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A disease found in dogs where abnormally thick mucus accumulates in the gallbladder — called a mucocele — is characterized by altered levels and function of the CFTR protein that’s also faulty in cystic fibrosis (CF) patients, according to recent research. In contrast to the disease in humans, which…

Combining telehealth with home spirometry to assess lung function, along with some in-person visits to the doctor’s office, was as effective as in-person visits only to manage cystic fibrosis (CF) in children, a Swedish study reported. The findings suggest “that it may be safe to introduce telemedicine and home…

CFTR correctors, such as elexacaftor, a component of the cystic fibrosis (CF) therapy Trikafta, may improve lung function through enhanced potassium flow via a protein channel called BKCa, a study has found. Potassium flow via BKCa is known to support the hydration of lung surfaces, similar to CFTR,…

The diversity and composition of bacteria in the gut of adults with cystic fibrosis (CF) differed significantly from that of these patients’ non-CF counterparts in a new study, data revealed. Further, poor nutritional status and certain mutations in the CFTR gene, the underlying cause of CF — and…

Treatment with Kalydeco (ivacaftor) for almost eight years consistently improved lung function and reduced mortality in people with cystic fibrosis (CF), according to a study looking at several measures of disease progression. The therapy also significantly improved nutritional status, which together with reduced pulmonary exacerbations, hospitalizations, and lung…

The first cystic fibrosis (CF) patient has been dosed in Part A of a Phase 2 clinical trial in Europe that’s testing Enterprise Therapeutics‘ novel CF treatment candidate ETD001. The Phase 2a trial (NCT06478706) is still recruiting adults with CF at a few sites in the U.K. and…

Biofilms of Pseudomonas aeruginosa visualized directly in sputum (phlegm) samples collected from children with cystic fibrosis (CF) did not match biofilms when bacteria were first isolated from sputum and grown as an in vitro model, a study revealed. In vitro (in-the-lab) model systems are typically used to study…

Two non-invasive tests — MRI scans and the lung clearance index (LCI) — were more sensitive to early lung disease in children with cystic fibrosis (CF) than standard lung function tests, a study from Switzerland reports. Better ways of evaluating lung health in infants and children are needed,…

Lung transplant rates among people with cystic fibrosis (CF) in the U.S. have declined dramatically since the approval of Trikafta (elexacaftor/tezacaftor/ivacaftor), a study found. However, almost half of patients who received transplants in recent years were on Trikafta before their procedures. Moreover, a notable proportion of CF patients…

Nearly two-thirds of cystic fibrosis (CF) patients in a study showed signs of type 2 inflammation, which was associated with worse lung function, more infections, and a higher risk of death. This form of inflammation, most commonly linked to allergic conditions, was reduced somewhat after treatment with CFTR…