Upper Respiratory Tract Involvement in Primary Ciliary Dyskinesia: Therapeutic Continuity
摘要
Primary ciliary dyskinesia (PCD) is a rare autosomal recessive genetic disease characterized by cilia dysfunction. Chronic rhinosinusitis, rhinorrhea or nasal congestion, acute otitis, recurrent otitis media with effusion, hearing impairment, speech problems, recurrent infections, and bronchiectasis can be seen in PCD. Clinical findings can be variable according to the age. The main goals in PCD treatment are to increase mucociliary clearance, to prevent repeated upper and lower respiratory tract infections due to mucociliary activity disorder and reduce inflammation. Protective measures and vaccination are important issues in the follow-up of children with PCD. Different treatment options are available depending on otologic, sinonasal, and pulmonologic disease.