遗传性支气管扩张的发病机制及治疗研究进展

摘要

支气管扩张(bronchiectasis)是指由于感染、理化、免疫或遗传等原因引起终末支气管不可逆的扩张和管壁的增厚,包括支气管壁肌肉和弹力支撑组织的破坏.而遗传性支气管扩张特别是原发纤毛不动综合征(PCD)的发病机制及治疗作为近年来支气管扩张的研究热点,表现备受关注.本文通过探讨支气管扩张的分类及PCD的发病机制和治疗,对遗传性支气管扩张的发病机制 及治疗研究进展进行描述.%Bronchiectasis refers to irreversible enlargement and thickening of the wall of the terminal bronchi,including destruction of the bronchial wall muscles and elastic supporting tissues.It can be caused by infection,physiochemical,immunological or genetic and so on.The pathogenesis and treatment of hereditary bronchiectasis,especially primary ciliary dyskinesia (PCD),has attracted much attention in recent years.In this paper,the classification of bronchiectasis and the pathogenesis and treatment of PCD are discussed.The pathogenesis and treatment of hereditary bronchiectasis are described.