D air liquid interface-differentiated nasal epithelia for use in a functional CFTR assay.Rodenburg LW, van der Windt IS, Dreyer HHM, Smits SMA, den Hertog-Oosterhoff LA, Aarts EM, Beekman JM, Amatngalim GD2023-09-15Cell culture, OrganoidsSTAR protocols
The European reference network on rare lung diseases (ERN-LUNG) ambition to offer better lung health for all.Humbert M, Wagner T2023-09-10Presse medicale (Paris, France : 1983)
Design of a phase III, double-blind, randomised, placebo-controlled trial of BI 1015550 in patients with progressive pulmonary fibrosis (FIBRONEER-ILD).Maher TM, Assassi S, Azuma A, Cottin V, Hoffmann-Vold AM, Kreuter M, Oldham JM, Richeldi L, Valenzuela C, Wijsenbeek MS, Coeck C, Schlecker C, Voss F, Wachtlin D, Martinez FJ2023-09-01Interstitial Fibrosis, Rare lung diseasesBMJ open respiratory research
Treatment of pulmonary arterial hypertension: recent progress and a look to the future.Humbert M, Sitbon O, Guignabert C, Savale L, Boucly A, Gallant-Dewavrin M, McLaughlin V, Hoeper MM, Weatherald J2023-09-01The Lancet. Respiratory medicine
Interstitial lung disease-associated pulmonary hypertension - what the future holds.Cottin V, Diesler R, Turquier S, Valenzuela C2023-09-01Current opinion in pulmonary medicine
Pulmonary alveolar proteinosis - current and future therapeutical strategies.Jehn LB, Bonella F2023-09-01Current opinion in pulmonary medicine
Autoantibodies against the NineTeen complex and U5 RNP in systemic sclerosis.Vulsteke JB, Smith V, Bonroy C, Lenaerts JL, Derua R, Wuyts WA, Blockmans D, De Haes P, Piette Y, De Langhe E, Bossuyt X2023-09-01Journal of autoimmunity
Comprehensive Clinical Diagnostic Pipelines Reveal New Variants in Alpha-1 Antitrypsin Deficiency.Ottaviani S, Bartoli G, Carroll TP, Gangemi F, Balderacchi AM, Barzon V, Corino A, Piloni D, McElvaney NG, Corsico AG, Irving JA, Fra A, Ferrarotti I2023-09-01SERPINA1 rare variants, alpha-1 antitrypsin polymers, pathogenicity predictions, serpinopathies, serpinsAmerican journal of respiratory cell and molecular biology
Emerging Role of Balloon Pulmonary Angioplasty in Chronic Thromboembolic Pulmonary Disease-Insights from the 2022 ESC Guidelines.Banaszkiewicz M, Kurzyna P, Kubikowska N, Mucha M, Rudnik A, Gąsecka A, Pietrasik A, Grabowski M, Jaguszewski MJ, Kasprzyk P, Kędzierski P, Ciećwierz D, Żuk G, Szwed P, Piłka M, Florczyk M, Kurzyna M, Darocha S2023-08-16balloon pulmonary angioplasty, chronic thromboembolic pulmonary disease, chronic thromboembolic pulmonary hypertension, pulmonary endarterectomy, pulmonary hypertensionJournal of clinical medicine
Design of a phase III, double-blind, randomised, placebo-controlled trial of BI 1015550 in patients with idiopathic pulmonary fibrosis (FIBRONEER-IPF).Richeldi L, Azuma A, Cottin V, Kreuter M, Maher TM, Martinez FJ, Oldham JM, Valenzuela C, Gordat M, Liu Y, Stowasser S, Zoz DF, Wijsenbeek MS2023-08-01Lung TransplantationBMJ open respiratory research
Alpha1-antitrypsin deficiency: An updated review.Mornex JF, Traclet J, Guillaud O, Dechomet M, Lombard C, Ruiz M, Revel D, Reix P, Cottin V2023-07-29Presse medicale (Paris, France : 1983)
Cystic fibrosis.Fajac I, Burgel PR2023-07-27Bronchiectasis, CFTR modulators, Cystic fibrosis transmembrane conductance regulator (CFTR), Pseudomonas aeruginosaPresse medicale (Paris, France : 1983)
Optimal treatment of the underlying aetiology is the most effective antimicrobial stewardship for chronic respiratory disease: a lesson learned from cystic fibrosis.Rademacher J, Martin L, Theloe A, Stahl M, Mall MA, Joean O, Fuge J, Hansen G, Welte T, Schütz K, Ringshausen FC, Dittrich AM2023-07-01ERJ open research
Diagnosis and management of pulmonary veno-occlusive disease.Solinas S, Boucly A, Beurnier A, Kularatne M, Grynblat J, Eyries M, Dorfmüller P, Sitbon O, Humbert M, Montani D2023-07-01EIF2AK4, Pulmonary veno-occlusive disease, chemotherapy, pulmonary edema, pulmonary hypertension, solvents, transplantationExpert review of respiratory medicine
Treatable traits: a comprehensive precision medicine approach in interstitial lung disease.Khor YH, Cottin V, Holland AE, Inoue Y, McDonald VM, Oldham J, Renzoni EA, Russell AM, Strek ME, Ryerson CJ2023-07-01The European respiratory journal
Lung Function Decline in Cystic Fibrosis: Impact of Data Availability and Modeling Strategies on Clinical Interpretations.Szczesniak R, Andrinopoulou ER, Su W, Afonso PM, Burgel PR, Cromwell E, Gecili E, Ghulam E, Goss CH, Mayer-Hamblett N, Keogh RH, Liou TG, Marshall B, Morgan WJ, Ostrenga JS, Pasta DJ, Stanojevic S, Wainwright C, Zhou GC, Fernandez G, Fink AK, Schechter MS2023-07-01epidemiology, longitudinal analysis, pulmonary function, rate of decline, registry analysisAnnals of the American Thoracic Society
CT imaging shows specific pancreatic abnormalities in persons with cystic fibrosis related diabetes.Alexandre-Heymann L, Puerto M, Martin C, Burnet E, Mosnier-Pudar H, Burgel PR, Larger E2023-06-27Scientific reports
Inhaled treprostinil for interstitial lung disease-associated pulmonary hypertension: a silver lining on a very dark cloud.Cottin V, Valenzuela C, Humbert M2023-06-01The European respiratory journal
Transforming clinical research and science in bronchiectasis: EMBARC3, a European Respiratory Society Clinical Research Collaboration.Chalmers JD, Aliberti S, Altenburg J, Blasi F, Clarke C, Chotirmall SH, Crichton ML, Dhar R, Goeminne P, Haworth C, Loebinger MR, Lorent N, Polverino E, Ringshausen FC, Shoemark A, Shteinberg M, Sibila O, Spinou A, Welte T2023-06-01The European respiratory journal
Evidence-Based Guideline for the diagnosis and management of eosinophilic granulomatosis with polyangiitis.Emmi G, Bettiol A, Gelain E, Bajema IM, Berti A, Burns S, Cid MC, Cohen Tervaert JW, Cottin V, Durante E, Holle JU, Mahr AD, Del Pero MM, Marvisi C, Mills J, Moiseev S, Moosig F, Mukhtyar C, Neumann T, Olivotto I, Salvarani C, Seeliger B, Sinico RA, Taillé C, Terrier B, Venhoff N, Bertsias G, Guillevin L, Jayne DRW, Vaglio A2023-06-01Nature reviews. Rheumatology
French recommendations for the diagnosis and management of lymphangioleiomyomatosis.Cottin V, Blanchard E, Kerjouan M, Lazor R, Reynaud-Gaubert M, Taille C, Uzunhan Y, Wemeau L, Andrejak C, Baud D, Bonniaud P, Brillet PY, Calender A, Chalabreysse L, Court-Fortune I, Desbaillets NP, Ferretti G, Guillemot A, Hardelin L, Kambouchner M, Leclerc V, Lederlin M, Malinge MC, Mancel A, Marchand-Adam S, Maury JM, Naccache JM, Nasser M, Nunes H, Pagnoux G, Prévot G, Rousset-Jablonski C, Rouviere O, Si-Mohamed S, Touraine R, Traclet J, Turquier S, Vagnarelli S, Ahmad K2023-06-01Angiomyolipoma, Lymphangioleiomyomatosis, Pneumothorax, Sirolimus, Tuberous sclerosisRespiratory medicine and research
Validating organoid-derived human intestinal monolayers for personalized therapy in cystic fibrosis.Birimberg-Schwartz L, Ip W, Bartlett C, Avolio J, Vonk AM, Gunawardena T, Du K, Esmaeili M, Beekman JM, Rommens J, Strug L, Bear CE, Moraes TJ, Gonska T2023-06-01Life science alliance
French practical guidelines for the diagnosis and management of idiopathic pulmonary fibrosis - 2021 update. Full-length version.Cottin V, Bonniaud P, Cadranel J, Crestani B, Jouneau S, Marchand-Adam S, Nunes H, Wémeau-Stervinou L, Bergot E, Blanchard E, Borie R, Bourdin A, Chenivesse C, Clément A, Gomez E, Gondouin A, Hirschi S, Lebargy F, Marquette CH, Montani D, Prévot G, Quetant S, Reynaud-Gaubert M, Salaun M, Sanchez O, Trumbic B, Berkani K, Brillet PY, Campana M, Chalabreysse L, Chatté G, Debieuvre D, Ferretti G, Fourrier JM, Just N, Kambouchner M, Legrand B, Le Guillou F, Lhuillier JP, Mehdaoui A, Naccache JM, Paganon C, Rémy-Jardin M, Si-Mohamed S, Terrioux P2023-06-01Biopsy, Interstitial lung disease, Pulmonary fibrosis, Usual interstitial pneumoniaRespiratory medicine and research
Usefulness of genetics for clinical reclassification and refinement of prognostic stratification in pulmonary arterial hypertension.Cruz-Utrilla A, Gallego-Zazo N, Pérez-Olivares C, Hernández-González I, Bedate P, Martínez Meñaca A, López Meseguer M, Lapunzina P, Pérez Núñez M, Ochoa Parra N, Valverde D, Tenorio-Castaño JA, Escribano-Subias P2023-06-01Enfermedad venooclusiva pulmonar, Genetics, Genética, Hipertensión arterial pulmonar, Pulmonary arterial hypertension, Pulmonary veno-oclusive diseaseRevista espanola de cardiologia (English ed.)
Primary Ciliary Dyskinesia Patient-Specific hiPSC-Derived Airway Epithelium in Air-Liquid Interface Culture Recapitulates Disease Specific Phenotypes In Vitro.von Schledorn L, Puertollano Martín D, Cleve N, Zöllner J, Roth D, Staar BO, Hegermann J, Ringshausen FC, Nawroth J, Martin U, Olmer R2023-05-24PCD, air-liquid interface culture, human induced pluripotent stem cellsCells
Pseudomonas aeruginosa population genomics among adults with bronchiectasis across Germany.Rosenboom I, Oguz S, Lüdemann IM, Ringshausen FC, Rademacher J, Sedlacek L, Tümmler B, Cramer N2023-05-01ERJ open research
The RISE study protocol: resilience impacted by positive stressful events for people with cystic fibrosis.van der Heijden E, van den Bor RM, van der Ent CK, Nijhof SL, van der Laan SEI2023-05-01ERJ open research
The U UGA C sequence provides a favorable context to ELX-02 induced CFTR readthrough.Pranke IM, Varilh J, Hatton A, Faucon C, Girodon E, Dreano E, Chevalier B, Karri S, Reix P, Durieu I, Bidou L, Namy O, Taulan M, Hinzpeter A, Sermet-Gaudelus I2023-05-01Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society
Long COVID syndrome after SARS-CoV-2 survival in patients with pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension.Wieteska-Miłek M, Kuśmierczyk-Droszcz B, Betkier-Lipińska K, Szmit S, Florczyk M, Zieliński P, Hoffman P, Krzesińki P, Kurzyna M2023-04-01COVID‐19, chronic thromboembolic pulmonary hypertension, long COVID syndrome, pulmonary arterial hypertension, risk of deathPulmonary circulation
Epidemiological trends in nontuberculous mycobacterial infection among people with cystic fibrosis in Germany.Steindor M, Hafkemeyer S, Ruckes C, Stehling F, Naehrlich L, Ringshausen FC2023-04-01Cystic fibrosis transmembrane conductance regulator, Epidemiology, Germany, Mycobacterium abscessus, Mycobacterium avium, RegistriesInternational journal of infectious diseases : IJID : official publication of the International Society for Infectious Diseases
CFTR Modulators: Current Status and Evolving Knowledge.Regard L, Martin C, Da Silva J, Burgel PR2023-04-01Seminars in respiratory and critical care medicine
The world of rare interstitial lung diseases.Buschulte K, Cottin V, Wijsenbeek M, Kreuter M, Diesler R2023-03-31European respiratory review : an official journal of the European Respiratory Society
Ordinal outcomes add value to clinical trials - Authors' reply.Weatherald J, Humbert M2023-03-25Lancet (London, England)
European Respiratory Society statement on familial pulmonary fibrosis.Borie R, Kannengiesser C, Antoniou K, Bonella F, Crestani B, Fabre A, Froidure A, Galvin L, Griese M, Grutters JC, Molina-Molina M, Poletti V, Prasse A, Renzoni E, van der Smagt J, van Moorsel CHM2023-03-01The European respiratory journal
Progressive pulmonary fibrosis: an expert group consensus statement.Rajan SK, Cottin V, Dhar R, Danoff S, Flaherty KR, Brown KK, Mohan A, Renzoni E, Mohan M, Udwadia Z, Shenoy P, Currow D, Devraj A, Jankharia B, Kulshrestha R, Jones S, Ravaglia C, Quadrelli S, Iyer R, Dhooria S, Kolb M, Wells AU2023-03-01The European respiratory journal
GLPG1205 for idiopathic pulmonary fibrosis: a phase 2 randomised placebo-controlled trial.Strambu IR, Seemayer CA, Fagard LMA, Ford PA, Van der Aa TAK, de Haas-Amatsaleh AA, Modgill V, Santermans E, Sondag EN, Helmer EG, Maher TM, Costabel U, Cottin V2023-03-01The European respiratory journal
PDE5 to keep them alive: The use of phosphodiesterase type-5 inhibitors in severe pulmonary hypertension associated with interstitial lung disease.Weatherald J, Humbert M2023-03-01interstitial lung disease, phosphodiesterase type 5 inhibitor, pulmonary fibrosis, pulmonary hypertension, sildenafil, tadalafil, treatmentRespirology (Carlton, Vic.)
Lung Dual-Energy CT Perfusion Blood Volume as a Marker of Severity in Chronic Thromboembolic Pulmonary Hypertension.Si-Mohamed SA, Zumbihl L, Turquier S, Boccalini S, Mornex JF, Douek P, Cottin V, Boussel L2023-02-17X-ray computed/methods, comparative study, lung, perfusion, tomographyDiagnostics (Basel, Switzerland)
The French Compassionate Program of elexacaftor-tezacaftor-ivacaftor in people with cystic fibrosis with advanced lung disease and no F508del CFTR variant.Burgel PR, Sermet-Gaudelus I, Durieu I, Kanaan R, Macey J, Grenet D, Porzio M, Coolen-Allou N, Chiron R, Marguet C, Douvry B, Dufeu N, Danner-Boucher I, Foucaud P, Lemonnier L, Girodon E, Da Silva J, Martin C2023-02-16The European respiratory journal
Proteomic profiling of sweat in patients with cystic fibrosis provides new insights into epidermal homoeostasis.Cornet M, Nguyen-Khoa T, Kelly-Aubert M, Jung V, Chedevergne F, Le Bourgeois M, Aoust L, Roger K, Guerrera CI, Sermet-Gaudelus I2023-02-01Skin health and disease
Identification of new telomere- and telomerase-associated autoantigens in systemic sclerosis.Vulsteke JB, Smith V, Bonroy C, Derua R, Blockmans D, De Haes P, Vanderschueren S, Lenaerts JL, Claeys KG, Wuyts WA, Verschueren P, Vanhandsaeme G, Piette Y, De Langhe E, Bossuyt X2023-02-01Autoantibodies, Mass spectrometry, Systemic sclerosis, Telomerase, TelomereJournal of autoimmunity
Towards Translation of PqsR Inverse Agonists: From In Vitro Efficacy Optimization to In Vivo Proof-of-Principle.Hamed MM, Abdelsamie AS, Rox K, Schütz C, Kany AM, Röhrig T, Schmelz S, Blankenfeldt W, Arce-Rodriguez A, Borrero-de Acuña JM, Jahn D, Rademacher J, Ringshausen FC, Cramer N, Tümmler B, Hirsch AKH, Hartmann RW, Empting M2023-02-01Pseudomonas aeruginosa, bronchiectasis, in vivo proof-of-concept, pathoblocker, quorum sensingAdvanced science (Weinheim, Baden-Wurttemberg, Germany)
Qualitative and quantitative evaluation of computed tomography changes in adults with cystic fibrosis treated with elexacaftor-tezacaftor-ivacaftor: a retrospective observational study.Dettmer S, Weinheimer O, Sauer-Heilborn A, Lammers O, Wielpütz MO, Fuge J, Welte T, Wacker F, Ringshausen FC2023-01-01CFTR, computed tomography, cystic fibrosis, elexacaftor-tezacaftor-ivacaftor, quantitative CT, therapy responseFrontiers in pharmacology
A practical approach to the 2022 ESC cardio-oncology guidelines: Comments by a team of experts - cardiologists and oncologists.Leszek P, Klotzka A, Bartuś S, Burchardt P, Czarnecka AM, Długosz-Danecka M, Gierlotka M, Koseła-Paterczyk H, Krawczyk-Ożóg A, Kubiatowski T, Kurzyna M, Maciejczyk A, Mitkowski P, Prejbisz A, Rutkowski P, Sierko E, Sterliński M, Szmit S, Szwiec M, Tajstra M, Tycińska A, Witkowski A, Wojakowski W, Cybulska-Stopa B2023-01-01Kardiologia polska
Case report: Transected Hickman catheter and its thrombotic occlusion in a patient with idiopathic pulmonary arterial hypertension-can a catheter replacement be avoided?Sławiński G, Zieleniewicz P, Faran A, Dąbrowska-Kugacka A, Kurzyna M, Kempa M, Daniłowicz-Szymanowicz L, Lewicka E2023-01-01epoprostenol, hickman and broviac catheters, pulmonary hypertension, repair kit, thrombosisFrontiers in cardiovascular medicine
Spirometric and anthropometric improvements in response to elexacaftor/tezacaftor/ivacaftor depending on age and lung disease severity.Schütz K, Pallenberg ST, Kontsendorn J, DeLuca D, Sukdolak C, Minso R, Büttner T, Wetzke M, Dopfer C, Sauer-Heilborn A, Ringshausen FC, Junge S, Tümmler B, Hansen G, Dittrich AM2023-01-01BMI, cystic fibrosis, elexacaftor/tezacaftor/ivacaftor, modulator, pediatricFrontiers in pharmacology
ECMO in pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension as a bridge to therapy.Cruz-Utrilla A, García-Martín EP, Domínguez Pérez L, Ruiz Curiel A, Quezada A, Durante López A, Vicent L, Martín Asenjo R, Hinojosa W, Eixerés A, Forcén Acebal L, Galindo M, Arribas Ynsaurriaga F, Escribano-Subias P, Bueno H2023-01-01Kardiologia polska
COVID-19 in patients with pulmonary alveolar proteinosis: a European multicentre study.Papiris SA, Campo I, Mariani F, Kallieri M, Kolilekas L, Papaioannou AI, Gonca Chousein E, Cetinkaya E, Bonella F, Borie R, Kokosi M, Pickworth T, Molina-Molina M, Gasa M, Radzikowska E, Fijolek J, Jouneau S, Gomez E, McCarthy C, Bendstrup E, Piotrowski WJ, Pabary R, Hadchouel A, Coolen-Allou N, Alfaro T, Robalo Cordeiro C, Antonogiannaki EM, Tomos IP, Papakosta D, Kontakiotis T, Panagiotou P, Douros K, Schams A, Lettieri S, Papaevangelou V, Kanaka-Gantenbein C, Karakatsani A, Loukides S, Costabel U, Crestani B, Morgan C, Tazawa R, Bush A, Griese M, Manali ED2023-01-01ERJ open research
Lung transplantation for end-stage cystic fibrosis before and after the availability of elexacaftor-tezacaftor-ivacaftor, Germany, 2012-2021.Ringshausen FC, Sauer-Heilborn A, Büttner T, Dittrich AM, Schwerk N, Ius F, Nährlich L, Welte T, Greer M2023-01-01The European respiratory journal
Normalisation of circulating neutrophil counts after 12 months of elexacaftor-tezacaftor-ivacaftor in patients with advanced cystic fibrosis.Dhote T, Martin C, Regard L, Pesenti L, Kanaan R, Carlier N, Honoré I, Da Silva J, Witko-Sarsat V, Burgel PR2023-01-01The European respiratory journal
The Changing Epidemiology of Cystic Fibrosis: The Implications for Adult Care.Burgel PR, Burnet E, Regard L, Martin C2023-01-01cystic fibrosis, cystic fibrosis transmembrane conductance regulator, lung transplantation, registryChest
Crosstalk between cilia and autophagy: implication for human diseases.Morleo M, Vieira HLA, Pennekamp P, Palma A, Bento-Lopes L, Omran H, Lopes SS, Barral DC, Franco B2023-01-01Autophagy, cilia, human diseases, lysosome, macroautophagy, mitochondriaAutophagy
Redefining Hypo- and Hyper-Responding Phenotypes of CFTR Mutants for Understanding and Therapy.Hillenaar T, Beekman J, van der Sluijs P, Braakman I2022-12-02CFTR, hyper-responders, hypo-responding mutants, modulators, protein foldingInternational journal of molecular sciences
European Respiratory Society guidelines on transbronchial lung cryobiopsy in the diagnosis of interstitial lung diseases.Korevaar DA, Colella S, Fally M, Camuset J, Colby TV, Hagmeyer L, Hetzel J, Maldonado F, Morais A, Ravaglia C, Spijker R, Tomassetti S, Troy LK, Verschakelen JA, Wells AU, Tonia T, Annema JT, Poletti V2022-11-01The European respiratory journal
Drug Repurposing for Cystic Fibrosis: Identification of Drugs That Induce CFTR-Independent Fluid Secretion in Nasal Organoids.Rodenburg LW, Delpiano L, Railean V, Centeio R, Pinto MC, Smits SMA, van der Windt IS, van Hugten CFJ, van Beuningen SFB, Rodenburg RNP, van der Ent CK, Amaral MD, Kunzelmann K, Gray MA, Beekman JM, Amatngalim GD2022-10-21TMEM16A, cystic fibrosis, drug repurposing, nasal organoids, screening assayInternational journal of molecular sciences
Phenotypes of idiopathic pulmonary arterial hypertension.Cruz-Utrilla A, Pérez-Olivares C, Martínez-Meñaca A, López-Meseguer M, Escribano-Subias P2022-10-01The Lancet. Respiratory medicine
Drug-induced interstitial lung disease.Spagnolo P, Bonniaud P, Rossi G, Sverzellati N, Cottin V2022-10-01The European respiratory journal
Pharmacological chaperone-rescued cystic fibrosis CFTR-F508del mutant overcomes PRAF2-gated access to endoplasmic reticulum exit sites.Saha K, Chevalier B, Doly S, Baatallah N, Guilbert T, Pranke I, Scott MGH, Enslen H, Guerrera C, Chuon C, Edelman A, Sermet-Gaudelus I, Hinzpeter A, Marullo S2022-09-27Arginin-based retention motif, BRET, CFTR, Corrector, ERES, Gatekeeper, Protein export, Sec 24, Sec 31Cellular and molecular life sciences : CMLS
Clinical Implications of the Genetic Background in Pediatric Pulmonary Arterial Hypertension: Data from the Spanish REHIPED Registry.Cruz-Utrilla A, Gallego-Zazo N, Tenorio-Castaño JA, Guillén I, Torrent-Vernetta A, Moya-Bonora A, Labrandero C, Rodríguez-Monte MEG, Rodríguez-Ogando A, Rey MDMRVD, Espín J, Plata-Izquierdo B, Álvarez-Fuente M, Moreno-Galdó A, Escribano-Subias P, Marín MJDC2022-09-09genetics, heritable pulmonary arterial hypertension, pediatric pulmonary hypertension, pulmonary veno-occlusive diseaseInternational journal of molecular sciences
[Test Your Knowledge].Angelini E, Dettmer S, Goecke T, Länger F, Ringshausen FC2022-09-01Pneumologie (Stuttgart, Germany)
Clinical impact of TERT somatic mutation in telomerase-related gene mutation carriers after lung transplantation.Ba I, Kannengiesser C, Mal H, Reynaud-Gaubert M, Cottin V, Hirschi S, Picard C, Borie R2022-09-01TERT somatic mutation, digital droplet PCR, idiopathic pulmonary fibrosis, lung transplantation, telomerase-related geneThe Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation
[French practical guidelines for the diagnosis and management of IPF - 2021 update, full version].Cottin V, Bonniaud P, Cadranel J, Crestani B, Jouneau S, Marchand-Adam S, Nunes H, Wémeau-Stervinou L, Bergot E, Blanchard E, Borie R, Bourdin A, Chenivesse C, Clément A, Gomez E, Gondouin A, Hirschi S, Lebargy F, Marquette CH, Montani D, Prévot G, Quetant S, Reynaud-Gaubert M, Salaun M, Sanchez O, Trumbic B, Berkani K, Brillet PY, Campana M, Chalabreysse L, Chatté G, Debieuvre D, Ferretti G, Fourrier JM, Just N, Kambouchner M, Legrand B, Le Guillou F, Lhuillier JP, Mehdaoui A, Naccache JM, Paganon C, Rémy-Jardin M, Si-Mohamed S, Terrioux P2022-09-01Biopsie, Biopsy, Common interstitial lung disease, Fibrose pulmonaire, Interstitial lung disease, Pneumopathie interstitielle commune, Pneumopathie interstitielle diffuse, Pulmonary fibrosisRevue des maladies respiratoires
Differential CFTR-Interactome Proximity Labeling Procedures Identify Enrichment in Multiple SLC Transporters.Chevalier B, Baatallah N, Najm M, Castanier S, Jung V, Pranke I, Golec A, Stoven V, Marullo S, Antigny F, Guerrera IC, Sermet-Gaudelus I, Edelman A, Hinzpeter A2022-08-11CFTR, KCNK3, SLC transporters, cystic fibrosis, interactome, proximity labelingInternational journal of molecular sciences
Measuring cystic fibrosis drug responses in organoids derived from 2D differentiated nasal epithelia.Amatngalim GD, Rodenburg LW, Aalbers BL, Raeven HH, Aarts EM, Sarhane D, Spelier S, Lefferts JW, Silva IA, Nijenhuis W, Vrendenbarg S, Kruisselbrink E, Brunsveld JE, van Drunen CM, Michel S, de Winter-de Groot KM, Heijerman HG, Kapitein LC, Amaral MD, van der Ent CK, Beekman JM2022-08-03Life science alliance
Integrating Clinical Probability into the Diagnostic Approach to Idiopathic Pulmonary Fibrosis: An International Working Group Perspective.Cottin V, Tomassetti S, Valenzuela C, Walsh SLF, Antoniou KM, Bonella F, Brown KK, Collard HR, Corte TJ, Flaherty KR, Johannson KA, Kolb M, Kreuter M, Inoue Y, Jenkins RG, Lee JS, Lynch DA, Maher TM, Martinez FJ, Molina-Molina M, Myers JL, Nathan SD, Poletti V, Quadrelli S, Raghu G, Rajan SK, Ravaglia C, Remy-Jardin M, Renzoni E, Richeldi LK, Spagnolo P, Troy L, Wijsenbeek M, Wilson KC, Wuyts W, Wells AU, Ryerson CJ2022-08-01American journal of respiratory and critical care medicine
Diversity of approaches in artificial intelligence: an opportunity for discoveries in thoracic imaging.Chassagnon G, Campredon A, Vakalopoulou M, Burgel PR2022-07-01The European respiratory journal
Inflammation biomarkers in sputum for clinical trials in cystic fibrosis: current understanding and gaps in knowledge.Lepissier A, Addy C, Hayes K, Noel S, Bui S, Burgel PR, Dupont L, Eickmeier O, Fayon M, Leal T, Lopes C, Downey DG, Sermet-Gaudelus I2022-07-01cystic fibrosis, inflammation, sputum biomarkersJournal of cystic fibrosis : official journal of the European Cystic Fibrosis Society
Embolization of Recurrent Pulmonary Arteriovenous Malformations by Ethylene Vinyl Alcohol Copolymer (Onyx®) in Hereditary Hemorrhagic Telangiectasia: Safety and Efficacy.Si-Mohamed SA, Cierco A, Gamondes D, Restier LM, Delagrange L, Cottin V, Dupuis-Girod S, Revel D2022-06-30Rendu–Osler–Weber disease, arteriovenous malformations, embolization, hereditary hemorrhagic telangiectasia, thoraxJournal of personalized medicine
Trial of a Preferential Phosphodiesterase 4B Inhibitor for Idiopathic Pulmonary Fibrosis.Richeldi L, Azuma A, Cottin V, Hesslinger C, Stowasser S, Valenzuela C, Wijsenbeek MS, Zoz DF, Voss F, Maher TM2022-06-09The New England journal of medicine
Respiratory management of spinal muscular atrophy type 1 patients treated with Nusinersen.Menard J, Seferian AM, Fleurence E, Barzic A, Binoche A, Labouret G, Coutier L, Vuillerot C, Bieleu BM, Gomez Garcia de la Banda M, Corvol H, Servais L, Taytard J2022-06-01children, nocturnal gas exchange, noninvasive ventilation, nusinersen, respiratory management, spinal muscular atrophy type 1Pediatric pulmonology
CFTR Modulators in People with Cystic Fibrosis: Real-World Evidence in France.Regard L, Martin C, Burnet E, Da Silva J, Burgel PR2022-05-28CFTR modulators, cystic fibrosis, elexacaftor, ivacaftor, real-world studies, tezacaftorCells
The role of cardiopulmonary exercise testing in identifying and monitoring pulmonary veno-oclusive disease: a case report with ING.Segura de la Cal T, Pérez-Olivares C, Cristo Ropero MJ, Luna López R, Escribano-Subías P2022-05-01CPET, Cardiopulmonary exercise test, EIF2AK4, Pulmonary arterial hypertension, Pulmonary veno-occlusive diseaseEuropean heart journal. Case reports
Respiratory failure due to concomitant interstitial lung disease and diaphragmatic involvement in a patient with anti-MDA5 dermatomyositis: a case report.Grignaschi S, Mongodi S, Alfonsi E, Mojoli F, Vertui V, Zanframundo G, Cavagna L2022-05-01Clinical and experimental rheumatology
Sustained effectiveness of elexacaftor-tezacaftor-ivacaftor in lung transplant candidates with cystic fibrosis.Martin C, Reynaud-Gaubert M, Hamidfar R, Durieu I, Murris-Espin M, Danner-Boucher I, Chiron R, Leroy S, Douvry B, Grenet D, Mely L, Ramel S, Montcouquiol S, Lemonnier L, Burnet E, Paillasseur JL, Da Silva J, Burgel PR2022-05-01Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society
Systemic bis-phosphinic acid derivative restores chloride transport in Cystic Fibrosis mice.da Cunha MF, Pranke I, Sassi A, Schreiweis C, Moriceau S, Vidovic D, Hatton A, Carlon MS, Creste G, Berhal F, Prestat G, Freund R, Odolczyk N, Jais JP, Gravier-Pelletier C, Zielenkiewicz P, Jullien V, Hinzpeter A, Oury F, Edelman A, Sermet-Gaudelus I2022-04-12Scientific reports
Safety and tolerability of nintedanib in patients with progressive fibrosing interstitial lung diseases: data from the randomized controlled INBUILD trial.Cottin V, Martinez FJ, Jenkins RG, Belperio JA, Kitamura H, Molina-Molina M, Tschoepe I, Coeck C, Lievens D, Costabel U2022-04-07Adverse drug event, Clinical trial, Diarrhea, Patient adherence, Pulmonary fibrosisRespiratory research
Targeted therapy for pulmonary alveolar proteinosis: the time is now.Bonella F, Borie R2022-04-01The European respiratory journal
Anetumab ravtansine versus vinorelbine in patients with relapsed, mesothelin-positive malignant pleural mesothelioma (ARCS-M): a randomised, open-label phase 2 trial.Kindler HL, Novello S, Bearz A, Ceresoli GL, Aerts JGJV, Spicer J, Taylor P, Nackaerts K, Greystoke A, Jennens R, Calabrò L, Burgers JA, Santoro A, Cedrés S, Serwatowski P, Ponce S, Van Meerbeeck JP, Nowak AK, Blumenschein G, Siegel JM, Kasten L, Köchert K, Walter AO, Childs BH, Elbi C, Hassan R, Fennell DA2022-04-01The Lancet. Oncology
Tobramycin safety and efficacy review article.Schwarz C, Taccetti G, Burgel PR, Mulrennan S2022-04-01Cystic fibrosis, FEV(1), Hospitalizations, Pseudomonas aeruginosa, Sputum density, TobramycinRespiratory medicine
Determinants of survival after lung transplantation in telomerase-related gene mutation carriers: A retrospective cohort.Phillips-Houlbracq M, Mal H, Cottin V, Gauvain C, Beier F, Sicre de Fontbrune F, Sidali S, Mornex JF, Hirschi S, Roux A, Weisenburger G, Roussel A, Wémeau-Stervinou L, Le Pavec J, Pison C, Marchand Adam S, Froidure A, Lazor R, Naccache JM, Jouneau S, Nunes H, Reynaud-Gaubert M, Le Borgne A, Boutboul D, Ba I, Boileau C, Crestani B, Kannengiesser C, Borie R2022-04-01clinical research, fibrosis, lung disease, lung transplantation, practice, pulmonology, transplant social workerAmerican journal of transplantation : official journal of the American Society of Transplantation and the American Society of Transplant Surgeons
European Respiratory Society International Congress 2021: highlights from best-abstract awardees.Ramakrishnan S, Beaufils F, De Brandt J, Viney K, Bradley C, Cottin V, Hassan M, Cruz J2022-03-01Breathe (Sheffield, England)
Pulmonary thromboendarterectomy in chronic thromboembolic pulmonary hypertension: the Spanish experience.López-Gude MJ, Blanco I, Benito-Arnáiz V, Castellà M, Escribano-Subías P, Martin C, Barberà JA, Cortina-Romero JM2022-03-01Pulmonary thromboendarterectomy, pulmonary hypertension (PH), pulmonary vascular resistance (PVR), residual pulmonary hypertension, operabilityAnnals of cardiothoracic surgery
[French practical guidelines for the diagnosis and management of IPF - 2021 update, short version].Cottin V, Bonniaud P, Cadranel J, Crestani B, Jouneau S, Marchand-Adam S, Nunes H, Wémeau-Stervinou L, Bergot E, Blanchard E, Borie R, Bourdin A, Chenivesse C, Clément A, Gomez E, Gondouin A, Hirschi S, Lebargy F, Marquette CH, Montani D, Prévot G, Quetant S, Reynaud-Gaubert M, Salaun M, Sanchez O, Trumbic B, Berkani K, Brillet PY, Campana M, Chalabreysse L, Chatté G, Debieuvre D, Ferretti G, Fourrier JM, Just N, Kambouchner M, Legrand B, Le Guillou F, Lhuillier JP, Mehdaoui A, Naccache JM, Paganon C, Rémy-Jardin M, Si-Mohamed S, Terrioux P2022-03-01Biopsie, Biopsy, Common interstitial lung disease, Fibrose pulmonaire, Interstitial lung disease, Pneumopathie interstitielle commune, Pneumopathie interstitielle diffuse, Pulmonary fibrosisRevue des maladies respiratoires
Interstitial lung diseases associated with mutations of poly(A)-specific ribonuclease: A multicentre retrospective study.Philippot Q, Kannengiesser C, Debray MP, Gauvain C, Ba I, Vieri M, Gondouin A, Naccache JM, Reynaud-Gaubert M, Uzunhan Y, Bondue B, Israël-Biet D, Dieudé P, Fourrage C, Lainey E, Manali E, Papiris S, Wemeau L, Hirschi S, Mal H, Nunes H, Schlemmer F, Blanchard E, Beier F, Cottin V, Crestani B, Borie R2022-03-01PARN, idiopathic interstitial pneumonia, idiopathic pulmonary fibrosis, interstitial lung disease, mutation, poly(A)-specific ribonuclease, telomere homeostasis, variantRespirology (Carlton, Vic.)
Major Decrease in Lung Transplantation for Patients with Cystic Fibrosis in France.Martin C, Legeai C, Regard L, Cantrelle C, Dorent R, Carlier N, Kerbaul F, Burgel PR2022-03-01American journal of respiratory and critical care medicine
Natural history of respiratory muscle strength in spinal muscular atrophy: a prospective national cohort study.Veldhoen ES, Wijngaarde CA, Hulzebos EHJ, Wösten-van Asperen RM, Wadman RI, van Eijk RPA, Asselman FL, Stam M, Otto LAM, Cuppen I, Scheijmans FEV, den Oudenrijn LPV, Bartels B, Gaytant MA, van der Ent CK, van der Pol WL2022-02-21Lung function, Natural history, Neuromuscular, Respiratory muscle strength, Spinal muscular atrophyOrphanet journal of rare diseases
Pirfenidone in Unclassifiable Interstitial Lung Disease: A Subgroup Analysis by Concomitant Mycophenolate Mofetil and/or Previous Corticosteroid Use.Kreuter M, Maher TM, Corte TJ, Molina-Molina M, Axmann J, Gilberg F, Kirchgaessler KU, Cottin V2022-02-01Corticosteroid, Immunomodulator, Mycophenolate mofetil, Pirfenidone, Unclassifiable interstitial lung diseaseAdvances in therapy
Sjögren disease, not Sjögren's: comment on the article by Baer and Hammitt.Cottin V2022-02-01Arthritis & rheumatology (Hoboken, N.J.)
Efficacy of Pirfenidone vs. Placebo in Unclassifiable Interstitial Lung Disease, by Surgical Lung Biopsy Status: Data From a post-hoc Analysis.Molina-Molina M, Kreuter M, Cottin V, Corte TJ, Gilberg F, Kirchgaessler KU, Axmann J, Maher TM2022-01-01lung function, pirfenidone, post-hoc analysis, surgical lung biopsy, unclassifiable interstitial lung diseaseFrontiers in medicine
Are anaplastic lymphoma kinase (ALK) and O6-methylguanine-DNA methyltransferase (MGMT) promoter methylation driver biomarkers of pulmonary neuroendocrine tumors (NETs) and carcinomas (NECs)?Hiddinga B, Zwaenepoel K, Janssens A, Van Meerbeeck J, Pauwels P2022-01-01MGMT promoter methylation, anaplastic lymphoma kinase, neuroendocrine carcinoma, neuroendocrine tumor, small cell lung cancerOncotarget
The Burden of Progressive-Fibrosing Interstitial Lung Diseases.Cottin V, Teague R, Nicholson L, Langham S, Baldwin M2022-01-01economic burden, epidemiology, humanistic burden, progressive fibrosing ILD, quality of life, survivalFrontiers in medicine
Post hoc Analysis of Clinical Outcomes in Placebo- and Pirfenidone-Treated Patients with IPF Stratified by BMI and Weight Loss.Jouneau S, Crestani B, Thibault R, Lederlin M, Vernhet L, Yang M, Morgenthien E, Kirchgaessler KU, Cottin V2022-01-01Body composition, Body mass index, Idiopathic pulmonary fibrosis, Interstitial lung diseaseRespiration; international review of thoracic diseases
A call for evidence in connective tissue diseases-associated interstitial lung disease.Cottin V, Lega JC, Coury F, Nasser M2022-01-01Evidence, Guidelines, Interstitial Lung Disease, Pulmonary fibrosis, Recommendations, Sjogren diseaseJoint bone spine
Real-world assessment of LCI following lumacaftor-ivacaftor initiation in adolescents and adults with cystic fibrosis.Reix P, Tatopoulos A, Ioan I, Le Bourgeois M, Bui S, Choukroun ML, Bessaci-Kabouya K, Gerardin M, Bokov P, Da Silva J, Paillasseur JL, Burgel PR2022-01-01CFTR modulators, Cystic fibrosis, Lumacaftor, Multiple breath washoutJournal of cystic fibrosis : official journal of the European Cystic Fibrosis Society
Impact of a high emergency lung transplantation programme for cystic fibrosis in France: insight from a comparison with Canada.Coriati A, Sykes J, Lemonnier L, Ma X, Stanojevic S, Dehillotte C, Carlier N, Stephenson AL, Burgel PR2022-01-01The European respiratory journal
Interstitial pneumonia with autoimmune features: challenges and controversies.Mackintosh JA, Wells AU, Cottin V, Nicholson AG, Renzoni EA2021-12-31European respiratory review : an official journal of the European Respiratory Society
Clinical utility of diagnostic biomarkers in malignant pleural mesothelioma: a systematic review and meta-analysis.Schillebeeckx E, van Meerbeeck JP, Lamote K2021-12-31European respiratory review : an official journal of the European Respiratory Society
Spectral Photon-Counting CT Technology in Chest Imaging.Si-Mohamed SA, Miailhes J, Rodesch PA, Boccalini S, Lacombe H, Leitman V, Cottin V, Boussel L, Douek P2021-12-09computed tomography, diagnostic imaging, lung, photon-counting detectors, thoraxJournal of clinical medicine
Using chest CT scan and unsupervised machine learning for predicting and evaluating response to lumacaftor-ivacaftor in people with cystic fibrosis.Campredon A, Battistella E, Martin C, Durieu I, Mely L, Marguet C, Belleguic C, Murris-Espin M, Chiron R, Fanton A, Bui S, Reynaud-Gaubert M, Reix P, Hoang-Thi TN, Vakalopoulou M, Revel MP, Da Silva J, Burgel PR, Chassagnon G2021-11-18The European respiratory journal
Long-term amikacin liposome inhalation suspension in cystic fibrosis patients with chronic P. aeruginosa infection.Bilton D, Fajac I, Pressler T, Clancy JP, Sands D, Minic P, Cipolli M, Galeva I, Solé A, Quittner AL, Jumadilova Z, Ciesielska M, Konstan MW2021-11-01ALIS, Amikacin, Cystic fibrosis, Liposomal, Pseudomonas aeruginosaJournal of cystic fibrosis : official journal of the European Cystic Fibrosis Society
Patient perspectives following initiation of elexacaftor-tezacaftor-ivacaftor in people with cystic fibrosis and advanced lung disease.Martin C, Burnet E, Ronayette-Preira A, de Carli P, Martin J, Delmas L, Prieur B, Burgel PR2021-11-01Advanced CF disease, CFTR modulator, Grounded theory, Patient perspectives, Patient-reported outcomes, Quality of lifeRespiratory medicine and research
Involvement of CFTR in the pathogenesis of pulmonary arterial hypertension.Le Ribeuz H, To L, Ghigna MR, Martin C, Nagaraj C, Dreano E, Rucker-Martin C, Girerd B, Bouligand J, Pechoux C, Lambert M, Boet A, Issard J, Mercier O, Hoetzenecker K, Manoury B, Becq F, Burgel PR, Cottart CH, Olschewski A, Sermet-Gaudelus I, Perros F, Humbert M, Montani D, Antigny F2021-11-01The European respiratory journal
[ care atlas for people with rare diseases].Neff M, Schaaf J, Tegtbauer N, Schäfer J, Till M, Wagner TOF, Graeßner H, Mundlos C, Storf H2021-10-01Care centers, Health facilities, Health personnel, Patient portals, Rare diseasesDer Internist
Tuberous sclerosis complex for the pulmonologist.Rebaine Y, Nasser M, Girerd B, Leroux C, Cottin V2021-09-30European respiratory review : an official journal of the European Respiratory Society
Recent developments in the treatment of small cell lung cancer.Hiddinga BI, Raskin J, Janssens A, Pauwels P, Van Meerbeeck JP2021-09-30European respiratory review : an official journal of the European Respiratory Society
Improved survival albeit with persistent disparities in prognosis for people with cystic fibrosis in European countries.Martin C, Burgel PR2021-09-01The European respiratory journal
Looking forward: key initiatives to improve the care of rare diseases and streamline the delivery of medicines and vaccines in Europe.Humbert M, Simonds AK2021-09-01ERS, European Respiratory Society, WLD2021, World Lung Day, pulmonary medicineAmerican journal of physiology. Lung cellular and molecular physiology
Updated guidance on the management of children with cystic fibrosis transmembrane conductance regulator-related metabolic syndrome/cystic fibrosis screen positive, inconclusive diagnosis (CRMS/CFSPID).Barben J, Castellani C, Munck A, Davies JC, de Winter-de Groot KM, Gartner S, Kashirskaya N, Linnane B, Mayell SJ, McColley S, Ooi CY, Proesmans M, Ren CL, Salinas D, Sands D, Sermet-Gaudelus I, Sommerburg O, Southern KW2021-09-01CFSPID, CRMS, Cystic fibrosis, Management, Newborn screeningJournal of cystic fibrosis : official journal of the European Cystic Fibrosis Society
Misconceptions regarding symptoms of sarcoidosis.Drent M, Costabel U, Crouser ED, Grunewald J, Bonella F2021-08-01The Lancet. Respiratory medicine
Reply to Kuek et al.: Optimism with Caution: Elexacaftor-Tezacaftor-Ivacaftor in Patients with Advanced Pulmonary Disease.Burgel PR, Da Silva J, Paillasseur JL, Martin C2021-08-01American journal of respiratory and critical care medicine
Radiological differences between chronic thromboembolic pulmonary disease (CTEPD) and chronic thromboembolic pulmonary hypertension (CTEPH).Capone C, Valentini A, Spinillo SL, Klersy C, Celentano A, Pin M, Monterosso C, Dore R, Bassi EM, Zacchino M, Rodolico G, Corsico AG, Preda L, Ghio S, D'Armini AM2021-08-01Dual-energy computed tomography, Pulmonary embolism, Pulmonary hypertensionEuropean radiology
Low income and outcome in idiopathic pulmonary fibrosis: An association to uncover.Sesé L, Caliez J, Annesi-Maesano I, Cottin V, Pesce G, Didier M, Carton Z, Israel-Biet D, Crestani B, Dudoret SG, Cadranel J, Wallaert B, Tazi A, Maître B, Prévot G, Marchand-Adam S, Hirschi S, Dury S, Giraud V, Gondouin A, Bonniaud P, Traclet J, Juvin K, Borie R, Bernaudin JF, Valeyre D, Cavalin C, Nunes H2021-07-01Air pollution, Idiopathic pulmonary fibrosis, Income, Occupational exposure, Socioeconomic statusRespiratory medicine
Care Delivery Models and Interstitial Lung Disease: The Role of the Specialized Center.Fisher JH, Cottin V2021-06-01Interstitial lung disease, Multidisciplinary team, Specialized centerClinics in chest medicine
The role of cardiopulmonary exercise test in identifying pulmonary veno-occlusive disease.Pérez-Olivares C, Segura de la Cal T, Flox-Camacho Á, Nuche J, Tenorio J, Martínez Meñaca A, Cruz-Utrilla A, de la Cruz-Bertolo J, Pérez Núñez M, Consortium SP, Arribas-Ynsaurriaga F, Escribano Subías P2021-06-01The European respiratory journal
Mental Well-being and General Health in Adolescents with Asthma: The Prevention and Incidence of Asthma and Mite Allergy Birth Cohort Study.van der Laan SEI, de Hoog MLA, Nijhof SL, Gehring U, Vonk JM, van der Ent CK, Wijga AH2021-06-01The Journal of pediatrics
Estimates of epidemiology, mortality and disease burden associated with progressive fibrosing interstitial lung disease in France (the PROGRESS study).Nasser M, Larrieu S, Boussel L, Si-Mohamed S, Bazin F, Marque S, Massol J, Thivolet-Bejui F, Chalabreysse L, Maucort-Boulch D, Hachulla E, Jouneau S, Le Lay K, Cottin V2021-05-24Algorithms, Epidemiology, Healthcare resource utilisation, Interstitial lung disease, Progressive fibrosisRespiratory research
PH CARE COVID survey: an international patient survey on the care for pulmonary hypertension patients during the early phase of the COVID-19 pandemic.Godinas L, Iyer K, Meszaros G, Quarck R, Escribano-Subias P, Vonk Noordegraaf A, Jansa P, D'Alto M, Luknar M, Milutinov Ilic S, Belge C, Sitbon O, Reis A, Rosenkranz S, Pepke-Zaba J, Humbert M, Delcroix M2021-05-01COVID-19, Chronic thromboembolic pulmonary hypertension, Patient survey, Pulmonary arterial hypertension, Pulmonary hypertensionOrphanet journal of rare diseases
Expectations about treatment of idiopathic pulmonary fibrosis: Comparative survey of patients, carers and physicians (the RESPIR French survey).Cottin V, Bergot E, Bourdin A, Nunes H, Prévot G, Wallaert B, Marchand-Adam S2021-05-01Drug therapy, Idiopathic pulmonary fibrosis, Management, Patient and carer experience, SurveyRespiratory medicine and research
Educational aspects of rare and orphan lung diseases.Alfaro TM, Wijsenbeek MS, Powell P, Stolz D, Hurst JR, Kreuter M, Moor CC2021-03-24Delayed diagnosis, Medical education, Medical societies, Patient education, Patient participation, Rare diseasesRespiratory research
Safety of G-CSF with concurrent chemo-radiotherapy in limited-stage small cell lung cancer - Secondary analysis of the randomised phase 3 CONVERT trial.Gomes F, Faivre-Finn C, Mistry H, Bezjak A, Pourel N, Fournel P, Van Meerbeeck J, Blackhall F2021-03-01Concurrent chemo-radiotherapy, G-CSF, SCLC, Small cell lung cancer, cCTRTLung cancer (Amsterdam, Netherlands)
Clinical response to lumacaftor-ivacaftor in patients with cystic fibrosis according to baseline lung function.Burgel PR, Durieu I, Chiron R, Mely L, Prevotat A, Murris-Espin M, Porzio M, Abely M, Reix P, Marguet C, Macey J, Sermet-Gaudelus I, Corvol H, Bui S, Biouhee T, Hubert D, Munck A, Lemonnier L, Dehillotte C, Silva JD, Paillasseur JL, Martin C2021-03-01Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society
Cystic Fibrosis Newborn Screening in Austria Using PAP and the Numeric Product of PAP and IRT Concentrations as Second-Tier Parameters.Zeyda M, Schanzer A, Basek P, Bauer V, Eber E, Ellemunter H, Kallinger M, Riedler J, Thir C, Wadlegger F, Zacharasiewicz A, Renner S2021-02-13IRT-PAP, IRT×PAP, false-positives, neonatal screening, recallsDiagnostics (Basel, Switzerland)
Hemodynamic Response to Treatment and Outcomes in Pulmonary Hypertension Associated With Interstitial Lung Disease Versus Pulmonary Arterial Hypertension in Systemic Sclerosis: Data From a Study Identifying Prognostic Factors in Pulmonary Hypertension Associated With Interstitial Lung Disease.Chauvelot L, Gamondes D, Berthiller J, Nieves A, Renard S, Catella-Chatron J, Ahmad K, Bertoletti L, Camara B, Gomez E, Launay D, Montani D, Mornex JF, Prévot G, Sanchez O, Schott AM, Subtil F, Traclet J, Turquier S, Zeghmar S, Habib G, Reynaud-Gaubert M, Humbert M, Cottin V2021-02-01Arthritis & rheumatology (Hoboken, N.J.)
Radiological Findings in Multidetector Computed Tomography (MDCT) of Hereditary and Sporadic Pulmonary Veno-Occlusive Disease: Certainties and Uncertainties.Pérez Núñez M, Alonso Charterina S, Pérez-Olivares C, Revilla Ostolaza Y, Morales Ruiz R, Enguita Valls AB, Tenorio JA, Gallego Zazo N, De Pablo Gafas A, Lapunzina P, Rodríguez Chaverri A, Escribano Subías P2021-01-19multidetector computed tomography (MDCT), pulmonary hypertension, pulmonary veno-occlusive diseaseDiagnostics (Basel, Switzerland)
Mortality and Respiratory-Related Hospitalizations in Idiopathic Pulmonary Fibrosis Not Treated With Antifibrotics.Cottin V, Spagnolo P, Bonniaud P, Nolin M, Dalon F, Kirchgässler KU, Kamath TV, Van Ganse E, Belhassen M2021-01-01acute hospitalizations, antifibrotics, claims data, idiopathic pulmonary fibrosis, mortalityFrontiers in medicine
Variability in Global Prevalence of Interstitial Lung Disease.Kaul B, Cottin V, Collard HR, Valenzuela C2021-01-01epidemiology—descriptive, global epidemiology, idiopathic pulmonary fibrosis, interstitial lung disease, mortalityFrontiers in medicine
Epidemiology, Mortality and Healthcare Resource Utilization Associated With Systemic Sclerosis-Associated Interstitial Lung Disease in France.Cottin V, Larrieu S, Boussel L, Si-Mohamed S, Bazin F, Marque S, Massol J, Thivolet-Bejui F, Chalabreysse L, Maucort-Boulch D, Jouneau S, Hachulla E, Chollet J, Nasser M2021-01-01cost, epidemiology, pulmonary fibrosis, scleroderma, systemic sclerosisFrontiers in medicine
Gender Differences in Idiopathic Pulmonary Fibrosis: Are Men and Women Equal?Sesé L, Nunes H, Cottin V, Israel-Biet D, Crestani B, Guillot-Dudoret S, Cadranel J, Wallaert B, Tazi A, Maître B, Prévot G, Marchand-Adam S, Hirschi S, Dury S, Giraud V, Gondouin A, Bonniaud P, Traclet J, Juvin K, Borie R, Carton Z, Freynet O, Gille T, Planès C, Valeyre D, Uzunhan Y2021-01-01gender differences, idiopathic pulmonary fibrosis, lung transplantation, occupational exposures, womenFrontiers in medicine
The Experience, Prerequisites, and the Barriers in Organizing a Specialized Rehabilitation Program for Patients with Pulmonary Hypertension.Palevičiūtė E, Gumbienė L, Jurevičienė E, Šimbelytė T, Laucevičienė I, Laucevičius A, Barysienė J, Eichstaedt CA, Benjamin N, Grünig E, Čelutkienė J2021-01-01Exercise program, Implementation, Pulmonary hypertension, Rehabilitation, TrainingRespiration; international review of thoracic diseases
Concurrence of 1- and 3-Min Sit-to-Stand Tests with the 6-Min Walk Test in Idiopathic Pulmonary Fibrosis.Fedi A, Keddache S, Quétant S, Guillien A, Antoniadis A, Soumagne T, Ritter O, Glérant JC, Cottin V, Degano B, Aguilaniu B2021-01-016-min walk test, Idiopathic pulmonary fibrosis, Sit-to-stand testsRespiration; international review of thoracic diseases
Update on Respiratory Fungal Infections in Cystic Fibrosis Lung Disease and after Lung Transplantation.Renner S, Nachbaur E, Jaksch P, Dehlink E2020-12-21ABPA, antifungals, fungi, respiratory exacerbationJournal of fungi (Basel, Switzerland)
Quantification of Cystic Fibrosis Lung Disease with Radiomics-based CT Scores.Chassagnon G, Zacharaki EI, Bommart S, Burgel PR, Chiron R, Dangeard S, Paragios N, Martin C, Revel MP2020-12-01Radiology. Cardiothoracic imaging
Reduced Intestinal Inflammation With Lumacaftor/Ivacaftor in Adolescents With Cystic Fibrosis.Tétard C, Mittaine M, Bui S, Beaufils F, Maumus P, Fayon M, Burgel PR, Lamireau T, Delhaes L, Mas E, Enaud R2020-12-01Journal of pediatric gastroenterology and nutrition
Feasibility of a Noninvasive Operability Assessment in Chronic Thromboembolic Pulmonary Hypertension under Real-World Practice.Rodriguez Chaverri A, Revilla Ostolaza Y, Lopez-Gude MJ, Velazquez MT, Ponz de Antonio I, Alonso Charterina S, Albarran Gonzalez-Trevilla A, Perez Nunez M, Perez Vela JL, Morales Ruiz R, Delgado Jimenez JF, Arribas Ynsaurriaga F, Cortina JM, Escribano Subias P2020-10-21endarterectomy, hypertension, pulmonary, pulmonary embolismDiagnostics (Basel, Switzerland)
Urinary Exosomes of Patients with Cystic Fibrosis Unravel CFTR-Related Renal Disease.Gauthier S, Pranke I, Jung V, Martignetti L, Stoven V, Nguyen-Khoa T, Semeraro M, Hinzpeter A, Edelman A, Guerrera IC, Sermet-Gaudelus I2020-09-10cystic fibrosis, cystic fibrosis transmembrane conductance regulator, kidney, klotho, proteomicInternational journal of molecular sciences
Current treatment of sarcoidosis.Sellarés J, Francesqui J, Llabres M, Hernandez-Gonzalez F, Baughman RP2020-09-01Current opinion in pulmonary medicine
R117H-CFTR function and response to VX-770 correlate with mRNA and protein expression in intestinal organoids.Van Mourik P, van Haaren P, Kruisselbrink E, Korkmaz C, Janssens HM, de Winter-de Groot KM, van der Ent CK, Hagemeijer MC, Beekman JM2020-09-01CFTR, Organoids, R117H, VX-770, mRNAJournal of cystic fibrosis : official journal of the European Cystic Fibrosis Society
Prediction of Prognostic Hemodynamic Indices in Pulmonary Hypertension Using Non-Invasive Parameters.Mańczak R, Kurzyna M, Piłka M, Darocha S, Florczyk M, Wieteska-Miłek M, Mańczak M, Torbicki A2020-08-27biomarkers, echocardiography, logistic regression, prognostic indices, pulmonary hypertension, right heart catheterization, risk stratificationDiagnostics (Basel, Switzerland)
Desquamative interstitial pneumonia: a systematic review of its features and outcomes.Hellemons ME, Moor CC, von der Thüsen J, Rossius M, Odink A, Thorgersen LH, Verschakelen J, Wuyts W, Wijsenbeek MS, Bendstrup E2020-06-30European respiratory review : an official journal of the European Respiratory Society
Diagnostic approach of fibrosing interstitial lung diseases of unknown origin.Cottin V, Valenzuela C2020-06-01Presse medicale (Paris, France : 1983)
Patients' and Healthcare Professionals' Experiences of Idiopathic Pulmonary Fibrosis Treatment with the Pirfenidone 801 mg Tablet Formulation: A Multinational Survey.Lancaster LH, Valenzuela C, Mason W, Neurohr C, Ripamonti E, Kirchgaessler KU, Cottin V2020-06-01Antifibrotics, Idiopathic pulmonary fibrosis, Pill burden, Pirfenidone, Quality of life, QuestionnairePulmonary therapy
Impact of COVID-19 on people with cystic fibrosis.Colombo C, Burgel PR, Gartner S, van Koningsbruggen-Rietschel S, Naehrlich L, Sermet-Gaudelus I, Southern KW2020-05-01The Lancet. Respiratory medicine
Antimicrobial resistance in cystic fibrosis: A Delphi approach to defining best practices.Zemanick E, Burgel PR, Taccetti G, Holmes A, Ratjen F, Byrnes CA, Waters VJ, Bell SC, VanDevanter DR, Stuart Elborn J, Flume PA2020-05-01Antimicrobial resistance, Consensus, Cystic fibrosisJournal of cystic fibrosis : official journal of the European Cystic Fibrosis Society
Getting neural about airway gland secretion.Amatngalim GD, Ribeiro CMP2020-04-01The European respiratory journal
Prolidase deficiency: a new genetic cause of combined pulmonary fibrosis and emphysema syndrome in the adult.Cottin V, Nasser M, Traclet J, Chalabreysse L, Lèbre AS, Si-Mohamed S, Philit F, Thivolet-Béjui F2020-04-01The European respiratory journal
CheckMate 171: A phase 2 trial of nivolumab in patients with previously treated advanced squamous non-small cell lung cancer, including ECOG PS 2 and elderly populations.Felip E, Ardizzoni A, Ciuleanu T, Cobo M, Laktionov K, Szilasi M, Califano R, Carcereny E, Griffiths R, Paz-Ares L, Duchnowska R, Garcia MA, Isla D, Jassem J, Appel W, Milanowski J, Van Meerbeeck JP, Wolf J, Li A, Acevedo A, Popat S2020-03-01Comorbidity, Elderly, Health status indicators, Nivolumab, Non-small cell lung cancerEuropean journal of cancer (Oxford, England : 1990)
Genetic diagnosis in practice: From cystic fibrosis to CFTR-related disorders.Pagin A, Sermet-Gaudelus I, Burgel PR2020-02-01CFTR-related disorders (CFTR-RD), CFTR-related metabolic syndrome(CRMS)/CF screen positive inconclusive diagnosis (CFSPID), Cystic fibrosis (CF), Cystic fibrosis transmembrane conductance regulator (CFTR)Archives de pediatrie : organe officiel de la Societe francaise de pediatrie
Real-Life Safety and Effectiveness of Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis.Burgel PR, Munck A, Durieu I, Chiron R, Mely L, Prevotat A, Murris-Espin M, Porzio M, Abely M, Reix P, Marguet C, Macey J, Sermet-Gaudelus I, Corvol H, Bui S, Lemonnier L, Dehillotte C, Da Silva J, Paillasseur JL, Hubert D2020-01-15cystic fibrosis, lumacaftor–ivacaftor, postmarketing studyAmerican journal of respiratory and critical care medicine
Risk and outcome of COVID-19 infection in sarcoidosis patients: results of a self-reporting questionnaire.Baughman RP, Lower EE, Buchanan M, Rottoli P, Drent M, Sellares J, Terwiel M, Elfferich M, Francesqui J, Barriuso Cabrerizo MR, Sweiss N, Martone F, Al-Hakim T, Judson MA2020-01-01COVID-19, immunosuppression, sarcoidosisSarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG
Validation of short- and long-term demographic forecasts using the Canadian Cystic Fibrosis Registry.Martelli V, Sykes J, Burgel PR, Bellis G, Coriati A, Stanojevic S, Stephenson AL2020-01-01The European respiratory journal
Cystic Fibrosis Diagnosis in Newborns, Children, and Adults.Castellani C, Linnane B, Pranke I, Cresta F, Sermet-Gaudelus I, Peckham D2019-12-01Seminars in respiratory and critical care medicine
Tumour Treating Fields in combination with pemetrexed and cisplatin or carboplatin as first-line treatment for unresectable malignant pleural mesothelioma (STELLAR): a multicentre, single-arm phase 2 trial.Ceresoli GL, Aerts JG, Dziadziuszko R, Ramlau R, Cedres S, van Meerbeeck JP, Mencoboni M, Planchard D, Chella A, Crinò L, Krzakowski M, Rüssel J, Maconi A, Gianoncelli L, Grosso F2019-12-01The Lancet. Oncology
Association between surgical volume and post-operative mortality and survival after surgical resection in lung cancer in Belgium: A population-based study.Schillemans V, Vrijens F, De Gendt C, Robays J, Silversmit G, Verleye L, Camberlin C, Dubois C, Stordeur S, Wauters I, Van Meerbeeck JP, Van Eycken E, De Leyn P2019-12-01Hospital surgical volume, Lung cancer, Mortality, SurvivalEuropean journal of surgical oncology : the journal of the European Society of Surgical Oncology and the British Association of Surgical Oncology
Influence of Antisynthetase Antibodies Specificities on Antisynthetase Syndrome Clinical Spectrum Time Course.Cavagna L, Trallero-Araguás E, Meloni F, Cavazzana I, Rojas-Serrano J, Feist E, Zanframundo G, Morandi V, Meyer A, Pereira da Silva JA, Matos Costa CJ, Molberg O, Andersson H, Codullo V, Mosca M, Barsotti S, Neri R, Scirè C, Govoni M, Furini F, Lopez-Longo FJ, Martinez-Barrio J, Schneider U, Lorenz HM, Doria A, Ghirardello A, Ortego-Centeno N, Confalonieri M, Tomietto P, Pipitone N, Rodriguez Cambron AB, Blázquez Cañamero MÁ, Voll RE, Wendel S, Scarpato S, Maurier F, Limonta M, Colombelli P, Giannini M, Geny B, Arrigoni E, Bravi E, Migliorini P, Mathieu A, Piga M, Drott U, Delbrueck C, Bauhammer J, Cagnotto G, Vancheri C, Sambataro G, De Langhe E, Sainaghi PP, Monti C, Gigli Berzolari F, Romano M, Bonella F, Specker C, Schwarting A, Villa Blanco I, Selmi C, Ceribelli A, Nuno L, Mera-Varela A, Perez Gomez N, Fusaro E, Parisi S, Sinigaglia L, Del Papa N, Benucci M, Cimmino MA, Riccieri V, Conti F, Sebastiani GD, Iuliano A, Emmi G, Cammelli D, Sebastiani M, Manfredi A, Bachiller-Corral J, Sifuentes Giraldo WA, Paolazzi G, Saketkoo LA, Giorgi R, Salaffi F, Cifrian J, Caporali R, Locatelli F, Marchioni E, Pesci A, Dei G, Pozzi MR, Claudia L, Distler J, Knitza J, Schett G, Iannone F, Fornaro M, Franceschini F, Quartuccio L, Gerli R, Bartoloni E, Bellando Randone S, Zampogna G, Gonzalez Perez MI, Mejia M, Vicente E, Triantafyllias K, Lopez-Mejias R, Matucci-Cerinic M, Selva-O'Callaghan A, Castañeda S, Montecucco C, Gonzalez-Gay MA2019-11-18antisynthetase antibodies, antisynthetase syndrome, arthritis, interstitial lung disease, myositisJournal of clinical medicine
Precision medicine: The future of diagnostic approach to pulmonary hypertension?Kedzierski P, Torbicki A2019-09-01Anatolian journal of cardiology
Where are the gaps in education in the field of rare lung disease? Perspectives from the ERN-LUNG educational programme survey.Powell P, Kreuter M, Wijsenbeek-Lourens M2019-06-01Breathe (Sheffield, England)
The Ariane-IPF ERS Clinical Research Collaboration: seeking collaboration through launch of a federation of European registries on idiopathic pulmonary fibrosis.Cottin V, Annesi-Maesano I, Günther A, Galvin L, Kreuter M, Powell P, Prasse A, Reynolds G, Richeldi L, Spagnolo P, Valenzuela C, Wijsenbeek M, Wuyts WA, Crestani B2019-05-01The European respiratory journal
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Last update: July 6th, 2024 on 01:30