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Disorders of Ciliary Motility

 

作者: Mark Cowan,   Mark Gladwin,   James Shelhamer,  

 

期刊: The American Journal of the Medical Sciences  (OVID Available online 2001)
卷期: Volume 321, issue 1  

页码: 3-10

 

ISSN:0002-9629

 

年代: 2001

 

出版商: OVID

 

关键词: Kartagener syndrome;Primary ciliary dyskinesia;Cilia;Bronchiectasis.

 

数据来源: OVID

 

摘要:

Clearance of mucus and other debris from the airways is achieved by 3 main mechanisms: mucociliary activity, coughing, and alveolar clearance. Disorders of ciliary structure or function results in impaired clearance, and result 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 dysfunctional ciliary activity. The termprimary ciliary dyskinesiahas been proposed for the spectrum of these diseases. The termKartagener syndromeapplies to this syndrome when accompanied by infertility and dextrocardia or situs inversus. The more common types of ciliary dysmotility syndromes are characterized by missing dynein arms, central microtubule pairs, inner sheath, radial spokes, or nexin links. In addition to structural defects within the cilia, disordered ciliary beating and disordered ciliary arrays on epithelial cell surfaces have been described in this syndrome. Treatment includes rigorous lung physiotherapy, prophylactic and organism-specific antibiotics, and immunization against common pulmonary pathogens. Late stages of the disease may require surgical intervention for bronchiectasis or lung transplant for end-stage lung disease.

 

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