New measures are needed to rapidly assess emerging treatments for cystic fibrosis (CF) lung disease. Using an imaging approach, we evaluated the absorptive clearance of the radiolabeled small molecule probe diethylene triamine penta-acetic acid (DTPA) as an in vivo indicator of changes in airway liquid absorption. DTPA absorption and mucociliary clearance rates were measured in 21 patients with...

mutation has an incidence of 43.5% (4 ), lower than in the rest of Spain (53.2%) (3 ), the incidence of these 11 mutations may be higher. Used in conjunction with the 31 mutations analyzed with the CF Genetic Analysis assay from Applied Biosystems, the multimutational analysis of the 11 mutations presented here could enhance the detection rate in Spanish and Mediterranean populations to 80%.

Objective: To observe the gains in bone mass in children and adolescents with cystic fibrosis (CF) over 24 months and examine the relationship between areal bone mineral density (aBMD) and associated clinical parameters including physical activity, nutrition and 25-hydroxyvitamin D (25OHD). Methods: Areal BMD of the total body (TB), lumbar spine (LS) and total femoral neck (FNt) were repeatedly...

In patients with cystic fibrosis (CF) and non-CF bronchiectasis, Pseudomonas aeruginosa is the most important respiratory pathogen. It is able to synthesise hydrogen cyanide, a potent inhibitor of cellular respiration. The present study investigated whether cyanide is present in the sputum of CF and non-CF bronchiectasis patients infected with P. aeruginosa, and whether the detection of cyanide...

background cystic fibrosis (cf) is a genetic disease with an autosomal recessive pattern of inheritance. cf caused by a mutation in the cystic fibrosis transmembrane conductance regulator (cftr) gene and characterized by impaired transport of chloride ions across the cell membrane. staphylococcus aureus, pseudomonas aeruginosa, and burkholderia cepacia have been identified in the cultures of re...

BACKGROUND Cystic fibrosis (CF) with pancreatic insufficiency is associated with poor absorption of fat and fat-soluble vitamins, including vitamin D. Pancreatic enzyme supplementation does not completely correct fat malabsorption in CF patients. OBJECTIVE The objective of the study was to compare the vitamin D status of children, adolescents, and young adults with CF who were treated with ro...

Chronic systemic inflammation is a clinical symptom in children with cystic fibrosis (CF), but the effects on skeletal muscle development are unknown. The aims of this study were to determine (1) the effects of systemic factors from children with CF and healthy controls on myoblast proliferation, and (2) whether exercise serum can have an effect on proliferation in vitro. Eleven children with C...

Cloud fraction (CF) plays a crucial role in the Earth’s radiative energy budget and thus in the climate. Reliable long-term measurements of CF are rare. The ground-based TROpospheric WAter RAdiometer (TROWARA) at Bern, Switzerland continuously measures integrated liquid water and infrared brightness temperature with a time resolution of 6–11 s since 2004. The view direction of TROWARA is consta...

BACKGROUND Microparticles (MPs) are membrane vesicles released during cell activation and apoptosis. MPs have different biological effects depending on the cell from they originate. Cystic fibrosis (CF) lung disease is characterized by massive neutrophil granulocyte influx in the airways, their activation and eventually apoptosis. We investigated on the presence and phenotype of MPs in the sput...