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首页> 外文期刊>American Journal of Physiology >Acid in the airways. Focus on 'Hyperacidity of secreted fluid from submucosal glands in early cystic fibrosis'
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Acid in the airways. Focus on 'Hyperacidity of secreted fluid from submucosal glands in early cystic fibrosis'

机译：气道中的酸。专注于“早期囊性纤维化中黏膜下腺分泌液的高酸度”

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摘要

cystic fibrosis (CF) is caused by defects in an anion channel called CF transmembrane conductance regulator (CFTR) that is found primarily in the apical membranes of epithelial cells. Most CF symptoms, such as high salt in sweat and obstruction of the pancreatic ducts, intestine, or vas defer ens occur because of defective electrolyte transport. In most organs, the link between pathophysiology and loss of CFTR-mediated anion conductance is relatively easy to explain as obstruction following the loss of CFTR-mediated fluid secretion. This is not the situation in the airways, where the major symptom is chronic airway infection. These infections provoke massive neutro-philic inflammation that relentlessly destroys the lungs. Because chronic lung infection is by far the major cause of death in CF patients, it is essential to understand precisely how the loss of CFTR compromises the innate defenses of the airways
机译：囊性纤维化（CF）是由称为CF跨膜电导调节剂（CFTR）的阴离子通道中的缺陷引起的，该缺陷主要存在于上皮细胞的顶膜中。大多数CF症状，例如汗液中的高盐分和胰管，肠子或输精管阻塞，都是由于电解质运输不良所致。在大多数器官中，病理生理与CFTR介导的阴离子电导率丧失之间的联系相对容易解释为CFTR介导的液体分泌丧失后的阻塞。气道情况并非如此，主要症状是慢性气道感染。这些感染引起大量嗜中性发炎，无情地破坏了肺部。由于到目前为止，慢性肺部感染是CF患者的主要死亡原因，因此必须准确了解CFTR的丧失如何影响气道的固有防御系统，这一点至关重要

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