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…
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…
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