ENT manifestations in patients with primary ciliary dyskinesia: prevalence and significance of otorhinolaryngologic co-morbidities.

European Archives of Oto-rhino-laryngology : Official Journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : Affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery
J Ulrich SommerBoris A Stuck

Abstract

Primary ciliary dyskinesia (PCD) is a rare inherited disease with a prevalence of about 1:20,000. The underlying pathogenesis is disrupted ciliary function, which results in delayed mucus transportation leading to chronic inflammation, mainly in the upper and lower respiratory tract. Although the pathogenesis of the disease and its clinical presentation is somewhat understood, data regarding the prevalence of accompanying symptoms is limited, especially in the field of otorhinolaryngology. A total of 44 patients diagnosed with PCD answered a questionnaire regarding the diagnosis and clinical presentation of the disease, their medical history and clinical manifestations, and medical treatment in the field of otorhinolaryngology. The majority of participants (70%) had seen a physician more than 50 times before the diagnosis was made at an average age of 10.9 ± 14.4 years. As much as 59% of all patients had recurring problems at the paranasal sinuses and 69% of these patients needed corresponding surgical intervention. Even more patients (81%) suffered from recurring otitis media and, as a result, 78% of these patients underwent paracentesis with temporary tympanostomy tubes at least once at an average age of 9.5 ± 13.0 years. Oto...Continue Reading

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