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.Lung disease in cystic fibrosis (CF) begins early in life, and therefore the detection of early lung changes and intervention to prevent chronic airway infection and inflammation are critical. Clinical investigations in young children with CF have been limited by the lack of appropriate outcome measures. Recently investigators have demonstrated that reproducible spirometry can be obtained from preschool age children using modified criteria. Normal values have been obtained cross-sectionally, however multicenter data in normal healthy controls is not available. Only one single center study has evaluated spirometry in children with CF. This proposal seeks to characterize lung function in children with CF ages 3-6 years using a standardized equipment and testing procedures for spirometry, forced oscillometry (FO) and inductance plethysomgraphy (IP), rigorous training of study personnel, ongoing quality control, and review of all studies by an expert panel. A subset of CF children previously enrolled in a multicenter study of infant pulmonary function tests (IPFTs) will enter the current study allowing for the evaluation of lung functions longitudinally from infancy to age 6. Normal healthy controls will also be enrolled at selected sites. The goals of this study are to obtain the information necessary for the planning of future multicenter clinical trials in preschool CF patients in which lung function is the primary outcome measure and to better characterize the early natural history of CF lung disease in a cohort of children in which spirometry is measured continuously from infancy through age 6. We hypothesize that the spirometry FO and IP will serve as feasible, sensitive and reproducible outcome measures of early lung disease for future multicenter clinical trials in preschool CF patients. Development of clinical outcome measures in preschool children with CF will allow clinical trials to be performed and therapeutic agents to be studies in this young population.
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