News

First Wave BioPharma has developed a new optimized formulation of adrulipase, a yeast-derived enzyme designed to treat exocrine pancreatic insufficiency in people with cystic fibrosis (CF). The new formulation ensures that the enzyme is packed in acid-resistant micro-granules and protected from the acid environment of the stomach. The enzyme then reaches…

In children with cystic fibrosis (CF), certain genetic variants were found to be associated with the rate of absorption of the antibacterial agent ciprofloxacin — used to prevent bacterial infections, mainly those caused by Pseudomonas aeruginosa — according to a new study. Researchers believe that these new data will…

The Cystic Fibrosis Foundation (CFF) has joined the CEO Action for Diversity and Inclusion, adding its name to the more than 2,300 corporate leaders, representing 85 industries, who have pledged to bring greater inclusion and diversity into their workplaces. Organizations with diverse teams perform better, the CFF stated…

The U.S. Food and Drug Administration (FDA) approved the expanded use of Orkambi (lumacaftor/ivacaftor) to treat children starting at 12 months old with cystic fibrosis (CF) who have two copies of the F508del mutation in the cystic…

A poorer adherence to pulmonary therapies does not seem to greatly influence disease severity in patients with cystic fibrosis (CF) who have a nearly normal lung function and good medication adherence rates overall, a study suggests. For this subgroup of patients, CF severity seems to not be associated with…

A defect in an anti-inflammatory pathway called IL-37-SIGIRR contributes to the hyperinflammation seen in lung epithelial cells in patients with cystic fibrosis (CF), a new study suggests. The study shows, for the first time, that a mutated form of the single immunoglobulin interleukin-1 receptor (IL-1R)-related molecule (SIGIRR), named delta…

The clinical outcome of lung infection with Mycobacterium abscessus (M. abscessus) in cystic fibrosis (CF) patients is associated with genetic differences in the bacterium strains, according to a recent study. The study identified virulence factors related to disease severity, and sheds light on the development of novel and more effective…

Children with cystic fibrosis (CF) being treated with Orkambi (ivacaftor/lumacaftor) have distinct clinical response profiles after six months of treatment in a real-world setting, a French-Italian study found. While changes in lung function were the largest driver of treatment responses in older children ages 12–18, body mass index…

A compound called beta-sitosterol, abundant in plants, eased the burden of Pseudomonas aeruginosa infection — a common bacteria infecting the lungs of people with cystic fibrosis (CF) — and lessened chronic inflammation in a mouse model. Findings suggest that beta-sitosterol, or BSS, “has the potential to undergo pharmaceutical development…

Exposure to carbon nanoparticles — tiny air pollution particles that account for a substantial part of air pollution in urban areas — significantly reduces the levels of CFTR, the faulty protein in cystic fibrosis (CF), according to the results of a new study. Being exposed to these air pollution…