Marta Figueiredo, PhD, science writer —

A small compound called c407 restored the function of CFTR — the defective protein in cystic fibrosis (CF) — and its associated chloride flow in mice carrying F508del, the most common CF-causing mutation, a study shows. These benefits were associated with a stabilization of CFTR’s folding and its increased transport to the…

The frequency of cystic fibrosis-associated liver disease (CFLD) has increased in the past two decades and is linked to several simultaneous health complications, a U.S. study shows. CFLD was also found to be independently associated with a higher risk of mortality and increased healthcare resource use in children hospitalized due…

Cystic fibrosis (CF) patients with non-tuberculous mycobacteria (NTM) lung infection show significant changes in airway metabolites relative to those without such infections, a study shows. Metabolites are intermediate or end products of cellular metabolism, and some of these altered metabolites play roles in immune responses and bacterial growth. Combining…

A lung and pancreatic cell transplant from a single donor to people with end-stage cystic fibrosis (CF) and CF-related diabetes (CFRD) safely and effectively improved their lung function, metabolic control, and quality of life, data from a small Phase 1/2 trial show. Given that CFRD increases the risk of complications…

Adults with cystic fibrosis (CF) have significantly higher levels of anti-inflammatory oxylipins — a type of fatty molecule — and lower levels of pro-inflammatory fatty molecules in their saliva relative to healthy individuals, a study shows. This suggests a shift toward an anti-inflammatory response in the fatty molecule profile…

A noninvasive measure of lung function called lung clearance index (LCI) could be used to identify children and adolescents with cystic fibrosis (CF) who are at higher risk of a pulmonary exacerbation, an Italian study found. Notably, this work involved patients with normal or mildly reduced forced expiratory volume…

Multiple breath washout testing is superior to chest X-rays at detecting early signs of cystic fibrosis (CF) in infants and preschoolers, a study shows. The data also suggest that combining information from both techniques provides a considerable degree of assurance regarding the absence or potential presence of early lung…

Most people with cystic fibrosis (CF) responding to a U.S.-based online survey were generally satisfied with their treatment plan and were not considering changing their medication. The survey was conducted by Cystic Fibrosis News Today. Shortness of breath was the most commonly reported symptom among CF patients, as well as…

The introduction of CFTR modulators — a type of disease-modifying treatment for cystic fibrosis (CF) caused by specific mutations — has brought a general sense of hope to the lives of young adults with the disease, according to a small single-center U.S. study. Notably, patients reported greater physical…

More than half of children and teens with cystic fibrosis (CF) who do not show breathing difficulties during physical exercise were found to have reduced cardiorespiratory fitness (CRF), a measure of oxygen supply to the muscles, according to a single-center study in the Netherlands. Notably, glucose intolerance and…