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. Recent studies have demonstrated that CF lung disease begins far earlier than previously understood, frequently in infancy and prior to the onset of respiratory symptoms. In order for very young children to benefit from new CF therapies, including gene replacement therapy, clinical trials need to be conducted in the youngest age range. To date, reseach in this age range has been hindered by the lack of sensitive and reproducible measures of lung disease. In older subjects spirometry has stood for decades as the gold standard for measuring respiratory status, both for investigational and clinical purposes. Spirometry requires the voluntary generation of a maximal inspiratory effort followed by a rapid and maximal forced expiration. Because infants cannot perform such a maneuver, measurements in the first several years of life have been restricted to those obtained in the tidal volume (normal breathing) range, where they are highly variable and poorly reproducible.
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