[Rhinosinusitis in cystic fibrosis].
In cystic fibrosis (CF) mucociliary clearance of the entire respiratory system is impaired. This allows pathogens, such as Pseudomonas aeruginosa to persist and proliferate, which by progressive pulmonary destruction causes 90 % of premature deaths due to this inherited disease. The dramatic improvement in life expectation of patients due to intensive therapy has resulted in the inevitable but variably expressed sinonasal involvement coming into the clinical and scientific focus. Thereby, almost all CF patients reveal sinonasal pathology and many suffer from chronic rhinosinusitis. Recently, the sinonasal niche has been recognized as a site of initial and persistent colonization by pathogens. This article presents the pathophysiological background of this multiorgan disease as well as general diagnostic and therapeutic standards. The focus of this article is on sinonasal involvement and conservative and surgical options for treatment. Prevention of pathogen acquisition is an essential issue in the otorhinolaryngological treatment of CF patients.
Mukoviszidosezentrum für Kinder und Erwachsene, Universitätsklinikum Jena, Jena, Deutschland
J G
JG
Mainz
A
A
Gerber
C
C
Arnold
J
J
Baumann
I
I
Baumann
A
A
Koitschev
D003550:Cystic Fibrosis / Q000150:complications / Q000175:diagnosis* / Q000628:therapy*; D006801:Humans; D012220:Rhinitis / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy*; D012852:Sinusitis / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy*