[Pulmonary metabolism of nitric oxide (NO) in patients with cystic fibrosis].
Airway nitric oxide (NO) and its metabolites are involved in a number of physiological and pathophysiological processes. For instance, NO relaxes airway smooth muscle, improves airway ciliary motility, has antimicrobial effects, and increases expression of the CFTR (cystic fibrosis transmembrane regulator) protein in airway epithelial cells. Of interest, concentrations of NO and of bioactive S-nitrosothiols (SNOs) are decreased in the airways of patients with cystic fibrosis (CF). When compared to patients with relatively normal pulmonary NO formation, CF patients with low NO-concentrations have a significantly reduced pulmonary function and a higher frequency of bacterial colonisation of the airways with pathogens such as P. aeruginosa. As a consequence of these observations clinical trails have now been initiated to study possible effects of an augmented bronchial NO-concentration in CF-patients.
Grasemann
H
H
Abteilung für Allgemeine Pädiatrie, Zentrum für Kinder und Jugendmedizin, Universitätsklinikum Essen, Germany. hartmutg@hotmail.com
Ratjen
F
F
D003550:Cystic Fibrosis / Q000378:metabolism*; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics; D006801:Humans; D008168:Lung / Q000378:metabolism*; D009569:Nitric Oxide / Q000378:metabolism*; D012016:Reference Values
C505032:CFTR protein, human; D019005:Cystic Fibrosis Transmembrane Conductance Regulator; D009569:Nitric Oxide
Besonderheiten im pulmonalen NO-Stoffwechsel bei Patienten mit zystischer Fibrose.