Indoor Air Pollution and Outcomes in Cystic Fibrosis

项目总结 囊性纤维化是一种致命性常染色体隐性遗传病，全世界有近8万人患病。尽管 在治疗方面的进展，CF仍然是一种进行性肺部疾病，导致肺功能下降并频繁发生 肺部恶化。 室外空气污染水平较高，包括直径在10微米或以下的颗粒物(PM)(PMR10R) 2.5µm或更少(PMR2.5R)、二氧化氮(NOR2R)和臭氧被证明会导致更糟糕的CF 结果，如肺功能下降和肺加重。许多研究表明 室内空气污染水平较高会增加阻塞性肺部疾病的发病率，如哮喘和 慢性阻塞性肺疾病（慢阻肺）。据我们所知，这还没有在CF患者身上进行研究，这也是一种阻塞性肺疾病。 在这项建议中，我们旨在研究室内空气污染如何影响慢性萎缩性胃炎患者。我们假设 室内空气污染水平越高，心力衰竭的结局越差，例如呼吸增加 症状和肺功能下降。 为了探索我们的假设，我们将从美国CF双胞胎和兄弟姐妹收集的数据中进行子分析 这项研究包括2000多年来暴露于室内空气污染源和临床结果的数据 Cf 2000-13年期间的个人。接下来，我们将向我们当地的CF队列分发一份调查报告，以收集更详细的信息 家庭暴露数据，并随着时间的推移跟踪呼吸道症状和肺功能。最后，我们将进行一次 20例CF患者家庭内每日PMR2.5R和每周NOR2R和尼古丁R水平的初步研究 为期两周，按季节分开，并要求患者回答每日一次的呼吸道症状 问卷调查，完成日常活动日记，并进行日常家庭肺活量测定。然后我们将确定是否 PMR2.5兰特的日变化和NOR2R和尼古丁的周水平与呼吸的日变化有关 症状和肺功能。 这项建议中进行的研究具有公共卫生意义，并将提供关于 室内空气污染如何影响CF。这项研究的结果可能会影响整个社区，并将 作为未来多中心和干预性研究的催化剂，这将是我K23的基础 申请。

PROJECT SUMMARY Cystic Fibrosis (CF) is a lethal autosomal recessive disease affecting nearly 80,000 people worldwide. Despite advances in therapy, CF remains a progressive lung disease that leads to decline in lung function and frequent pulmonary exacerbations. Higher levels of outdoor air pollution, including particulate matter (PM) with a diameter of 10µm or less (PMR10R) and 2.5µm or less (PMR2.5R), nitrogen dioxide (NOR2R), and ozone, have been shown to lead to worse CF outcomes such as decline in lung function and increased pulmonary exacerbations. Many studies have shown that higher levels of indoor air pollution increase morbidity in obstructive lung diseases such as asthma and COPD. To our knowledge, this has not been studied in individuals with CF, also an obstructive lung disease. In this proposal we aim to study how indoor air pollution affects individuals with CF. We hypothesize that higher levels of indoor air pollution are associated with worse CF outcomes, such as increased respiratory symptoms and decline in lung function. To explore our hypothesis, we will conduct a sub-analysis from data collected in the US CF Twin and Sibling Study, which includes data on exposure to sources of indoor air pollution and clinical outcomes on over 2000 CF individuals from 2000-13. Next, we will distribute a survey to our local CF cohort to gather more detailed home exposure data and follow respiratory symptoms and lung function over time. Finally, we will conduct a pilot study to measure daily PMR2.5R and weekly NOR2R and nicotineR Rlevels inside the homes of 20 patients with CF for two one-week periods, separated by season, and ask patients to answer a daily respiratory symptoms questionnaire, complete daily activity diaries, and perform daily home spirometry. We will then determine if daily variations in PMR2.5 Rand weekly levels of NOR2R and nicotine are associated with daily variation in respiratory symptoms and lung function. The research carried out in this proposal is of public health significance and will provide new information on how indoor air pollution affects CF. The results of this study may impact the entire CF community and will serve as a catalyst for a future, multi-center and interventional study which will be the basis for my K23 application.