Results 21 to 30 of about 2,457 (125)

Experience of using lumacaftor/ivacaftor in children with cystic fibrosis in the Astrakhan region

Acta Biomedica Scientifica, 2022
Background. Targeted therapy in patients with cystic fibrosis, which aims to restore the function of the cystic fibrosis transmembrane conductance regulator protein, is currently revolutionary in the treatment of the disease.
A. R. Kosareva, O. A. Bashkina, D. F. Sergienko   +2 more
doaj   +1 more source

tert-Butyl 3-(3-methyl-1-oxidopyridin-1-ium-2-yl)benzoate

IUCrData, 2016
In the title compound, C17H19NO3, which was obtained by oxidation of the corresponding pyridine derivative, the dihedral angle between the benzene and the pyridine rings is 68.2 (1)°.
Gerhard Laus, Volker Kahlenberg, Sven Nerdinger, Frank Richter, Herwig Schottenberger   +4 more
doaj   +1 more source

Innovative Therapeutic Strategies for Cystic Fibrosis: Moving Forward to CRISPR Technique

Frontiers in Pharmacology, 2018
One of the most revolutionary technologies in recent years in the field of molecular biology is CRISPR-Cas9. CRISPR technology is a promising tool for gene editing that provides researchers the opportunity to easily alter DNA sequences and modify gene ...
Michele Marangi, Giuseppa Pistritto, Giuseppa Pistritto   +2 more
doaj   +1 more source

Variable Responses to CFTR Correctors in vitro: Estimating the Design Effect in Precision Medicine

Frontiers in Pharmacology, 2018
Interest in precision medicine has grown in recent years due to the variable clinical benefit provided by some medications, their cost, and by new opportunities to tailor therapies to individual patients.
Elizabeth Matthes, Elizabeth Matthes, Julie Goepp, Julie Goepp, Carolina Martini, Carolina Martini, Jiajie Shan, Jiajie Shan, Jie Liao, Jie Liao, David Y. Thomas, David Y. Thomas, John W. Hanrahan, John W. Hanrahan, John W. Hanrahan   +14 more
doaj   +1 more source

Diabetes outbreak during COVID19 lock-down in a prediabetic patient with cystic fibrosis long treated with glargine

Italian Journal of Pediatrics, 2021
Background Cystic Fibrosis Related Diabetes (CFRD) is a frequent comorbidity of patients with Cystic Fibrosis (CF). A worsening of clinical conditions appears before CFRD.
Francesco Maria Rosanio, Enza Mozzillo, Chiara Cimbalo, Alberto Casertano, Angela Sepe, Valeria Raia, Adriana Franzese, Antonella Tosco   +7 more
doaj   +1 more source

Lumacaftor/ivacaftor changes the lung microbiome and metabolome in cystic fibrosis patients

ERJ Open Research, 2021
Rationale Targeted cystic fibrosis (CF) therapy with lumacaftor/ivacaftor partly restores chloride channel function and improves epithelial fluid transport in the airways.
Anne H. Neerincx, Katrine Whiteson, Joann L. Phan, Paul Brinkman, Mahmoud I. Abdel-Aziz, Els J.M. Weersink, Josje Altenburg, Christof J. Majoor, Anke H. Maitland-van der Zee, Lieuwe D.J. Bos   +9 more
doaj   +1 more source

Sinus Disease Grading on Computed Tomography Before and After Modulating Therapy in Adult Patients with Cystic Fibrosis

Journal of the Belgian Society of Radiology, 2022
Objectives: Cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapy effects on respiratory function, pulmonary exacerbations and quality of life have been well documented. However, CFTR modulator therapy effects on sinus disease have
Corrado Tagliati, Stefano Pantano, Giuseppe Lanni, Davide Battista, Matteo Marcucci, Marco Fogante, Giulio Argalia, Enrico Paci, Gabriella Lucidi Pressanti, Mingliang Ying, Pietro Ripani   +10 more
doaj   +1 more source

Impact of lumacaftor/ivacaftor on the bacterial and fungal respiratory pathogens in cystic fibrosis: a prospective multicenter cohort study in Sweden

Therapeutic Advances in Respiratory Disease
Background: A significant decline in pulmonary exacerbation rates has been reported in CF patients homozygous for F508del treated with lumacaftor/ivacaftor. However, it is still unclear whether this reduction reflects a diminished microbiological burden.
Mahasin Al Shakirchi, Kimmo Sorjonen, Lena Hjelte, Lena Klingspor, Peter Bergman, Petrea Ericson, Marcus Svedberg, Ulrika Lindberg, Christine Hansen, Isabelle de Monestrol   +9 more
doaj   +1 more source

Lumacaftor/ivacaftor initiation in two liver transplantation patients under tacrolimus and antifungal azoles

Clinical Case Reports, 2019
Key Clinical Message We report the initiation of CFTR modulator lumacaftor/ivacaftor combination (LUM/IVA) in two adolescents with cystic fibrosis who were treated with antifungal azoles (AZO) and tacrolimus (TCS) for liver transplantation.
Ikrame Chouchane, Nathalie Stremler‐Lebel, Philippe Reix   +2 more
doaj   +1 more source

CFTR Modulators Dampen Aspergillus-Induced Reactive Oxygen Species Production by Cystic Fibrosis Phagocytes

Frontiers in Cellular and Infection Microbiology, 2020
Excessive inflammation by phagocytes during Aspergillus fumigatus infection is thought to promote lung function decline in CF patients. CFTR modulators have been shown to reduce A.
Alexander J. Currie, Alexander J. Currie, Ellen T. Main, Heather M. Wilson, Darius Armstrong-James, Adilia Warris   +5 more
doaj   +1 more source