Results 41 to 50 of about 3,864 (165)

Impact of Elexacaftor/Tezacaftor/Ivacaftor Therapy on the Cystic Fibrosis Airway Microbial Metagenome

Microbiology Spectrum, 2022
The introduction of mutation-specific combination therapy with the cystic fibrosis transmembrane conductance regulator (CFTR) modulators elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) has substantially improved lung function and quality of life of people
Sophia T. Pallenberg, Marie-Madlen Pust, Ilona Rosenboom, Gesine Hansen, Lutz Wiehlmann, Anna-Maria Dittrich, Burkhard Tümmler   +6 more
doaj   +1 more source

Longitudinal effects of elexacaftor/tezacaftor/ivacaftor on the oropharyngeal metagenome in adolescents with cystic fibrosis [PDF]

Background Triple modulator therapy elexacaftor/tezacaftor/ivacaftor (ETI) improves lung function and impacts upon the respiratory microbiome in people with Cystic fibrosis (pwCF) with advanced lung disease. However, adolescents with cystic fibrosis (CF)
Gisler, Amanda, Hilty, Markus, Moeller, Alexander, Mostacci, Nadja, Steinberg, Ruth, Usemann, Jakob   +5 more
core   +1 more source

Theratyping of the Rare CFTR Variants E193K and R334W in Rectal Organoid-Derived Epithelial Monolayers [PDF]

, 2022
Background: The effect of presently available CFTR modulator combinations, such as elexacaftor (ELX), tezacaftor (TEZ), and ivacaftor (IVA), on rare CFTR alleles is often unknown.
Alessandro Fiocchi, Claudio Sorio, Fabiana Ciciriello, Federico Alghisi, Hugo R. De Jonge, Kelly F. Meijsen, Luca Cristiani, Marcel Bijvelds, Paola Melotti, Vincenzina Lucidi   +9 more
core   +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

3D Automated Segmentation of Bronchial Abnormalities on Ultrashort Echo Time MRI: A Quantitative MR Outcome in Cystic Fibrosis

Journal of Magnetic Resonance Imaging, EarlyView.
ABSTRACT Background Cystic fibrosis (CF) monitoring relies on computed tomography (CT), but ultra‐short echo time MRI (UTE‐MRI) offers a radiation‐free alternative. However, its clinical adoption is hindered by the laborious and subjective manual analysis, which prevents standardized quantification of bronchial abnormalities.
Amel Imene Hadj Bouzid, Ilyes Benlala, Baudouin Denis de Senneville, Fabien Baldacci, Julie Macey, Wadie Benhassen, Pan Su, Stephanie Bui, Maeva Zysman, Aurelien Bustin, Patrick Berger, Gael Dournes   +11 more
wiley   +1 more source

Safety and efficacy of elexacaftor/tezacaftor/ivacaftor in people with Cystic Fibrosis following liver transplantation:A systematic review [PDF]

BACKGROUND & AIMS: Cystic Fibrosis (CF) liver disease progresses to liver failure requiring transplantation in about 3 % of patients, 0.7 % of CF patients are post liver transplant.
Brugha, Rossa, Indolfi, Giuseppe, Terlizzi, Vito, Testa, Ilaria, Verkade, Henkjan J   +4 more
core   +1 more source

State of the art on approved cystic fibrosis transmembrane conductance regulator (Cftr) modulators and triple-combination therapy [PDF]

, 2021
Cystic fibrosis (CF) is the most common life-limiting inherited disease in Caucasian populations, affecting approximately 80,000 people worldwide. CF is a complex multi-organ monogenic autosomal recessive disorder caused by a mutation in cystic fibrosis ...
Caminiti C., Chiopris G., Deolmi M., Esposito S., Fainardi V., Marinelli F., Meoli A., Pisi G.   +7 more
core   +1 more source

Comparison of Single‐Breath and Multi‐Breath Xe‐MRI in the Longitudinal Assessment of Treatment in Children With Cystic Fibrosis

Journal of Magnetic Resonance Imaging, EarlyView.
ABSTRACT Background Elexacaftor/tezacaftor/ivacaftor (ETI) is a current standard therapy for pediatric cystic fibrosis (CF). Multiple‐breath washout 129Xe MRI (MBW Xe‐MRI) is improved following 1 month of treatment. However, the utility of MBW Xe‐MRI over extended ETI treatment and its comparison to single‐breath Xe‐MRI and pulmonary function tests ...
Faiyza S. Alam, Samal Munidasa, Brandon Zanette, Daniel Li, Sharon Braganza, Felix Ratjen, Giles Santyr   +6 more
wiley   +1 more source

Pharmacokinetics of Ivacaftor, Tezacaftor, Elexacaftor, and Lumacaftor in Special Cystic Fibrosis Populations:A Systematic Review [PDF]

BACKGROUND AND OBJECTIVE: Following the development of cystic fibrosis transmembrane conductance regulator (CFTR) modulators (ivacaftor, tezacaftor, elexacaftor, and lumacaftor), the prognosis for people diagnosed with cystic fibrosis (pwCF) has improved.
Akkerman, Onno W, Elzinga, Femke A, Koppelman, Gerard H, Malik, Paul R V, Mian, Paola, Rottier, Bart L, Touw, Daan J, van der Vaart, Hester   +7 more
core   +1 more source

Impact of elexacaftor-tezacaftor-ivacaftor in lung transplantation for cystic fibrosis in the United States

JHLT Open
Background: Cystic fibrosis (CF) is an autosomal recessive condition leading to progressive lung disease and often necessitating lung transplantation. Historically, CF has been one of the leading indications for lung transplants in the United States. The
Tahuanty A. Pena, MD, MS, Brittany Wright, PharmD, Kalpaj R. Parekh, MBBS, Julia Kleney-Tait, MD, PhD   +3 more
doaj   +1 more source