This subproject is one of many research subprojects utilizing theresources provided by a Center grant funded by NIH/NCRR. The subproject andinvestigator (PI) may have received primary funding from another NIH source,and thus could be represented in other CRISP entries. The institution listed isfor 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.
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