Cystic fibrosis (CF), a monogenic disease caused by mutations in the CFTR gene on
chromosome 7, is complex and greatly variable in clinical expression. Airways, pancreas, male …

BACKGROUND: Open-label, parallel-group, international trial comparing aztreonam for
inhalation solution (AZLI) and tobramycin nebulizer solution (TNS) for cystic fibrosis patients with …

The innate immune response to adenovirus (Ad)-derived gene transfer vectors has been
shown to initiate immediately after interaction of Ad with respiratory epithelial cells, through the …

Rationale: Cystic fibrosis (CF) is a common genetic disease caused by mutations of the cystic
fibrosis transmembrane conductance regulator (CFTR) gene. Persistent lung inflammation, …

Background As survival among patients with cystic fibrosis has improved in recent decades,
complications have become increasingly relevant. The most frequent complication is cystic-…

BACKGROUND: The periodic administration of positive airway pressure combined with directed
cough could aid mucus clearance in patients with cystic fibrosis (CF) and severe airway …

Newborn screening (NBS) for cystic fibrosis (CF) has been gradually established in several
countries, but scant data are available on its long-term effects on survival. Our objective was …

BACKGROUND: Influenza, like other respiratory viral infections, can cause acute deterioration
of lung function in patients with cystic fibrosis (CF). Previous studies on a small number of …

Liver Transplant (LTx) has been rarely performed in cystic fibrosis (CF) patients and indications
and outcomes are not well defined. A questionnaire was sent to all European CF and …

This population-based study was conducted in Veneto and Trentino (northwestern Italy,
population 5 million). In this area, neonatal screening for cystic fibrosis started in 1973 and has …