LUNG DISEASE IN CHILDREN WITH MILD CF LUNG DISEASE

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。 这项为期两年的前瞻性自然病史研究旨在研究最初患有轻度肺部疾病的CF儿童的肺活量控制的容量CT成像和定量CT空气滞留和支气管气道测量的变化，以及CFCT评分和传统肺功能测量以及临床评分的变化。此外，在这两年的自然病史研究中，对于能够有效排痰并提供痰标本的10-18岁轻度CF儿童亚组，还将评估痰IL-8和痰弹性蛋白酶，以评估呼吸道炎症对CT测量和其他临床参数的影响。 预计在两年的研究期间，肺活量控制的肺CT成像和定量CT测量将发现患有轻度肺部疾病的CF儿童肺部疾病的初始和进展性变化。这些肺部疾病的最初表现是局部空气滞留和支气管壁增厚，随后是支气管扩张和更大的呼吸道粘液堵塞。与传统的基于CT扫描视觉评估的CFCT评分相比，这些变化应该为未来的多中心药物干预试验提供更可行的结果衡量标准。

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. This prospective, two year natural history study is designed to study changes in spirometry-controlled volumetric lung CT imaging and quantitative CT measurements of air trapping and bronchial airway measurements together with CF CT scoring and traditional pulmonary function measurements, and clinical scores in CF children with initially mild lung disease. In addition, in a subgroup of mild CF children ages 10-18 years who can effectively expectorate and provide sputum specimens, sputum IL-8 and sputum elastase will also be evaluated to assess the effect of airway inflammation on CT measurements and other clinical parameters during this two year natural history study. It is predicted that the spirometry-controlled lung CT imaging & quantitative CT measurements will detect initial and progressive changes in lung disease in CF children with mild lung disease over the two year study period. These initial manifestations of lung disease will be regional air trapping and increased bronchial wall thickness, followed by bronchiectasis and larger airway mucus plugging. These changes should provide more viable outcome measures for future multi-center drug intervention trials compared to traditional CF CT scoring which is based on visual assessment of the CT scans.