Although cystic fibrosis (CF) patients display multiorgan dysfunction (eg pancreas, gut, and
lung) it is lung disease that is the leading cause of premature death in these patients. CF …

Background The airways in patients with cystic fibrosis (CF) are characterized by the
accumulation of tenacious, dehydrated mucus that is a precursor for chronic infection …

Epithelial sodium channels (ENaC) are processed by proteases as they transit the
biosynthetic pathway. We recently observed that furin-dependent processing of the α …

Background Inhaled hypertonic saline (HS) improves lung function and decreases
pulmonary exacerbations in older patients with cystic fibrosis (CF). Initiating therapies in …

Cystic fibrosis (CF) is an autosomal recessive disease caused by a defect in the cystic
fibrosis transmembrane conductance regulator (CFTR) gene. CFTR is expressed in the …

Introdução: O uso de solução salina hipertônica já foi documentado em DPOC como um
método de broncoprovocação e indução de escarro, mas estudos que avaliem outros …