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. The conduct of clinical trials in infants with cystic fibrosis (CF) has been hindered by the lack of sensitive and reproducible outcome measures. Recently, new techniques have been developed for pulmonary function testing (PFT) in infants. These techniques have the potential to dramatically improve the understanding of the early natural history and pathophysiology of CF lung disease, and serve as reproducible outcome measures for clinical trials. In the current study, changes in pulmonary function will be characterized in a cohort of infants with CF, utilizing both new and conventional methods of infant PFTs. Ten to 15 subjects will be enrolled at each of 9 participating sites. Each subject will undergo testing at enrollment and 1, 6, and 12 months after enrollment. The goal of the proposed study is to test the hypothesis that measurements produced by the new PFT techniques will serve as useful outcome measures for clinical trials in infants with CF.
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