This comprehensive State of the Art review summarizes the current published knowledge
base regarding the pathophysiology and microbiology of pulmonary disease in cystic …

Background and Methods We conducted two multicenter, double-blind, placebo-controlled
trials of intermittent administration of inhaled tobramycin in patients with cystic fibrosis and …

Chronic pulmonary infection with Pseudomonas aeruginosa continues to be the major
cause of morbidity and mortality in cystic fibrosis (CF). Several characteristics of CF …

Background The Cystic Fibrosis Questionnaire (CFQ) is a disease-specific instrument that
measures health-related quality of life (HRQOL) for adolescents and adults with cystic …

Cystic fibrosis (CF) is caused by mutations in the cystic fibrosis transmembrane conductance
regulator (CFTR) gene [1, 2]. This results in dysfunction of the apical membrane CFTR …

With the improving survival of patients with cystic fibrosis (CF), the clinical spectrum of this
complex multisystem disease continues to evolve. One of the most important clinical events …

Objective: To determine the clinical consequences of acquiring Pseudomonas aeruginosa
infection during early childhood in children with cystic fibrosis (CF). Design: Prospective …

OBJECTIVE: To assess pulmonary abnormalities detected by high-resolution computed
tomography (HRCT) in young children with cystic fibrosis (CF) and mild to moderate lung …

Background Many patients with cystic fibrosis are malnourished at the time of diagnosis.
Whether newborn screening and early treatment may prevent the development of a …

Objectives: To evaluate the effects of a 3-year home exercise program on pulmonary
function and exercise tolerance in mildly to moderately impaired patients with cystic fibrosis …