Results 81 to 90 of about 1,122,604 (303)

VX‐659–Tezacaftor–Ivacaftor in Patients with Cystic Fibrosis and One or Two Phe508del Alleles

New England Journal of Medicine, 2018
BACKGROUND The next‐generation cystic fibrosis transmembrane conductance regulator (CFTR) corrector VX‐659, in triple combination with tezacaftor and ivacaftor (VX‐659–tezacaftor–ivacaftor), was developed to restore the function of Phe508del CFTR protein
J. Davies, S. Moskowitz, C. Brown, A. Horsley, M. Mall, E. McKone, B. Plant, D. Prais, B. Ramsey, J. Taylor-Cousar, E. Tullis, A. Uluer, C. Mckee, S. Robertson, R. Shilling, C. Simard, F. van Goor, D. Waltz, F. Xuan, T. Young, S. Rowe   +20 more
semanticscholar   +1 more source

A medium composition containing normal resting glucose that supports differentiation of primary human airway cells

Scientific Reports, 2022
Primary cells isolated from the human respiratory tract are the state-of-the-art for in vitro airway epithelial cell research. Airway cell isolates require media that support expansion of cells in a basal state to maintain the capacity for ...
Rachel Morgan, Candela Manfredi, Kristen F. Easley, Lionel D. Watkins, William R. Hunt, Steven L. Goudy, Eric J. Sorscher, Michael Koval, Samuel A. Molina   +8 more
doaj   +1 more source

MRI of the lung (3/3)-current applications and future perspectives [PDF]

, 2011
BACKGROUND: MRI of the lung is recommended in a number of clinical indications. Having a non-radiation alternative is particularly attractive in children and young subjects, or pregnant women.
A Hebestreit, A Kluge, A Kluge, A Kluge, AA Bankier, AC Henderson, AS Brody, BJ Pichler, BJ Pichler, C Fink, C Hintze, C Hintze, C Rieger, CA Yi, CA Yi, CP Heussel, DM Mannino, F Molinari, F Risse, F Santamaria, G Antoch, G Antoch, G Bauman, G Bauman, G Lutterbey, G Lutterbey, G Sergiacomi, H Hatabu, H Zaidi, HF Wehrl, HK Song, HK Song, IL Theissen, J Biederer, J Biederer, J Biederer, J Biederer, J Biederer, J Biederer, J Biederer, J Ley-Zaporozhan, J Ley-Zaporozhan, JC Hogg, K Cederlund, K Sandek, K Suga, K Suga, LP Qi, M Bhalla, M Decramer, M Eichinger, M Gaeta, M Oudkerk, M Puderbach, M Puderbach, M Puderbach, M Puderbach, M Stern, M Wagner, MD Cohen, ND Abolmaali, NJ Morrison, NL Müller, O Ratib, PA Jong de, PA Jong de, PD Stein, PD Stein, R Eibel, R Failo, RE Jacob, RG McFadden, RJ Theilmann, RL Gibson, S Akata, S Montella, S Pauls, SK Alford, T Amundsen, T Iwasawa, T Iwasawa, T Iwasawa, T Mori, T Rupprecht, TA Kuder, TH Helbich, US Euler, V Peltola, W Hirsch, W Lin, Y Berthezène, Y Ohba, Y Ohno, Y Ohno, Y Ohno, Y Ohno, Y Shao   +96 more
core   +1 more source

Transition to adult services for children and young people with palliative care needs : a systematic review [PDF]

, 2009
Objective: To evaluate the evidence on the transition process from child to adult services for young people with palliative care needs. Design: Systematic review Setting: Child and adult services and interface between healthcare providers.
Adi, Y., Carter, Yvonne, Doug, Manjo, Kelly, Daniel, Paul, Moli, Petchey, Roland, Williams, Jacky   +6 more
core   +3 more sources

Inflammation in cystic fibrosis: An update

Pediatric Pulmonology, 2018
Inflammation plays a critical role in cystic fibrosis (CF) lung pathology and disease progression making it an active area of research and important therapeutic target.
E. Roesch, D. Nichols, J. Chmiel
semanticscholar   +1 more source

A smartphone app for preschool wheezing and reliability of medical history collection

Italian Journal of Pediatrics
Background The use of mobile applications helps improving self-management in adolescents with asthma. However, no evidence is available for children with preschool wheezing.
Nicola Ullmann, Adriana Fracchiolla, Alessandra Boni, Valentina Negro, Federica Porcaro, Antonio Di Marco, Salvatore Tripodi, Renato Cutrera   +7 more
doaj   +1 more source

Increased fat mass and obesity risk after elexacaftor–tezacaftor–ivacaftor therapy in young adults with cystic fibrosis

Frontiers in Nutrition
BackgroundWhen people with cystic fibrosis (PwCFs) are treated with cystic fibrosis transmembrane conductance regulator protein modulator (CFTRm), it leads to changes in body composition.
Ana Merino Sánchez-Cañete, Concepción Marina López Cárdenes, Saioa Vicente Santamaría, José Ramón Gutiérrez Martínez, Marta Suárez González, María Álvarez Merino, David González Jiménez   +6 more
doaj   +1 more source

The Anti-Sigma Factor MucA of Pseudomonas aeruginosa: Dramatic Differences of a mucA22 vs. a ΔmucA Mutant in Anaerobic Acidified Nitrite Sensitivity of Planktonic and Biofilm Bacteria in vitro and During Chronic Murine Lung Infection [PDF]

, 2019
Mucoid mucA22 Pseudomonas aeruginosa (PA) is an opportunistic lung pathogen of cystic fibrosis (CF) and chronic obstructive pulmonary disease (COPD) patients that is highly sensitive to acidified nitrite (A-NO2-).
Hassett, Daniel J., Lau, Gee W., Lu, Long J., McDaniel, Cameron T., Muruve, Daniel A., Ohman, Dennis E., Panmanee, Warunya, Panos, Ralph J., Ren, Zhaowei, Schurr, Michael J., Su, Shengchang, Thompson, Thomas B., Tseng, Boo Shan, Yu, Hongwei D., Zhu, Xiaoting   +14 more
core   +3 more sources

Small molecule ion channels increase host defenses in cystic fibrosis airway epithelia

Nature, 2019
Loss-of-function mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) compromise epithelial HCO3− and Cl− secretion, reduce airway surface liquid pH, and impair respiratory host defences in people with cystic fibrosis1–3.
Katrina A. Muraglia, Rajeev S. Chorghade, Bo Ram Kim, X. Tang, Viral S. Shah, A. Grillo, Page N. Daniels, Alexander G. Cioffi, Philip H. Karp, Lingyang Zhu, M. Welsh, M. Burke   +11 more
semanticscholar   +1 more source

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