EVALUATION OF PULMONARY FUNCTION TESTS FOR OUTCOME MEASURES IN INFANT WITH CF

这个子项目是许多研究子项目中的一个 由NIH/NCRR资助的中心赠款提供的资源。子项目及 研究者（PI）可能从另一个NIH来源获得了主要资金， 因此可以在其他CRISP条目中表示。所列机构为 研究中心，而研究中心不一定是研究者所在的机构。 最近的研究表明，CF肺病的发病时间比以前所了解的要早得多，通常发生在婴儿期和呼吸道症状发作之前。迄今为止，由于缺乏敏感和可重复的肺部疾病指标，这一年龄范围的研究受到阻碍。肺量测定法需要自主产生最大吸气努力，然后是快速和最大用力呼气。 由于婴儿不能进行这样的动作，在生命的最初几年的测量已被限制在潮气量（正常呼吸）范围内，在那里他们是高度可变的，重现性差。 最近，开发了一种新技术来测量镇静婴儿肺容量增加和肺容量分数的用力呼气流量，首次产生了与老年受试者获得的结果相当的结果。 这些新技术有可能大大提高对早期CF肺病的理解，并作为临床试验的可重复结果指标。 尽管这些试验已成功用于使用透析器制造器械的单中心研究，但从未用于多中心环境。 本研究旨在确认由柯林斯公司生产的婴儿肺实验室设备，用于未来的临床试验，并确定使用IPL器械进行婴儿肺功能测试是否安全，CF婴儿是否耐受良好。

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. To date, research in this age range has been hindered by the lack of sensitive and reproducible measures of lung disease. 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. Recently, a new technique has been developed for measuring forced expiratory flows from raised lung volumes and fractional lung volumes in sedated infants, for the first time producing results comparable to those obtained by older subjects. These new techniques have the potential to dramatically improve the understanding of early CF lung disease, and to serve as reproducible outcome measures for clinical trials. Although these tests have been successfully employed in single-center studies utilizing investigator-fabricated devices, they have never been employed in a multi-center setting. The purpose of this study is to validate the Infant Pulmonary Lab device, manufactured by Collins, Inc., for use in future clinical trials and to determine whether infant pulmonary function testing with the IPL device is safe and well tolerated in infants with CF.