Steve Bryson, PhD,  science writer—

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…

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,…

A woman and her daughter were diagnosed with chronic rhinosinusitis, an inflammatory condition of the sinuses and nasal passages, with features matching cystic fibrosis (CF), a case study reports. However, neither patient carried mutations in the CFTR gene that cause CF. The pair also presented with signs and…

One year of Trikafta treatment improved lung function, reduced abnormalities in lung structure, and halted disease progression among school-age children, ages 6-11, with cystic fibrosis (CF), according to a real-world analysis. “The majority of the children even achieved normal lung function with the triple combination therapy. That’s a…

Using a newly enhanced gene-editing technology called prime editing, researchers in the U.S. have efficiently corrected the most common mutation that causes cystic fibrosis (CF) in human lung cells. By correcting this mutation, known as F508del, in the CFTR gene, scientists at the Broad Institute of MIT and Harvard,…

Arcturus Therapeutics plans to soon submit an application to the U.S. Food and Drug Administration (FDA) seeking permission to begin testing multiple ascending doses of ARCT-032, an inhaled experimental treatment for cystic fibrosis (CF). The planned Phase 2 study, supported by promising safety and preliminary efficacy data…

Among young children with cystic fibrosis (CF), starting the approved oral therapy Orkambi (ivacaftor/lumacaftor) led to favorable changes in nutrition and growth status during the first six months of treatment, according to a small real-world study. Orkambi, first approved in the U.S. in 2015 and now indicated for…

Despite some similarities, the lung composition of microbes and their antimicrobial resistance profiles differed between people with bronchiectasis and cystic fibrosis (CF) and those with non-CF bronchiectasis, a study found. The findings suggest “the need for customized management strategies for each disease,” the researchers wrote in the study, “…