ENT manifestations in patients with primary ciliary dyskinesia: prevalence and signiWcance of otorhinolaryngologic co-morbidities
- نوع فایل : کتاب
- زبان : انگلیسی
- مؤلف : J. Ulrich Sommer · Kerstin Schنfer · Heymut Omran · Heike Olbrich · Julia Wallmeier · Andreas Blum · Karl Hِrmann · Boris A. Stuck
- چاپ و سال / کشور: 2011
Description
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 inXammation, 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 Weld 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 Weld 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%) suVered 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. Otorhinolaryngologic symptoms, especially chronic otitis media and chronic rhinosinusitis, are frequently associated with PCD. Surgical intervention to treat these symptoms is common. The awareness of this disease should be raised, especially among ENT physicians, and surgical intervention should be indicated carefully
Eur Arch Otorhinolaryngol (2011) 268:383–388, Received: 16 May 2010 / Accepted: 7 July 2010 / Published online: 22 July 2010 © Springer-Verlag 2010
