The extrapulmonary effects of cystic fibrosis transmembrane conductance regulator modulators in cystic fibrosis
V Sergeev, FY Chou, GY Lam, CM Hamilton… - Annals of the …, 2020 - atsjournals.org
The effects of cystic fibrosis (CF) transmembrane conductance regulator (CFTR) modulators
on lung function, pulmonary exacerbations, and quality of life have been well documented …
on lung function, pulmonary exacerbations, and quality of life have been well documented …
Targeting a genetic defect: cystic fibrosis transmembrane conductance regulator modulators in cystic fibrosis
N Derichs - European Respiratory Review, 2013 - Eur Respiratory Soc
Cystic fibrosis (CF) is caused by genetic mutations that affect the cystic fibrosis
transmembrane conductance regulator (CFTR) protein. These mutations can impact the …
transmembrane conductance regulator (CFTR) protein. These mutations can impact the …
A CFTR Potentiator in Patients with Cystic Fibrosis and the G551D Mutation
BW Ramsey, J Davies, NG McElvaney… - … England Journal of …, 2011 - Mass Medical Soc
Background Increasing the activity of defective cystic fibrosis transmembrane conductance
regulator (CFTR) protein is a potential treatment for cystic fibrosis. Methods We conducted a …
regulator (CFTR) protein is a potential treatment for cystic fibrosis. Methods We conducted a …
Cystic fibrosis transmembrane conductance regulator–modifying medications: the future of cystic fibrosis treatment
RS Pettit - Annals of Pharmacotherapy, 2012 - journals.sagepub.com
OBJECTIVE: To review and evaluate cystic fibrosis transmembrane conductance regulator
(CFTR) modulators for the treatment of cystic fibrosis (CF). DATA SOURCES: Literature was …
(CFTR) modulators for the treatment of cystic fibrosis (CF). DATA SOURCES: Literature was …
Current state of CFTR modulators for treatment of Cystic Fibrosis
KA Despotes, SH Donaldson - Current Opinion in Pharmacology, 2022 - Elsevier
Small molecular modulators of the cystic fibrosis transmembrane conductance regulator
protein are transforming the care of people with cystic fibrosis. Highly effective modulators …
protein are transforming the care of people with cystic fibrosis. Highly effective modulators …
Small-molecule drugs for cystic fibrosis: Where are we now?
O Laselva, L Guerra, S Castellani, M Favia… - Pulmonary …, 2022 - Elsevier
The cystic fibrosis (CF) lung disease is due to the lack/dysfunction of the CF Transmembrane
Conductance Regulator (CFTR), a chloride channel expressed by epithelial cells as the …
Conductance Regulator (CFTR), a chloride channel expressed by epithelial cells as the …
[HTML][HTML] From ivacaftor to triple combination: a systematic review of efficacy and safety of CFTR modulators in people with cystic fibrosis
A Gramegna, M Contarini, S Aliberti… - International journal of …, 2020 - mdpi.com
Over the last years CFTR (cystic fibrosis transmembrane conductance regulator) modulators
have shown the ability to improve relevant clinical outcomes in patients with cystic fibrosis …
have shown the ability to improve relevant clinical outcomes in patients with cystic fibrosis …
Cystic fibrosis foundation pulmonary guidelines. Use of cystic fibrosis transmembrane conductance regulator modulator therapy in patients with cystic fibrosis
CL Ren, RL Morgan, C Oermann… - Annals of the …, 2018 - atsjournals.org
Rationale: Cystic fibrosis (CF) transmembrane conductance regulator (CFTR) modulators
are a new class of medications targeting the underlying defect in CF. Ivacaftor (IVA) and IVA …
are a new class of medications targeting the underlying defect in CF. Ivacaftor (IVA) and IVA …
Efficacy and Safety of Ivacaftor in Patients Aged 6 to 11 Years with Cystic Fibrosis with a G551D Mutation
JC Davies, CE Wainwright, GJ Canny… - American journal of …, 2013 - atsjournals.org
Rationale: Ivacaftor (VX-770), a cystic fibrosis transmembrane conductance regulator
(CFTR) potentiator, has been shown to improve lung function, pulmonary exacerbation rate …
(CFTR) potentiator, has been shown to improve lung function, pulmonary exacerbation rate …
[HTML][HTML] Ivacaftor withdrawal syndrome in cystic fibrosis patients with the G551D mutation
AT Trimble, SH Donaldson - Journal of Cystic Fibrosis, 2018 - Elsevier
Ivacaftor use can lead to dramatic health improvements in cystic fibrosis (CF) patients with
gating mutations. Here, we report five instances of dramatic clinical decline following …
gating mutations. Here, we report five instances of dramatic clinical decline following …