Small changes in the shape of CFTR, the protein defective or absent in people with cystic fibrosis (CF), opened its gate outside the cell to allow the flow of chloride ions, a study reported. CF-causing mutations that suppress CFTR function disrupt these long-distance shape changes. By comparison, CFTR potentiator…
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Trikafta significantly improved the lung function of cystic fibrosis patients after only two weeks of treatment, with the benefits sustained after nearly four months, according to real-world data from a single center in Austria. “Early LCI [lung clearance index] measurements can help to assess the patient’s response to this…
An exclusive telehealth care model used during the COVID-19 pandemic was associated with a decrease in pulmonary function in Australian children with cystic fibrosis (CF). “It is likely that telehealth based care should complement, rather than replace in person assessment,” according to the researchers. “Further work is needed to…
Communities of fungi in the airways can be more diverse in cystic fibrosis (CF) patients with better lung function and those using medications known as CFTR modulators, a study found. “As CF lung disease progresses, bacterial richness and diversity are known to decrease, concomitant with increased absolute and relative…
Overactive T2 inflammation, a type of immune response linked to asthma, may be associated with worse symptoms of cystic fibrosis (CF), a finding that could pave the way for using anti-asthmatics for CF, a study suggests. The study, “Association between Cystic Fibrosis exacerbations, lung function, T2 inflammation and…
Packets of molecular cargo called extracellular vesicles (EVs) can be isolated from the blood of people with cystic fibrosis (CF) and their contents may differ from those who don’t have the disease. The findings also suggest these contents in CF may be changed by CFTR modulator therapy and…
A new company, Inspire Biotherapeutics, has launched with the goal of developing a new gene therapy platform that could be used in the treatment of cystic fibrosis (CF) and other disorders that affect the lungs. Inspire is planning to launch a first-in-human clinical trial to test the novel…
The Cystic Fibrosis Foundation is investing up to $2 million in Nanite to develop new non-viral methods of delivering gene therapies into the lungs of people with cystic fibrosis (CF). “We’re thrilled to announce this investment from the Cystic Fibrosis Foundation, which we will use to explore…
A better growth trajectory over the first years of life was associated with more favorable lung function at age 6 for children with cystic fibrosis (CF) in the U.S., a study reported. Children who were consistently above the 50th percentile in size through age 5 — as recommended by…
AR-501, an experimental, nonantibiotic inhaled treatment for chronic lung infections in cystic fibrosis (CF), was well tolerated among adults patients in a Phase 2a clinical trial. Top-line data, announced by the therapy’s developer Aridis Pharmaceuticals, suggest that the medication was present in lung fluid samples at concentrations…
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