[An update on cystic fibrosis for the ENT-specialist].
Patients with cystic fibrosis (CF) show a high morbidity with otorhinolaryngological disorders, especially of the nasal airways and the paranasal sinuses. Because of the underlying basic genetic defect of the respiratory mucosa in CF many patients suffer from chronic rhinosinusitis, partially combined with the development of nasal polyps. Chronic rhinosinusitis in CF is different from chronic sinusitis in Non-CF-patients. In CF mainly neutrophils are found in the nasal epithelium, whereas in Non-CF-patients eosinophils predominate. In CF-sinuses a characteristic mucosal thickening and increase of high viscous mucus is seen which is not typically found in Non-CF-patients with chronic sinusitis. The respiratory mucosa in CF is mostly colonized with Staphylococcus und Pseudomonas. These bacteria participate in the damage of the respiratory epithelia and infection of the paranasal sinuses. Repeated administration of antibiotics frequently fails to reduce subjective symptoms of the patients or polyp formations, and sinus surgery is often necessary. New therapeutic approaches, such as postoperative inhalation with Dornase-alpha, are only empiric and not frequently recommended yet. Besides the strict indication for sinus surgery, which should only be performed on an inpatient basis, the hygienic handling with CF-patients needs special attention, particularly in medical centers with many patients. An effective genetic therapy for CF is currently not available.
Rozsasi
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Hals-Nasen-Ohrenklinik des Universitätsklinikums Ulm. ajnirozsasi@hotmail.com
Keck
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D003550:Cystic Fibrosis / Q000175:diagnosis* / Q000235:genetics / Q000628:therapy; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics; D004802:Eosinophilia / Q000175:diagnosis / Q000235:genetics / Q000628:therapy; D006801:Humans; D009154:Mutation / Q000235:genetics; D009298:Nasal Polyps / Q000175:diagnosis* / Q000235:genetics / Q000628:therapy; D011379:Prognosis; D011552:Pseudomonas Infections / Q000175:diagnosis / Q000628:therapy; D020545:Respiratory Mucosa / Q000473:pathology; D012141:Respiratory Tract Infections / Q000175:diagnosis* / Q000235:genetics / Q000628:therapy; D012220:Rhinitis / Q000175:diagnosis* / Q000235:genetics / Q000628:therapy; D012852:Sinusitis / Q000175:diagnosis* / Q000235:genetics / Q000628:therapy; D013203:Staphylococcal Infections / Q000175:diagnosis / Q000628:therapy
C505032:CFTR protein, human; D019005:Cystic Fibrosis Transmembrane Conductance Regulator