Results 11 to 20 of about 2,230 (120)

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

J Magn Reson Imaging
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.
Bouzid AIH, Benlala I, de Senneville BD, Baldacci F, Macey J, Benhassen W, Su P, Bui S, Zysman M, Bustin A, Berger P, Dournes G.   +11 more
europepmc   +2 more sources

Quantitative Systems Toxicology Predicts Ivacaftor-Induced Oxidative Stress Contributes to CFTR Modulator Hepatotoxicity. [PDF]

Clin Pharmacol Ther
Cystic fibrosis (CF) is a chronic hereditary disease that affects tens of thousands of people worldwide. The introduction of CFTR modulator therapies such as elexacaftor/tezacaftor/ivacaftor (ETI) has significantly improved the quality of life of people with CF.
Shi A, Cornwell C, Yang K, Beringer PM.
europepmc   +2 more sources

The validated French CFAbd-Score reveals a lower burden of gastrointestinal symptoms in patients on Elexacaftor/Tezacaftor/Ivacaftor. [PDF]

J Pediatr Gastroenterol Nutr
Abstract Objectives Multiorgan abdominal involvement is a hallmark of Cystic fibrosis (CF). The CFAbd‐Score© is the first CF‐specific gastrointestinal patient reported outcome‐measure (PROM) developed following FDA‐guidelines. The PROM has proved to sensitively differentiate people with CF (pwCF) from healthy controls (HC).
Sermet-Gaudelus I, Sadrieh P, Bonnel AS, Enaud R, Wizla N, Languepin J, Nangui A, Deelawon A, Rave J, Dabelow S, Barucha A, Zagoya C, Duckstein F, Mainz JG.   +13 more
europepmc   +2 more sources

Novel CFTR modulator combinations maximise rescue of G85E and N1303K in rectal organoids

ERJ Open Research, 2022
Introduction Cystic fibrosis (CF) is a severe monogenic disorder caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene.
Marjolein M. Ensinck, Liesbeth De Keersmaecker, Anabela S. Ramalho, Senne Cuyx, Stephanie Van Biervliet, Lieven Dupont, Frauke Christ, Zeger Debyser, François Vermeulen, Marianne S. Carlon   +9 more
doaj   +1 more source

Regulatory T cell enhancement in adults with cystic fibrosis receiving Elexacaftor/Tezacaftor/Ivacaftor therapy

Frontiers in Immunology, 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.
Dirk Westhölter, Jonas Raspe, Hendrik Uebner, Johannes Pipping, Mona Schmitz, Svenja Straßburg, Sivagurunathan Sutharsan, Matthias Welsner, Christian Taube, Sebastian Reuter   +9 more
doaj   +1 more source

Effects of CFTR modulators on serum biomarkers of liver fibrosis in children with cystic fibrosis

Hepatology Communications, 2023
The cystic fibrosis (CF) transmembrane conductance regulator corrector/potentiator combinations lumacaftor/ivacaftor and elexacaftor/tezacaftor/ivacaftor improve sweat chloride, pulmonary function, and nutrition.
Steven Levitte, Yonathan Fuchs, Russell Wise, Zachary M. Sellers   +3 more
doaj   +1 more source

Personalized Selection of a CFTR Modulator for a Patient with a Complex Allele [L467F;F508del]

Current Issues in Molecular Biology, 2022
The presence of complex alleles in the CFTR gene can lead to difficulties in diagnosing cystic fibrosis and cause resistance to therapy with CFTR modulators.
Elena Kondratyeva, Nataliya Bulatenko, Yuliya Melyanovskaya, Anna Efremova, Elena Zhekaite, Viktoriya Sherman, Anna Voronkova, Irina Asherova, Alexander Polyakov, Tagui Adyan, Valeriia Kovalskaia, Tatiana Bukharova, Dmitry Goldshtein, Sergey Kutsev   +13 more
doaj   +1 more source

Malassezia Folliculitis following Triple Therapy for Cystic Fibrosis

Medicina, 2022
Triple-combination therapy with elexacaftor, tezacaftor and ivacaftor has been recently approved for cystic fibrosis patients with at least one F508del mutation in the transmembrane conductance regulator of the cystic fibrosis gene.
Federica Li Pomi, Luca Di Bartolomeo, Mario Vaccaro, Maria Lentini, Simona Cristadoro, Maria Cristina Lucanto, Mariangela Lombardo, Stefano Costa, Francesco Borgia   +8 more
doaj   +1 more source

Individualized approach to elexacaftor/tezacaftor/ivacaftor dosing in cystic fibrosis, in response to self-reported anxiety and neurocognitive adverse events: A case series

Frontiers in Pharmacology, 2023
The prevalence of mental health disorders is high among people with Cystic Fibrosis. The psychological symptoms in CF are associated with poor adherence, worse treatment outcomes, and greater health utilization/cost.
Hisham Ibrahim, Hisham Ibrahim, Hammad Danish, David Morrissey, David Morrissey, Kevin F. Deasy, Kevin F. Deasy, Mairead McCarthy, James Dorgan, Claire Fleming, Ciara Howlett, Sarah Twohig, Tamara Vagg, Tamara Vagg, Desmond M. Murphy, Desmond M. Murphy, Michael Maher, Michael Maher, Barry J. Plant, Barry J. Plant   +19 more
doaj   +1 more source

Lingering Identity as Chronically Ill and the Unanticipated Effects of Life-Changing Precision Medicine in Cystic Fibrosis: A Case Report

Journal of Patient Experience, 2021
Cystic fibrosis (CF) is the leading genetic disease among Caucasians; however, advances in diagnosis and treatment have improved both quality and quantity of life for those affected.
Sigrid Ladores PhD, RN, PNP, CNE, FAAN, Morgan Polen   +1 more
doaj   +1 more source