Molecular and functional changes of pulmonary surfactant in response to hyperoxia.

Surfactant comprises phosphatidylcholine (PC) together with anionic phospholipids, neutral lipids, and surfactant proteins SP-A to-D. Its composition is highly specific, with dipalmitoyl-PC, palmitoyl-myristoyl-PC, and palmitoyl-palmitoleoyl-PC as its predominant PC species, but with low polyunsaturated phospholipids. Changes in pulmonary metabolism and function in response to injuries depend on their duration and whether adaptation can occur.

Cost of care and clinical condition in paediatric cystic fibrosis patients.

Background: The clinical course of cystic fibrosis (CF) shows considerable variation resulting in differences in health care utilisation. We investigated important clinical parameters and their relation to costs.

Methods: We collected clinical parameters together with health care utilisation of a representative paediatric CF population (n=138 patients) attending Hanover Medical School over a period of 1 year.

Long term azithromycin therapy in cystic fibrosis patients: a study on drug levels and sputum properties.

Background: Following reports on the treatment of diffuse panbronchiolitis (DPB), recent studies demonstrate that long term therapy with azithromycin (AZM) is effective in cystic fibrosis (CF) patients. However, the underlying mechanisms remain uncertain. Some macrolides, including AZM, display inhibition of virulence factors and other antipseudomonal effects at subinhibitory levels in vitro.

Cystic fibrosis transmembrane conductance regulator (CFTR)-mediated residual chloride secretion does not protect against early chronic Pseudomonas aeruginosa infection in F508del homozygous cystic fibrosis patients.

Cystic fibrosis (CF) disease severity is characterized by a broad variability that has been attributed, in addition to the CF transmembrane conductance regulator (CFTR) genotype, to modulating factors such as CFTR-mediated residual chloride (Cl-) secretion. Moreover, CFTR has been suggested to function as a receptor for Pseudomonas aeruginosa (PA). In this study, we investigated whether or not the presence of residual Cl- secretion protects against early chronic PA colonization of patients' airways.

Phosphatidylcholine metabolism of rat trachea in relation to lung parenchyma and surfactant.

Pulmonary surfactant prevents alveolar collapse and contributes to airway patency by reducing surface tension. Although alveolar surfactant, consisting mainly of phospholipids (PL) together with neutral lipids and surfactant-specific proteins, originates from type II pneumocytes, the contribution of airway epithelia to the PL fraction of conductive airway surfactant is still debated. We, therefore, analyzed the composition, synthesis, and release of phosphatidylcholine (PC) molecular species as the main surfactant PL of the rat trachea compared with the lung.