MUCUS CLEARANCE DURING ACUTE EXACERBATIONS OF CYSTIC FIBROSIS

这个子项目是许多研究子项目中利用 资源由NIH/NCRR资助的中心拨款提供。子项目和 调查员(PI)可能从NIH的另一个来源获得了主要资金， 并因此可以在其他清晰的条目中表示。列出的机构是 该中心不一定是调查人员的机构。 囊性纤维化(CF)患者的病情呈间歇性恶化，称为恶化，通常需要住院或在家中进行强化治疗。随着慢性肺病的进展，这些恶化可能每年重复发生几次，从而严重影响 患者？S的生活质量和提高医疗资源利用率。然而，我们对导致病情恶化的生物过程的了解是有限的。我们假设，包括病毒感染在内的各种触发因素减缓了呼吸道粘液的清除，从而导致细菌感染恶化和呼吸道阻塞进展。我们建议通过测量患者身体状况良好时的粘液清除(通过跟踪吸入后标记颗粒的消失)来检验这一假设，以及 病情发展后再次恶化。我们还将使用新的、敏感的方法来确定这些患者生病时的痰中是否存在呼吸道病毒。最后，我们将测量痰中的物质，如果在病情加重时改变，可能会影响粘液清除率。在这项研究中收集的数据可能表明通过治疗保持/恢复粘液清除的重要性，以避免病情恶化，同时也识别可能针对新疗法的触发因素。

This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. Patients with cystic fibrosis (CF) experience episodic deteriorations of their condition, termed ?exacerbations?, which commonly require hospitalization or intensive treatments at home. As CF lung disease progresses, these exacerbations may reoccur several times a year, thus heavily impacting a patient?s quality of life and increasing healthcare resource utilization. Our understanding of the biological processes that lead to exacerbations is limited, however. We hypothesize that various triggers, including viral infection, slow the clearance of mucus from airways, thus causing bacterial infection to worsen and airway blockage to progress. We propose to test this hypothesis by measuring mucus clearance (by tracking the disappearance of labeled particles after they are inhaled) in patients while they are well, and again after the development of an exacerbation. We will also use new, sensitive methods to determine if respiratory viruses are present in the sputum of these patients while they are ill. Finally, we will measure substances in sputum that, if altered in an exacerbation, could influence mucus clearance rates. The data collected in this study may point toward the importance of maintaining/restoring mucus clearance therapeutically in order to avoid exacerbations while also identifying triggers that may be targeting with new therapies.