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Epithelial cells collected from the nasal passages and grown in the lab under a specialized protocol could be a useful model for studying the molecular underpinnings of cystic fibrosis (CF) and testing potential treatments, a study reported. The study, “Correlating genotype with phenotype using CFTR-mediated whole-cell…

A complete analysis of the CFTR gene, which is defective in cystic fibrosis (CF), identifies potential disease-causing mutations that would be missed by standard testing, as well as variations that may explain differences in disease course and treatment responses, a study of more than 5,000 CF patients shows. Still,…

Urinary incontinence, known to affect both girls and women with cystic fibrosis (CF), is increasingly prevalent as these patients age, and coughing or sneezing were common triggers among adults who took part in a study at a single site in Italy. Incontinence during physical exercise was also fairly common…

People with cystic fibrosis (CF) who score highly on measures of self-compassion tend also to report higher quality of life, a study found. “The current findings suggest that psychological interventions which increase self-compassion could be beneficial for enhancing better mental health and quality of life for adults with CF,”…

Inadequate nutrition, poorly working or chronically infected lungs, and mutations leading to more severe disease can shorten the lifespan of people with cystic fibrosis (CF), a study in adult patients reported. “All these factors interact with each other,” its scientists wrote. “Therefore, it is important to take into account…

Researchers generated functional bile duct cells from human-derived stem cells that can be used to support the development of therapies for liver disease in people with cystic fibrosis (CF), a study reported. “These studies highlight the importance of generating mature cells from stem cells that faithfully mimic the functional…

The microbiome diversity in the lungs of cystic fibrosis (CF) patients increased after treatment with Trikafta, becoming similar to a person without CF, an analysis showed. In addition, small molecule metabolites produced by cells varied widely across patients while on the therapy, with the largest decrease seen in…

For the first time, many features of cystic fibrosis (CF) were modeled accurately in a human Lung Airway Chip lined with cells from CF patients, which included enhanced mucus secretion, inflammation, and microbial infections, a study has shown. This “organ-on-a-chip model” of the disease may support the rapid development…

Large-scale clinical trials and other research are “urgently required” to determine the best ways to manage the growing number of infections with nontuberculous mycobacteria — known as NTM — among children with cystic fibrosis (CF). That’s the argument made by a team of scientists in the U.K., in the…

Eloxx Pharmaceuticals’ experimental therapy ELX-02 was generally safe and resulted in a significant reduction in sweat chloride levels — a biomarker of cystic fibrosis (CF) — in patients carrying the most common CF-causing nonsense mutation, according to top-line data from a Phase 2 clinical program. While the short…