Rationale: The promise of newborn screening (NBS) for cystic fibrosis (CF) has not been
fully realized, and the extent of improvement in respiratory outcomes is unclear. We …

Background Bronchiectasis develops early in the course of cystic fibrosis, being detectable
in infants as young as 10 weeks of age, and is persistent and progressive. We sought to …

… Representative images of CT scans showing normal inspiratory, A and expiratory scan, B;
bronchiectasis (indicated by white circles) with an extent of 1 in both the right and left lung C, …

Background Cross-sectional studies implicate neutrophilic inflammation and pulmonary
infection as risk factors for early structural lung disease in infants and young children with cystic …

Background. We hypothesized that the inflammatory response in the lungs of children with
cystic fibrosis (CF) would vary with the type of infecting organism, being greatest with …

… In addition, when specifically questioned 3–5 days after BAL, parents did not report fever
>38C or prolonged cough post BAL. Tobramycin trough levels were within nontoxic ranges and …

… Fever (>38 C) was reported within 24 h of the procedure in 48% (44/91), most commonly if
… and BAL, with high fever >38.5 C being more likely in children with lower respiratory tract …

… agar for Staphylococccus aureus was incubated at 35C for 48 h in an aerobic … C for 48 h,
then at 28C for a further 14 days; Fildes agar (for Haemophilus isolation) was incubated at 35C …

Background: The forced oscillation technique (FOT) requires minimal patient cooperation and
is feasible in preschool children. Few data exist on respiratory function changes measured …

Airway inflammation is an important component of cystic fibrosis (CF) lung disease. We
sought to determine whether alveolar macrophages were involved in early CF lung disease. …