Cystic Fibrosis: The Mechanisms of Pathogenesis of an Inherited Lung Disorder.
Cystic Fibrosis: The Mechanisms of Pathogenesis of an Inherited Lung Disorder.
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DOI：
10.1016/j.ddmec.2007.09.001
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发表时间：
2007-01-01
期刊：
影响因子：
--
通讯作者：
Boucher, Richard C
中科院分区：
文献类型：
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作者：
Clunes, Mark T;Boucher, Richard C
囊性纤维化患者表现出与先天呼吸道防御机制失败相一致的肺部疾病。离子转运异常与疾病的发生和发展之间的联系尚不完全清楚，但呼吸道粘液脱水似乎在CF肺部疾病的发生中起着至关重要的作用。目前正在开发新的治疗方法，针对CF中的离子运输缺陷，目的是恢复呼吸道表面的水化。
Cystic fibrosis patients exhibit lung disease consistent with a failure of innate airway defense mechanisms. The link between abnormal ion transport and disease initiation and progression is not fully understood, but airway mucus dehydration seems paramount in the initiation of CF lung disease. New therapies are currently in development that target the ion transport defects in CF with the intention of rehydrating airway surfaces.