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DOI: 10.1055/s-2002-32164
Besonderheiten im pulmonalen NO-Stoffwechsel bei Patienten mit zystischer Fibrose
Pulmonary Metabolism of Nitric Oxide (NO) in Patients with Cystic FibrosisPublication History
Publication Date:
12 June 2002 (online)
Zusammenfassung
In den Atemwegen generiertes Stickstoffmonoxid (NO) und seine Metabolite sind an einer Vielzahl physiologischer und pathophysiologischer Prozesse beteiligt. Unter anderem relaxiert NO die bronchiale Muskulatur, verbessert die Motilität der Zilien, hat antimikrobielle Effekte, und erhöht die Expression des CFTR-(cystic fibrosis transmembrane regulation)Proteins in Atemwegsepithelzellen. Interessanterweise finden sich in den Atemwegen von Patienten mit zystischer Fibrose (CF) erniedrigte Konzentrationen von NO und von bioaktiven S-Nitrosothiolen (SNOs). Im Vergleich zu Patienten mit einer relativ normalen pulmonalen NO-Synthese weisen CF-Patienten mit niedrigen NO-Konzentrationen eine signifikant schlechtere Lungenfunktion auf und neigen vermehrt zu einer Besiedlung der Atemwege mit pathogenen Keimen wie P. aeruginosa. Als Konsequenz aus diesen Beobachtungen werden nun erste klinische Studien durchgeführt, die möglichen Effekte einer Erhöhung der bronchialen NO-Konzentration bei CF-Patienten untersuchen.
Abstract
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.
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Dr.
H. Grasemann
Zentrum für Kinder- und Jugendmedizin · Universitätsklinikum Essen
Hufelandstr. 55
45122 Essen
Email: hartmutg@hotmail.com