News

Kalydeco (ivacaftor) is known to increase the activity of the cystic fibrosis transmembrane conductance regulator (CFTR) protein in cystic fibrosis (CF) patients. A new study says the drug also improves lung function and reduces inflammation, but does not eradicate Pseudomonas bacteria infection. The study, “Restoring CFTR Function Reduces Airway Bacteria…

Bone marrow cells can improve overall lung function and may have potential therapeutic benefits in treating cystic fibrosis, suggests a study published in the journal Molecular Therapy.

Anti-infective agents such as Mycamine and Coly-mycin M can help prevent formation of Exophiala dermatitidis associated-biofilm structures, commonly found in cystic fibrosis (CF) patients, a new study found. The study, “Biofilm formation of the black yeast-like fungus Exophiala dermatitidis and its susceptibility to antiinfective agents,” appeared in…

Researchers found a new defect called the R248G mutation in the CFTR gene in three Spanish siblings whose diagnoses indicate borderline cystic fibrosis, and suggest a possible link between this defect, pancreatitis (inflammation of the pancreas), and infertility. The findings were reported in the article “R248G cystic…

Scientists at the University of California-Riverside have developed a novel way to help target therapeutics for cystic fibrosis (CF) and other diseases. Their study, “Inhibition of nonsense-mediated RNA decay by ER stress,” appeared in the journal RNA. The information encoded in human DNA is first converted to RNA, and…

Once-a-day treatment with the antibiotic tobramycin does not harm the kidneys of patients with advanced cystic fibrosis (CF), a new study has found. Tobramycin taken three times a day was previously linked to acute and chronic kidney failure in CF patients. The study, “Glomerular and tubular renal function after repeated…

Rare mutations in the CFTR gene — which is defective in patients with cystic fibrosis (CF) — may occur in specific ethnic groups, suggests a new study that urges wider screening of mutations in patients who test negative for most previously identified mutations linked to CF. The study, “A rare CFTR mutation…

Certain bacteria may increase the risk of fungal colonization in cystic fibrosis (CF) patients, according to a study in Germany. CF makes a patient susceptible to chronic airway inflammation and infections, resulting in lung tissue damage. Scientists have long known that bacteria such as Pseudomonas aeruginosa and Staphylococcus aureus play roles…

Evaluating patients with cystic fibrosis (CF) before they board a flight may help predict whether they will need supplemental oxygen while in the air to prevent a severe decrease in blood oxygen levels, according to a study. The research, “Pre-Flight Evaluation Of Adult Patients With Cystic Fibrosis: A Cross-Sectional Study,”  was…

Researchers are developing a compact respiratory device that will allow children whose lungs have failed from cystic fibrosis (CF) or other diseases to move about a hospital rather than having to stay in a bed during treatment or while awaiting a transplant. The Ambulatory Assist Lung for Children project…