context cystic fibrosis (cf) is the most widespread autosomal recessive genetic disorder that limits life expectation amongst the caucasian population. as the median survival has increased related to early multidisciplinary intervention, other manifestations of cf have emergedespecially for the broad spectrum of hepatobiliary involvement. the present study reviews the existing literature on liv...

A relevant proportion of children and adults with cystic fibrosis (CF) have a marked decrease in exercise tolerance, which can be partly related to impaired muscle function and decreased physical activity levels in daily life, in addition to lung disease. Preliminary findings suggest that patients with non-CF bronchiectasis face the same problems. These patients might be excellent candidates fo...

Cystic fibrosis (CF) is caused by mutations in the CF transmembrane conductance regulator (CFTR), a channel that normally transports anions across epithelial cell membranes. The most common manifestation of CF is buildup of mucus in the airways and bacterial colonization of the lower respiratory tract, accompanied by chronic inflammation. Antibiotics are used to control CF-associated opportunis...

Cystic fibrosis (CF) is a multisystem, life-shortening genetic disease that requires complex care. To facilitate this expert, multidisciplinary care, the CF Foundation established a Care Center Network and accredited the first care centres in 1961. This model of care brings together physicians and specialists from other disciplines to provide care, facilitate basic and clinical research, and ed...

The ability to integrate and weight information across dimensions is central to perception and is particularly important for speech categorization. The present experiments investigate cue weighting by training participants to categorize sounds drawn from a two-dimensional acoustic space defined by the center frequency (CF) and modulation frequency (MF) of frequency-modulated sine waves. These d...

OBJECTIVE To understand the role of religiousness/spirituality in coping in children with cystic fibrosis (CF). METHODS Participants were a convenience sample of 23 patients with CF, ages 5 to 12 years, and their parent(s) in an ambulatory CF clinic. The design was a focused ethnography including in-depth interviews with children and parent(s), children's drawings, and self-administered writt...

Staphylococcus aureus is one of the first pathogens which often persistently infect the airways of cystic fibrosis (CF) patients. Nasal S. aureus carriage is a risk factor for S. aureus infections in non-CF patients. Topical treatment strategies successfully eradicate nasal S. aureus carriage, thereby decreasing S. aureus infection. A prospective longitudinal multicenter study was conducted to ...

Cystic fibrosis (CF) results in inflammation, malabsorption of fats and other nutrients, and obstruction in the gastrointestinal (GI) tract, yet the mechanisms linking these disease manifestations to microbiome composition remain largely unexplored. Here we used metagenomic analysis to systematically characterize fecal microbiomes of children with and without CF, demonstrating marked CF-associa...