Real-time breath metabolomics to assess early response to CFTR modulators in adults with cystic fibrosis: An open-label proof-of-concept study

Further Publications

Sweat chloride and lung function responses to elexacaftor-tezacaftor-ivacaftor in people with cystic fibrosis with two versus one responsive CFTR variants: an analysis of two real-world observational studies

Lancet Respir Med

Burgel PR, Da Silva J, Girodon E, Durieu I, Reynaud-Gaubert M, Murris-Espin M, Chiron R, Grenet D, Ramel S, Mely L, Hamidfar R, Douvry B, Houdouin V, Audousset C, Macey J, Mittaine M, Weiss L, Cosson L, Danner-Boucher I, Reix P, Baravalle M, Burnet E, Sermet-Gaudelus I, Martin C, Paillasseur JL, French Cystic Fibrosis Reference Network study group

doi: 10.1016/S2213-2600(25)00190-0

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Pulmonary Fibrosis

Safety and efficacy of elexacaftor/tezacaftor/ivacaftor in adolescents and adults with cystic fibrosis and F508del-gating and F508del-residual function genotypes: Results from an open-label extension study

★ 2 Recommended by Dr. med. Martin Frey, Faculty Member

★ 1 Recommended by Dr Marcus Hesse, Faculty Member

Ann Am Thorac Soc

Chmiel JF, McBennett K, Quon BS, Colombo C, De Wachter E, Fajac I, Mondejar-Lopez P, Polineni D, Robinson P, Sutharsan S, Mall MA, McKone EF, Ramsey B, Taylor-Cousar JL, Tullis E, Suresh NE, Zhang Y, Sosnay P, Jennings M, Barry PJ, 445-110 study group

doi: 10.1093/annalsats/aaoag053

Pulmonary Fibrosis

Medication adherence to cystic fibrosis transmembrane conductance regulator (CFTR) modulators: Analysis on the French cystic fibrosis population from 2012-2020

Respir Med

Eid E, Payet C, Fernandez V, Belhassen M, Bessou A, Durieu I, Reynaud Q, Viprey M, French Cystic Fibrosis Centers Network, Cinthia R, Françoise T, Marie-Laure D, Bénédicte RT, Stéphanie B, Julie M, Murie L, Sylvie M, Ralph E, Frédéric H, Manuela S, Laurent M, Rébecca H, Catherine L, Camille A, Nathalie WD, Jane L, Philippe R, Jean-Christophe D, Martine RG, Raphaël C, Aurélie T, Anne G, Isabelle DB, Tiphaine B, Sylvie L, Pierre-Régis B, Isabelle S, Véronique H, Harriet C, Katia BK, Chantal B, Eric D, Sophie R, Christophe M, Nathalie A, Caroline P, Benjamin RP, Laurence W, Dominique G, Léa R, Marlène ME, Julie M, Laure C, Baptiste A, Gabsi Asma

doi: 10.1016/j.rmed.2026.108857

RECOMMENDED

Pulmonary Fibrosis

Impact of CFTR Modulator Concentrations on Clinical Response in Cystic Fibrosis

★ 2 Recommended by Dr. med. Martin Frey, Faculty Member

★ 1 Recommended by Dr Marcus Hesse, Faculty Member

Eur Respir J

Chalamalla AR, Baker E, Ryan KJ, Dowell A, Natt JR, Zemanick ET, Konstan MW, Mayer-Hamblett N, Acosta EP, Guimbellot JS, CHEC-SC investigators

doi: 10.1183/13993003.01594-2025

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Pulmonary Fibrosis

Real-World Impact of Elexacaftor/Tezacaftor/Ivacaftor on Health-Related Quality of Life in Adults with Cystic Fibrosis: A 12-Month Multicenter Cohort Study

★ 2 Recommended by Dr. med. Martin Frey, Faculty Member

★ 1 Recommended by Dr Marcus Hesse, Faculty Member

Respir Med

Kharraz R, Tullis E, Sykes J, Ma X, Sandhu A, Gryz Z, Shulman Y, Tam J, Love A, Ratjen F, Rayment JH, Quon BS, Stephenson AL

doi: 10.1016/j.rmed.2026.108827

Pulmonary Fibrosis

Safety and efficacy of elexacaftor/tezacaftor/ivacaftor in children ≥2 years with cystic fibrosis: 96-week interim results from a phase 3 open-label extension study

J Cyst Fibros

Goralski JL, Daines C, Pittman JE, Rayment JH, Robinson P, Thursfield R, Stehling F, Ratjen F, Ahluwalia N, Shi P, Lu M, Weinstock TG, Mall MA, VX20-445-112 Study Group

doi: 10.1016/j.jcf.2026.05.014

Pulmonary HypertensionFurther Publications

Assessment of efficacy and tolerability of elexacaftor-tezacaftor-ivacaftor in an observational cohort study of "aged" people with cystic fibrosis

Ther Adv Respir Dis

Blankenship S, Flueckiger BV, Caleb G, Bailey J, Baker E, Garcia B, Krick S

doi: 10.1177/17534666251410246

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Pulmonary Fibrosis

Effectiveness of CFTR modulator therapy on risk of death for individuals with cystic fibrosis

★ 2 Recommended by Dr. med. Martin Frey, Faculty Member

★ 1 Recommended by Dr Marcus Hesse, Faculty Member

Chest

Kurgansky KE, Collaco JM, Ng DK, Lesko CR

doi: 10.1016/j.chest.2026.04.041

RECOMMENDED

Pulmonary Fibrosis

Comparison of Long Term Effects of Treatment of Different CFTR Modulators in People With Cystic Fibrosis

★ 2 Recommended by Dr. med. Martin Frey, Faculty Member

★ 2 Recommended by Dr Marcus Hesse, Faculty Member

Pediatr Pulmonol

Famulska P, Więckowska B, Narożna B, Sapiejka E, Osińska M, Musiał A, Szczepankiewicz A, Tąpolska-Jóźwiak K, Wiesner A, Steinert-Dymecki A, Jóźwiak M, Wojsyk-Banaszak I

doi: 10.1002/ppul.71681

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Sweat chloride and lung function responses to elexacaftor-tezacaftor-ivacaftor in people with cystic fibrosis with two versus one responsive CFTR variants: an analysis of two real-world observational studies

Safety and efficacy of elexacaftor/tezacaftor/ivacaftor in adolescents and adults with cystic fibrosis and F508del-gating and F508del-residual function genotypes: Results from an open-label extension study

Medication adherence to cystic fibrosis transmembrane conductance regulator (CFTR) modulators: Analysis on the French cystic fibrosis population from 2012-2020

Impact of CFTR Modulator Concentrations on Clinical Response in Cystic Fibrosis

Real-World Impact of Elexacaftor/Tezacaftor/Ivacaftor on Health-Related Quality of Life in Adults with Cystic Fibrosis: A 12-Month Multicenter Cohort Study

Safety and efficacy of elexacaftor/tezacaftor/ivacaftor in children ≥2 years with cystic fibrosis: 96-week interim results from a phase 3 open-label extension study

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