Magdalena Kegel,  —

Among people with rare mutations in the CFTR gene, the gene defective in cystic fibrosis (CF), studies are needed to map down which patients may benefit from CFTR potentiators, such as Kalydeco (ivacaftor), or CFTR correctors. In this way, researchers will also be able to identify those in need…

Liver disease in cystic fibrosis (CF) patients needs to be detected much earlier, but early detection is increasingly likely as research interest in severe forms of the condition has grown. That was the message relayed during a talk by Dr. Daniel Leung of Baylor College Of Medicine in Texas, presented…

Abnormal mucus clearance in patients with cystic fibrosis (CF) may be improved with specific treatments, but despite intense research on the subject, there are many unanswered questions about how atypical mucus contributes to the disease and how to best improve the problem. Researchers debated the issue in three discussions at a…

Tai chi, taught either in face-to-face home sessions or using internet-based video calls, improved ease of breathing and posture in cystic fibrosis (CF) patients who participated in a trial. Findings also showed that patients felt calmer and less stressed, which likely contributed to the ease of breathing. The study,…

Cystic fibrosis (CF) patients carrying the F508del mutation in both CFTR gene copies maintained their lung function during more than two years of treatment with the combination drug Orkambi (ivacaftor/lumacaftor). The treatment also lowered the risk of acute exacerbations and improved patients’ weight. The study, “Reduction in Rate…

According to a new study, acute exacerbations of cystic fibrosis (CF) are associated with a surge of bacteria that do not depend on oxygen (called anaerobic or fermentative bacteria), which thrive when mucus becomes more acidic. Antibiotic treatment kills this bacteria, allowing Pseudomonas aeruginosa to thrive, but as the treatment effect wanes,…

Researchers at the Perelman School of Medicine at the University of Pennsylvania, Cincinnati Children’s Hospital and Boston University have been awarded a $5.2 million grant, running for seven years, for collaborative work to explore mechanisms of lung regeneration in diseases such as cystic fibrosis. The grant, one…

The experimental drug Translarna (ataluren) can make the cellular protein-making machinery run the so-called “stop sign” mutation in the cystic fibrosis (CF)-causing CFTR gene, replacing it with amino acids (the protein building blocks) similar enough to allow the protein to do its work. Translarna is currently in Phase 3 clinical…

Toxins produced by mold found on nuts and corn can exacerbate cystic fibrosis (CF) and pave the way for airway infections, as they block self-protective mechanisms for the lungs. The study, “Fungal Aflatoxins Reduce Respiratory Mucosal Ciliary Function,” published in the journal Scientific Reports, showed that the toxins…

Arch Biopartners and  Catalent Inhalation, a division of Catalent Pharma Solutions, have entered agreements to start manufacturing AB569 for human trials in patients with chronic and antibiotic resistant bacterial lung infections. Representing a large part of the group affected, patients with cystic fibrosis  (CF) stand to benefit most. AB569, invented by Daniel Hassett,…