An orally available small molecule called XL147 rescued the function of CFTR — the faulty protein in cystic fibrosis (CF) patients — in lab-grown airway cells from patients carrying F508del, the most common CF-causing mutation, a study shows. Adding XL147 to C18, a compound similar to the CFTR modulator…
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An endoscopic sinus surgery (ESS) is best performed within a few years of a lung transplant in cystic fibrosis (CF) patients, as longer wait times are associated with worse lung function and more antibiotic use, a small study reported. This finding could be relevant because “lung transplant recipients with CF…
One year of Trikafta treatment increases the blood levels of vitamin D in people with cystic fibrosis (CF) who are on vitamin D supplements. These early findings from a small study suggest that the therapy may improve vitamin D supplement absorption, and that vitamin D levels should be…
People with cystic fibrosis (CF) in the U.K. are encouraged to join the new CF BioResource project — giving them a unique opportunity to help connect genetic data to existing clinical data registries and advance understanding of CF. The ultimate goal of this project, conducted within the…
People with cystic fibrosis (CF) in the U.K. are now living longer, with the life expectancy of children born today surpassing 50 years for the first time, according to a newly published U.K. CF Registry annual data report. The report covered 2020, and the researchers noted: “In this extraordinary year,…
Pseudomonas aeruginosa, the most common bacterium infecting the lungs of people with cystic fibrosis (CF), appears to go through a number of changes that enables it to adapt during the course of an infection, a study says. Using samples of sputum (mucus brought up from the airways by…
People with cystic fibrosis (CF) who had a liver transplant can be safely started on Trikafta (elexacaftor/tezacaftor/ivacaftor), a small study suggests. For most patients in the study, Trikafta’s use resulted in an easing of symptoms, better quality of life, and healthier body weight and lung function. Doctors with…
People in the U.K. at highest risk of becoming seriously ill from COVID-19 — including those with cystic fibrosis (CF) — starting on Dec. 16 will have access to medications that can lessen the risk of severe disease through the country’s National Health Service (NHS). Two medications will be available:…
Blocking the protein SGLT1, which regulates glucose uptake in cells, may be a useful in treating cystic fibrosis (CF), according to research done in a cell model of the disease. SGLT1 inhibitors are likely less effective that currently approved modulators for patients with eligible mutations, its scientists noted, but…
Many women with cystic fibrosis (CF) wish to have biological children, but they often lack proper education and resources about fertility preservation, a U.S. study found. The study, “Fertility preservation in women with cystic fibrosis pre-lung transplantation: A mixed methods study,” was published in the Journal…
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