Rhinitis, sinusitis and otitis media in Kartagener's syndrome (primary ciliary dyskinesia).
Ear, nose and throat symptoms and signs were studied in 15 patients with Kartagener's syndrome: a triad consisting of chronic rhinosinusitis, chronic bronchitis with bronchiectasis, and situs inversus. The triad is caused by primary ciliary dyskinesia and characterized by absent or considerably reduced mucociliary transport. Daily accumulation of secretions in the nose, chronic recurrent secretory otitis media and sinusitis occurred in all subjects. Characteristically, nasal discharge started at birth or was first detected in early childhood. Apparently, the frequency of common colds and of acute purulent otitis media was not increased. As primary ciliary dyskinesia can occur without situs inversus, knowledge of the typical ENT symptoms is essential for making an early diagnosis, which is important for the correct management of the disease. While a conservative surgical approach to treatment of the ENT symptoms is recommended, early and active treatment of the bronchial symptoms is probably important for prevention of further lung damage and development of bronchiectasis.
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D002923:Cilia / Q000502:physiology; D005260:Female; D006801:Humans; D007619:Kartagener Syndrome / Q000150:complications / Q000175:diagnosis* / Q000628:therapy; D008297:Male; D008875:Middle Aged; D010033:Otitis Media / Q000150:complications / Q000503:physiopathology*; D012220:Rhinitis / Q000150:complications / Q000503:physiopathology* / Q000628:therapy