News

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…

In children with cystic fibrosis (CF), chronic lung infection with methicillin-susceptible Staphylococcus aureus (MSSA) before age 4 was associated with poor lung function by age 8, a small study concluded. Early infection was also linked to an increased risk later of lung exacerbations, or a rapid worsening of symptoms.

Children who carry certain mutations associated with cystic fibrosis (CF), but do not have the disease — called CF carriers — may be more likely to develop asthma than children who don’t carry a mutation, a California study found. In particular, inheriting one copy of the most common CF-causing…

Most military cystic fibrosis (CF) centers in the U.S. are generally following mental health screening guidelines — but there’s still room for improvement — a study showed. Specifically, the identified areas needing improvement include the standardized use of screening tools for suicidal thoughts, improving healthcare providers’ comfort with all…