Primary ciliary dyskinesia and upper airway diseases

被引:0
作者
Marcus P. Kennedy
Lawrence E. Ostrowski
机构
[1] University of North Carolina,Cystic Fibrosis Pulmonary Research and Treatment Center
[2] School of Medicine,undefined
来源
Current Allergy and Asthma Reports | 2006年 / 6卷
关键词
Respir Crit; Mucociliary Clearance; Primary Ciliary Dyskinesia; Situs Inversus; Ciliary Function;
D O I
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中图分类号
学科分类号
摘要
Primary ciliary dyskinesia (PCD) is a rare and difficultto-diagnose disease with morbidity related to infections of the upper and lower respiratory tract. The disease is caused by mutations in genes that are required for proper ciliary function. The defect in ciliary function results in reduced or absent mucociliary clearance, thereby predisposing the affected individual to repeated bacterial infections. Recent advances in the understanding of the basic biology and function of the cilium have led to the identification of some of the genes that are mutated in cases of PCD. Further studies of this disease will likely lead to earlier diagnosis, better treatment, and improved outcomes.
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页码:513 / 517
页数:4
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