MUCUS CLEARANCE DURING ACUTE EXACERBATIONS OF CYSTIC FIBROSIS

这个子项目是许多利用 由NIH/NCRR资助的中心赠款提供的资源。子项目和 研究者（PI）可能从另一个NIH来源获得了主要资金， 因此可以在其他CRISP条目中表示。所列机构为 研究中心，而研究中心不一定是研究者所在的机构。 囊性纤维化（CF）患者的经验，他们的病情发作性恶化，称为？病情加重？这些疾病通常需要住院治疗或在家中进行强化治疗。 随着CF肺病的进展，这些加重可能每年复发数次，从而严重影响患者的健康。 病人？的生活质量和提高医疗资源的利用率。然而，我们对导致病情加重的生物学过程的理解是有限的。 我们假设，包括病毒感染在内的各种触发因素减缓了气道粘液的清除，从而导致细菌感染恶化和气道阻塞进展。 我们建议通过测量患者健康时的粘液清除率（通过追踪吸入标记颗粒后的消失情况）来验证这一假设， 在恶化发展后再次出现。 我们还将使用新的、敏感的方法来确定这些患者患病时的痰液中是否存在呼吸道病毒。 最后，我们将测量痰液中的物质，如果在急性加重中改变，可能会影响粘液清除率。 本研究中收集的数据可能表明在治疗上维持/恢复粘液清除的重要性，以避免病情加重，同时还确定了新疗法可能靶向的触发因素。

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.