Point of care lung ultrasound in preschool children with cystic fibrosis: a case-controlled, prospective, pilot study.

Aims: Respiratory complications in Cystic Fibrosis (CF) are still the leading cause of death nowadays in these patients. High-Resolution Computed Tomography is the gold standard method for staging lung disease in CF. In this study we assessed lung ultrasound findings in asymptomatic preschool patients affected by CF.

Ivacaftor improves lung disease in patients with advanced CF carrying CFTR mutations that confer residual function.

Background: Ivacaftor is an innovative treatment for CF. Ivacaftor monotherapy in a phase III trial for patients with F508del and a residual function (RF) mutation showed improvement in lung function. We evaluated the effectiveness and safety of ivacaftor in patients with severe CF carrying RF mutations.

A questionnaire-based study in Calabria on the knowledge of off-label drugs in pediatrics.

Off-label use is very common among pediatricians, and the main cause is attributable to the lack of drugs specifically designed and marketed for children in different age groups. In this study, we evaluated, through a questionnaire, the knowledge of off-label drugs in pediatrics. Furthermore, we made a directory of 28 off-label drugs most commonly used by pediatricians in agreement with data obtained from Italian Agency for drugs (AIFA) referred to the law no.