CFTR modulator therapy: transforming the landscape of clinical care in cystic fibrosis
JL Taylor-Cousar, PD Robinson, M Shteinberg… - The Lancet, 2023 - thelancet.com
Following discovery of the cystic fibrosis transmembrane conductance regulator (CFTR)
gene in 1989 and subsequent elucidation of the varied CFTR protein abnormalities that …
gene in 1989 and subsequent elucidation of the varied CFTR protein abnormalities that …
Understanding and addressing the needs of people with cystic fibrosis in the era of CFTR modulator therapy
Cystic fibrosis is a multiorgan disease caused by impaired function of the cystic fibrosis
transmembrane conductance regulator (CFTR). Since the introduction of the CFTR …
transmembrane conductance regulator (CFTR). Since the introduction of the CFTR …
Post-approval studies with the CFTR modulators Elexacaftor-Tezacaftor—Ivacaftor
B Tümmler - Frontiers in Pharmacology, 2023 - frontiersin.org
Triple combination therapy with the CFTR modulators elexacaftor (ELX), tezacaftor (TEZ)
and ivacaftor (IVA) has been qualified as a game changer in cystic fibrosis (CF). We provide …
and ivacaftor (IVA) has been qualified as a game changer in cystic fibrosis (CF). We provide …
Trials and tribulations of highly effective modulator therapies in cystic fibrosis
N Lieu, BJ Prentice, P Field, DA Fitzgerald - Paediatric Respiratory …, 2023 - Elsevier
Highly effective modulator therapies (HEMTs) have revolutionised the management
approach of most patients living with cystic fibrosis (CF) who have access to these therapies …
approach of most patients living with cystic fibrosis (CF) who have access to these therapies …
[HTML][HTML] Cystic fibrosis in Europe: improved lung function and longevity–reasons for cautious optimism, but challenges remain
Background Prognosis and disease severity in cystic fibrosis (CF) are linked to declining
lung function. To characterise lung function by the number of adults in countries with …
lung function. To characterise lung function by the number of adults in countries with …
Elexacaftor-Tezacaftor-Ivacaftor: a life-changing triple combination of CFTR modulator drugs for cystic fibrosis
M Bacalhau, M Camargo, GAV Magalhães-Ghiotto… - Pharmaceuticals, 2023 - mdpi.com
Cystic fibrosis (CF) is a potentially fatal monogenic disease that causes a progressive
multisystemic pathology. Over the last decade, the introduction of CF transmembrane …
multisystemic pathology. Over the last decade, the introduction of CF transmembrane …
Efficacy and safety profile of elexacaftor-tezacaftor-ivacaftor triple therapy on cystic fibrosis: a systematic review and single arm meta-analysis
W Xu, T Wu, Z Zhou, Z Zuo - Frontiers in Pharmacology, 2023 - frontiersin.org
Background: Elexacaftor-Tezacaftor-Ivacaftor (ELE/TEZ/IVA) is believed to be an effective
and well-tolerated treatment for cystic fibrosis (CF), but the exact efficacy and safety profile …
and well-tolerated treatment for cystic fibrosis (CF), but the exact efficacy and safety profile …
New drugs, new challenges in cystic fibrosis care
Cystic fibrosis (CF) is a genetic disease caused by variants in the gene encoding for the CF
transmembrane conductance regulator (CFTR) protein, a chloride and bicarbonate channel …
transmembrane conductance regulator (CFTR) protein, a chloride and bicarbonate channel …
Cystic fibrosis year in review 2023
DV Swetland, AP Savant - Pediatric Pulmonology, 2024 - Wiley Online Library
This past year, there were many important advances for patients with cystic fibrosis (CF). Of
the many publications related to CF in 2023, there was further evaluation of highly effective …
the many publications related to CF in 2023, there was further evaluation of highly effective …
Cystic fibrosis year in review 2022
J Kim, B Lyman, AP Savant - Pediatric Pulmonology, 2023 - Wiley Online Library
Remarkable medical advancements have been made for people with cystic fibrosis (CF) in
recent years, with an abundance of research continuing to be conducted worldwide. With …
recent years, with an abundance of research continuing to be conducted worldwide. With …