Results 71 to 80 of about 3,564 (173)

Sustained effectiveness of elexacaftor-tezacaftor-ivacaftor in lung transplant candidates with cystic fibrosis

, 2022
International audienceBackground: Elexacaftor-tezacaftor-ivacaftor induces rapid clinical improvement in patients with cystic fibrosis (CF) and advanced pulmonary disease, often leading to suspend the indication for lung transplantation. Yet no long-term
Montcouquiol, Sylvie, Murris-Espin, Marlène, Ramel, Sophie, Douvry, Benoit, Martin, Clémence, Lemonnier, Lydie, Burgel, Pierre-Régis, Grenet, Dominique, Hamidfar, Rebecca, da Silva, Jennifer, Durieu, Isabelle, Danner-Boucher, Isabelle, Chiron, Raphaël, Reynaud-Gaubert, Martine, Burnet, Espérie, Paillasseur, Jean-Louis, Leroy, Sylvie, Mely, Laurent   +17 more
core   +1 more source

Elexacaftor/tezacaftor/ivacaftor as rescue therapy in a patient with the cystic fibrosis genotype F508DEL/G1244E

Clinical Case Reports, 2021
Elexacaftor/tezacaftor/ivacaftor (ETI) is a cystic fibrosis (CF) transmembrane regulator (CFTR) modulator. It is known to be efficacious in stable patients with severe pneumopathy, but there are few data concerning its effectiveness during acute ...
Donatello Salvatore, Carmela Colangelo, Michele D’Andria, Giovanni Marsicovetere, Domenica Passarella   +4 more
doaj   +1 more source

Elexacaftor-Tezacaftor-Ivacaftor: A Life-Changing Triple Combination of CFTR Modulator Drugs for Cystic Fibrosis

Pharmaceuticals, 2023
Cystic fibrosis (CF) is a potentially fatal monogenic disease that causes a progressive multisystemic pathology. Over the last decade, the introduction of CF transmembrane conductance regulator (CFTR) modulator drugs into clinical practice has profoundly
Mafalda Bacalhau, Mariana Camargo, Grace A. V. Magalhães-Ghiotto, Sybelle Drumond, Carlos Henrique M. Castelletti, Miquéias Lopes-Pacheco   +5 more
doaj   +1 more source

DataSheet_1_Regulatory T cell enhancement in adults with cystic fibrosis receiving Elexacaftor/Tezacaftor/Ivacaftor therapy.docx

, 2023
IntroductionCystic fibrosis (CF), especially CF lung disease, is characterized by chronic infection, immune dysfunction including impairment of regulatory T cells (Tregs) and an exaggerated inflammatory response.
Matthias Welsner (11111694), Christian Taube (35629), Dirk Westhölter (14632553), Svenja Straßburg (11111691), Sivagurunathan Sutharsan (3352931), Sebastian Reuter (347960), Jonas Raspe (14494610), Hendrik Uebner (12886982), Mona Schmitz (14632559), Johannes Pipping (14632556)   +9 more
core   +1 more source

Efficacy of Trikafta (ELX/TEZ/IVA) & Symdeko (TEZ/IVA) in Treating Cystic Fibrosis with F508del Allele: A Systematic Review and Meta-analysis

Thoracic Research and Practice
The objective of the study was to assess and compare the efficacy of elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) treatment with TEZ/IVA treatment in individuals diagnosed with cystic fibrosis (CF) and carrying the F508del allele.
Kainat Hussain, Sonali Karhana, Aakriti Garg, Mohd Ashif Khan   +3 more
doaj   +1 more source

Frequency of CFTR variants in southern Brazil and indication for modulators therapy in patients with cystic fibrosis

Genetics and Molecular Biology, 2021
This is a descriptive cross-sectional study that aims to determine the distribution of the CFTR causing variant in a group of patients at a cystic fibrosis (CF) center in southern Brazil, as well as to describe causing variants that are treatable with ...
Eliandra da Silveira Lima, Luíse Sgarabotto Pezzin, Ana Carolina Fensterseifer, Leonardo Araújo Pinto   +3 more
doaj   +1 more source

Elexacaftor–Tezacaftor–Ivacaftor Reduces Revision Sinus Surgery in People With Cystic Fibrosis

The Laryngoscope, Volume 136, Issue 5, Page 2048-2053, May 2026.
Elexacaftor–tezacaftor–ivacaftor (ETI) was associated with a significant decrease in the frequency and rate of endoscopic sinus surgeries in patients with cystic fibrosis. These findings suggest an improvement in CF‐related chronic rhinosinusitis outcomes following ETI and may influence future CF treatment decisions.
Amy Lin, Makanko Komara, Isaac Kistler, Zheng Hong Tan, Meredith Lind, Kavitha Kotha, Tendy Chiang   +6 more
wiley   +1 more source

Image1_Effect of elexacaftor-tezacaftor-ivacaftor on nasal potential difference and lung function in Phe508del rats.PNG

Introduction:Phe508del is the most common cystic fibrosis transmembrane conductance regulator (CFTR) gene variant that results in the recessive genetic disorder cystic fibrosis (CF). The recent development of highly effective CFTR modulator therapies has
Nicole Reyne (14578076), Patricia Cmielewski (4683790), Ronan Smith (13513842), Alexandra McCarron (10699023), Nina Eikelis (11034252), Piraveen Pirakalathanan (18146215), Martin Donnelley (286375), David Parsons (4029998)   +7 more
core   +1 more source

The rescue of F508del-CFTR by elexacaftor/tezacaftor/ivacaftor (Trikafta) in human airway epithelial cells is underestimated due to the presence of ivacaftor

, 2021
International audienceTrikafta, currently the leading therapeutic in Cystic Fibrosis (CF), has demonstrated a real clinical benefit. This treatment is the triple combination therapy of two folding correctors elexacaftor/tezacaftor (VX445/VX661) plus the ...
Mirval, Sandra, Carrez, Thomas, Coraux, Christelle, Lévêque, Manuella, Cantereau, Anne, Sage, Edouard, Billet, Arnaud, Becq, Frédéric   +7 more
core   +1 more source

Challenges to Assessing the Prevalence of Cystic Fibrosis in the Caribbean

Pediatric Pulmonology, Volume 61, Issue 5, May 2026.
ABSTRACT Cystic fibrosis (CF) is likely underdiagnosed in Caribbean populations due to non‐representative cystic fibrosis transmembrane conductance regulator (CFTR) variant screening panels, limited newborn screening programs, and structural healthcare barriers.
Krystal L. Rivera‐Figueroa, Cole L. Martin, Christina A. Le, Wilfredo De Jesús Rojas, Leandra Cordero Oñate, Stephen G. Aller   +5 more
wiley   +1 more source