Results 41 to 50 of about 4,690 (200)

Elexacaftor–Tezacaftor–Ivacaftor improves exercise capacity in adolescents with cystic fibrosis

Pediatric Pulmonology, 2022
AbstractObjectiveElexacaftor/Tezacaftor/Ivacaftor is a cystic fibrosis transmembrane conductance regulator (CFTR) modulator with the potential to improve exercise capacity. This case series of three adolescents with CF aimed to investigate whether 6 weeks treatment with Elexacaftor/Tezacaftor/Ivacaftor could improve exercise capacity in CFTR modulator ...
Adam J. Causer, Janis K. Shute, Michael H. Cummings, Anthony I. Shepherd, Samuel R. Wallbanks, Richard M. Pulsford, Victoria Bright, Gary Connett, Zoe L. Saynor   +8 more
openaire   +3 more sources

USO DE EXACAFTOR/ TEZACAFTOR /IVACAFTOR EN PACIENTES CON FIBROSIS QUÍSTICA POST-TRASPLANTE PULMONAR

Neumología Pediátrica, 2023
El uso de moduladores de CFTR en pacientes con fibrosis quística post trasplante pulmonar es un tema todavía controversial. Varias publicaciones reportan los beneficios del modulador elexacaftor/tezacaftor/ivacaftor en los síntomas extrapulmonares de la ...
Pablo Jorquera Pinto
doaj   +1 more source

Successful pregnancy in a cystic fibrosis patient with a severe impairment of lung function receiving Elexacaftor-Tezacaftor-Ivacaftor

Respiratory Medicine Case Reports, 2022
Before the arrival of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) modulators women with CF and impaired lung function were experiencing a high risk of complications and mortality during and the years after pregnancy.
Zisis Balmpouzis, Annabelle Faure van Rossum, David Baud, Alice Panchaud, Georgia Mitropoulou, Jesica Mazza Stalder, Angela Koutsokera   +6 more
doaj   +1 more source

Elexacaftor/Tezacaftor/Ivacaftor Accelerates Wound Repair in Cystic Fibrosis Airway Epithelium

Journal of Personalized Medicine, 2022
Background: Cystic fibrosis (CF) airway epithelium shows alterations in repair following damage. In vitro studies showed that lumacaftor/ivacaftor (Orkambi) may favor airway epithelial integrity in CF patients. Our aim was to evaluate the effect of the novel triple combination elexacaftor/tezacaftor/ivacaftor (ETI) on wound repair in CF airway ...
Onofrio Laselva, Massimo Conese
openaire   +3 more sources

Lung function improvement on triple modulators: high-resolution, nationwide data from the Danish Cystic Fibrosis Cohort

ERJ Open Research
Background People living with cystic fibrosis in Denmark had early, universal access to triple modulator treatment with elexacaftor/tezacaftor/ivacaftor.
Christian Leo-Hansen, Daniel Faurholt-Jepsen, Tavs Qvist, Christine Højte, Bibi U. Nielsen, Thomas Bryrup, Esben H. Henriksen, Terese Katzenstein, Marianne Skov, Inger H.M. Mathiesen, Majbritt Jeppesen, Søren Jensen-Fangel, Hanne V. Olesen, Frederik Fouirnaies Buchvald, Kim Gjerum Nielsen, Espen Jimenez-Solem, Christian Ritz, Tacjana Pressler, Mette F. Olsen, the TransformCF Study Group   +19 more
doaj   +1 more source

CFTR Correctors and Antioxidants Partially Normalize Lipid Imbalance but not Abnormal Basal Inflammatory Cytokine Profile in CF Bronchial Epithelial Cells

Frontiers in Physiology, 2021
A deficiency in cystic fibrosis transmembrane conductance regulator (CFTR) function in CF leads to chronic lung disease. CF is associated with abnormalities in fatty acids, ceramides, and cholesterol, their relationship with CF lung pathology is not ...
Mieke Veltman, Mieke Veltman, Juan B. De Sanctis, Marta Stolarczyk, Nikolai Klymiuk, Nikolai Klymiuk, Andrea Bähr, Andrea Bähr, Rutger W. Brouwer, Rutger W. Brouwer, Edwin Oole, Edwin Oole, Juhi Shah, Tomas Ozdian, Jie Liao, Carolina Martini, Danuta Radzioch, John W. Hanrahan, John W. Hanrahan, Bob J. Scholte, Bob J. Scholte   +20 more
doaj   +1 more source

Cystic fibrosis modulator therapy can reverse cystic bronchiectasis

Respirology Case Reports, 2023
Bronchiectasis is often considered progressive and irreversible, so cases of regression or reversal are an important step in understanding the underlying pathophysiological mechanisms.
Peter G. Middleton, Nicholas J. Simmonds
doaj   +1 more source

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

European Respiratory Journal, 2023
Background In two pivotal phase 3 trials, up to 24 weeks of treatment with elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) was efficacious and safe in patients with cystic fibrosis (CF) ≥12 years of age who have at least one F508del allele.
C. Daines, Elizabeth Tullis, Stefano Costa, Rachel W. Linnemann, M. Mall, E. McKone, Deepika Polineni, B. Quon, Felix C. Ringshausen, S. Rowe, H. Selvadurai, J. Taylor-Cousar, Nicholas J. Withers, N. Ahluwalia, Samuel M. Moskowitz, Valentin Prieto-Centurion, Yaoyuan Vincent Tan, S. Tian, Tanya G Weinstock, Fengjuan Xuan, Yaohua Zhang, Bonnie W Ramsey, Matthias Griese   +22 more
semanticscholar   +1 more source

Mejoría de la inflamación intestinal tras tratamiento con moduladores de la proteína CFTR en pacientes con fibrosis quística

Anales de Pediatría
Resumen: Introducción: Los tratamientos con moduladores de la proteína CFTR han mejorado la salud respiratoria y digestiva de los pacientes con fibrosis quística.
Ruth García Romero, Concepción López Cárdenes, Elena Crehuá Gaudiza, Marina Álvarez Beltrán, Mercedes Murray Hurtado, Carlos Tutau Gómez, Inés Loverdos Eseverri, Encarni Torcuato Rubio, Camila García Volpe, Enrique Salcedo Lobato, María Medina Martínez, Carmen Martin Fernández, Ana Moreno Álvarez, Ana Reyes Domínguez, David González Jiménez   +14 more
doaj   +1 more source

Improvement of intestinal inflammation after treatment with CFTR modulators in cystic fibrosis patients

Anales de Pediatría (English Edition)
Introduction: Treatments with CFTR protein modulators have improved respiratory and digestive health in patients with cystic fibrosis. Objective: To assess changes in intestinal inflammation through the analysis of fecal calprotectin in patients with ...
Ruth García Romero, Concepción López Cárdenes, Elena Crehuá Gaudiza, Marina Álvarez Beltrán, Mercedes Murray Hurtado, Carlos Tutau Gómez, Inés Loverdos Eseverri, Encarni Torcuato Rubio, Camila García Volpe, Enrique Salcedo Lobato, María Medina Martínez, Carmen Martin Fernández, Ana Moreno Álvarez, Ana Reyes Domínguez, David González Jiménez, Saioa Vicente Santamaría, Ana Tabares González, Jose Ramon Gutierrez Martinez, Sara Sierra San Nicolás, Pilar Ortiz Pérez, Luis Peña Quintana   +20 more
doaj   +1 more source