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Muco-Obstructive Lung Diseases

Richard C. Boucher ... 呼吸系统疾病 • 2019.05.16
• 哪种抗生素最适合与囊性纤维化无关的支气管扩张发作 • VX-659–tezacaftor–ivacaftor三联疗法用于携带1或2个Phe508del等位基因的囊性纤维化患者 • VX-445–tezacaftor–ivacaftor三联疗法用于携带1或2个Phe508del等位基因的囊性纤维化患者 • COPD患者的三联疗法 • β受体阻滞剂可能改善慢性阻塞性肺疾病患者的结局


气道黏膜阻塞的发病机制如图1A所示。在健康人体内,充分水合的黏液层被迅速地从远端气道运输至气管(速度约为50 μm/s)。在黏液阻塞性疾病中,上皮在离子-液体运输、黏蛋白分泌方面有缺陷,或者有这些缺陷的组合,因而导致黏液超浓缩(脱水)、黏液运输失败以及黏液黏附于气道表面。咳嗽时,气管内积聚的黏液以痰的形式咳出11。小气道内的黏液无法通过咳嗽清除,因而积聚,形成气流阻塞、感染和炎症病灶11


Richard C. Boucher, M.D.
From the Marsico Lung Institute, University of North Carolina at Chapel Hill, Chapel Hill. Address reprint requests to Dr. Boucher at the Marsico Lung Institute, University of North Carolina at Chapel Hill, 7008 Marsico Hall, Chapel Hill, NC 27599, or at richard_boucher@med.unc.edu.



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