Combined CFTR modulator therapies are linked with anabolic benefits and insulin-sparing in cystic fibrosis-related diabetes [PDF]
Journal of Clinical & Translational Endocrinology, 2023 Aims: Combined CFTR modulator therapies have dramatically altered pulmonary outcomes in patients with cystic fibrosis (CF). Their impact on glucose metabolism requires further investigations.
Fabian Lurquin +3 more
doaj +4 more sources
Hypoglycaemia after Initiation of CFTR Modulator Therapy in a Cystic Fibrosis Patient without Diabetes [PDF]
Case Reports in Endocrinology, 2023 Introduction. Cystic fibrosis transmembrane regulator (CFTR) modulator therapies improve respiratory function and glycaemic control in patients with cystic fibrosis (CF).
Marie Yskout +10 more
doaj +4 more sources
CFTR modulator use and risk of nontuberculous mycobacteria positivity in cystic fibrosis, 2011–2018
ERJ Open Research, 2022 Background People with cystic fibrosis are at increased risk of pulmonary nontuberculous mycobacteria (NTM) disease. Cystic fibrosis transmembrane conductance regulator (CFTR) modulators are associated with reduced lung infection with pathogens like ...
Emily E. Ricotta +2 more
doaj +3 more sources
CFTR Modulator Therapy for Rare CFTR Mutants
Journal of Respiration, 2022 Cystic fibrosis (CF), the most common genetic disease among the Caucasian population, is caused by mutations in the gene encoding for the CF transmembrane conductance regulator (CFTR), a chloride epithelial channel whose dysfunction results in severe ...
Marco Mergiotti +3 more
doaj +3 more sources
Understanding and addressing the needs of people with cystic fibrosis in the era of CFTR modulator therapy. [PDF]
Lancet Respir Med, 2023 Cystic fibrosis (CF) is a multiorgan disease caused by impaired function of the cystic fibrosis transmembrane conductance regulator (CFTR). Since the introduction of the CFTR modulator combination elexacaftor/ tezacaftor/ ivacaftor (ETI), which acts ...
Hisert KB +12 more
europepmc +4 more sources
Personalized CFTR Modulator Therapy for G85E and N1303K Homozygous Patients with Cystic Fibrosis. [PDF]
Int J Mol Sci, 2023 CFTR modulator therapy with elexacaftor/tezacaftor/ivacaftor (ETI) has been approved for people with CF and at least one F508del allele in Europe. In the US, the ETI label has been expanded to 177 rare CFTR mutations responsive in Fischer rat thyroid ...
Graeber SY +13 more
europepmc +3 more sources
Changes in the Cystic Fibrosis Airway Microbiome in Response to CFTR Modulator Therapy
Frontiers in Cellular and Infection Microbiology, 2021 The development of CFTR modulator therapies significantly changed the treatment scheme of people with cystic fibrosis. However, CFTR modulator therapy is still a life-long treatment, which is not able to correct the genetic defect and cure the disease ...
Buqing Yi +2 more
exaly +4 more sources
The gut-lung axis in the CFTR modulator era. [PDF]
Front Cell Infect Microbiol, 2023 The advent of CFTR modulators represents a turning point in the history of cystic fibrosis (CF) management, changing profoundly the disease’s clinical course by improving mucosal hydration.
Lussac-Sorton F +8 more
europepmc +5 more sources
Real-world disparities and ethical considerations with access to CFTR modulator drugs: Mind the gap! [PDF]
Front Pharmacol, 2023 The third Sustainable Development Goal (SDG), to ensure healthy lives and promote well-being for all at all ages, has particular relevance and implementation challenges amongst people living with rare diseases such as cystic fibrosis (CF).
Zampoli M, Morrow BM, Paul G.
europepmc +2 more sources
Evolving nutrition therapy in cystic fibrosis: Adapting to the CFTR modulator era. [PDF]
Nutr Clin PractCystic fibrosis transmembrane regulator (CFTR)–directed therapies, such as modulators, have transformed the medical management of people with CF, resulting in better lung function, weight, and body mass index in recent years.
Vavrina K +5 more
europepmc +2 more sources