News

Researchers have identified a set of genes that could represent therapeutic targets to prevent severe complications from COVID-19 infection in people with cystic fibrosis (CF). Three of these genes were at higher levels in patients with either COVID-19, CF, or chronic kidney disease (CKD), and a larger network of…

In children with cystic fibrosis (CF), elevated levels of programmed death protein 1 (PD-1) in cells called macrophages are linked with lung disease, inflammation and infection, a study reports. Blocking PD-1 activation resulted in more efficient killing of Pseudomonas aeruginosa, the most common bacteria found in CF patients.

About 10% of adolescents and young adults with cystic fibrosis (CF) have abnormal eating patterns, according to a single-center study. Results suggested that nutrition-focused subscales of the Cystic Fibrosis Questionnaire-Revised (CFQ-R), a tool commonly used for assessing health-related quality of life in CF, may help identify young patients at risk…

People with cystic fibrosis (CF) treated with Kalydeco (ivacaftor) may have higher levels of the medication in their body than is needed for a therapeutic effect, according to a new study. “A key finding of our study revealed that some patients have higher than expected [Kalydeco] concentrations which…

The advent of newborn screening (NBS) programs in the U.S. a decade ago is linked to better outcomes today in children with cystic fibrosis (CF), a new study reports. Results show that children born after CF newborn screening became available in their states have improved nutritional outcomes, better…

The Phase 1 clinical trial into the safety and pharmacological properties of the antibiotic Xenleta (lefamulin) in adults with cystic fibrosis (CF) is fully enrolled. “We are happy to announce enrollment has been completed in this important study to evaluate the potential of XENLETA in the management of…

Treatment with the triple-combination therapy Trikafta improved lung function and eased respiratory symptoms for school-aged children with cystic fibrosis (CF) in a Phase 3 clinical trial. “I was both surprised and delighted to see that, even this early in the disease trajectory and despite the brief treatment duration,…

Efforts to promote physical activity in people with cystic fibrosis (CF) should be holistically integrated into routine care, rather than delivered as separate interventions. “The key message resulting from the current research is that the promotion of [physical activity] in this population would be most effective as part of…

Children with cystic fibrosis (CF) whose intestinal microbe populations — or gut microbiome — were dominated by Bifidobacteria had fewer hospitalizations and pulmonary exacerbations, needed fewer antibiotics, and showed lower inflammation in the gut than children with a gut microbiota favoring Bacteroides, a study reported. A dominance of Bifidobacteria…

Targeting a protein called eRF3a may offer a way of treating cystic fibrosis (CF) caused by nonsense mutations, potentially opening a treatment avenue to people whose rare disease-causing mutations do not respond to current CFTR modulators, researchers reported. Their study, “Small molecule eRF3a degraders rescue…