Primary ciliary dyskinesia (PCD) is an inherited,genetically heterogeneous disorder characterized by the dysfunction of motile cilia that are found in a number of human tissues including the upper and lower airways ep...Primary ciliary dyskinesia (PCD) is an inherited,genetically heterogeneous disorder characterized by the dysfunction of motile cilia that are found in a number of human tissues including the upper and lower airways epithelium (respiratory cilia).Respiratory cilia project from the apical membrane of epithelial cells that line up the airways and through their active and coordinated beating,drive the process of mucociliary clearance,which is responsible for the removal of inhaled pathogens and other hazardous substances from the respiratory system.In PCD,mucociliary clearance is impaired and as a result patients suffer from recurrent respiratory infections which lead to chronic destructive airway disease.1 Other common manifestations of the disease include sinus and ear infections,situs abnormalities and male infertility.展开更多
文摘Primary ciliary dyskinesia (PCD) is an inherited,genetically heterogeneous disorder characterized by the dysfunction of motile cilia that are found in a number of human tissues including the upper and lower airways epithelium (respiratory cilia).Respiratory cilia project from the apical membrane of epithelial cells that line up the airways and through their active and coordinated beating,drive the process of mucociliary clearance,which is responsible for the removal of inhaled pathogens and other hazardous substances from the respiratory system.In PCD,mucociliary clearance is impaired and as a result patients suffer from recurrent respiratory infections which lead to chronic destructive airway disease.1 Other common manifestations of the disease include sinus and ear infections,situs abnormalities and male infertility.
