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利用 资源由NIH/NCRR资助的中心拨款提供。子项目和 调查员(PI)可能从NIH的另一个来源获得了主要资金， 并因此可以在其他清晰的条目中表示。列出的机构是 该中心不一定是调查人员的机构。 最近的研究表明，慢性萎缩性肺病的发病时间远远早于人们所知的时间，通常发生在婴儿期和出现呼吸道症状之前。到目前为止，由于缺乏敏感和可重复的肺部疾病测量方法，这一年龄段的研究一直受到阻碍。肺活量测定法要求自愿产生最大吸气力，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，在生命的头几年，测量被限制在潮气量(正常呼吸)范围内，在这个范围内，它们高度可变，重复性很差。 最近，一种新的技术被开发出来，用于测量镇静婴儿肺容量增加和肺容量分数所产生的用力呼气流量，首次产生了与年龄较大的受试者相媲美的结果。这些新技术有可能极大地提高对早期CF肺部疾病的了解，并作为临床试验的可重复性结果指标。虽然这些测试已经成功地应用于使用调查者制造的装置的单中心研究，但它们从未被用于多中心设置。这项研究的目的是验证柯林斯公司生产的婴儿肺实验室设备在未来的临床试验中的使用，并确定使用IPL设备进行婴儿肺功能测试对患有CF的婴儿是否安全和耐受性良好。

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.