Disorders of ciliary motility

Clearance of mucus and other debris from the airways is achieved by 3 main mechanisms: mucociliary activity, coughing, and alveolar clearance. Disorde rs of ciliary structure or function results in impaired clearance, and resu lt in chronic sinopulmonary disease manifested as chronic sinusitis, otitis media, nasal polyposis, and ultimately bronchiectasis. In addition, situs inversus, dextrocardia, and infertility can be associated with dysfunctiona l ciliary activity. The term primary ciliary dyskinesia has been proposed f or the spectrum of these diseases. The term Kartagener syndrome applies to this syndrome when accompanied by infertility and dextrocardia or situs inv ersus. The more common types of ciliary dysmotility syndromes are character ized by missing dynein arms, central microtubule pairs, inner sheath, radia l spokes, or nexin links. In addition to structural defects within the cili a, disordered ciliary beating and disordered ciliary arrays on epithelial c ell surfaces have been described in this syndrome. Treatment includes rigor ous lung physiotherapy, prophylactic and organism-specific antibiotics, and immunization against common pulmonary pathogens. Late stages of the diseas e may require surgical intervention for bronchiectasis or lung transplant f or end-stage lung disease.