Systemic antifibrinolytic therapy in adult patients with cystic fibrosis has been associated with a reduced rate of hospital admissions for hemoptysis.
Improvements in pancreatic function biomarkers, including increases in fecal elastase-1 and decreases in serum immunoreactive trypsinogen, were seen in patients treated with lumacaftor and ivacaftor.
Inhaled liposomal ciprofloxacin is associated with a longer median time to first pulmonary exacerbation vs placebo in patients with non-cystic fibrosis bronchiectasis and chronic P aeruginosa lung infection requiring antibiotic therapy.
Due to brain excitability and hypoxia from coughing, patients with cystic fibrosis have a propensity for cortical spreading depression and hemiplegic migraine.
Mild bronchiectasis in smokers is associated with distal pulmonary vascular pruning.
Including invalid data decreases the validity of multiple breath washout measurements in clinical trials involving adults with bronchiectasis.
VX-445-tezacaftor-ivacaftor led to noticeable improvements in Phe508del CFTR protein function.
Adding VX-659 to tezacaftor and ivacaftor therapy improved lung function in patients with cystic fibrosis.