LUNG FUNCTION MEASURES IN PRESCHOOL CHILDREN WITH CYSTIC FIBROSIS

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。子项目和 研究者（PI）可能从另一个NIH来源获得了主要资金， 因此可在其他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。 随着认识到囊性纤维化（CF）肺病在生命早期开始，通常在症状发作之前，越来越多的人认识到需要在年轻CF患者中进行多中心临床试验。不幸的是，在这一关键的年轻人群的临床研究受到了阻碍，缺乏适当的结果的措施，特别是肺功能的措施。拟定研究是一项前瞻性、纵向、描述性、多中心研究，在学龄前CF儿童和健康对照受试者中对三种有前途的肺功能指标（肺功能测定、电感体积描记（IP）和强迫肺功能测定（FO））进行研究，目的是评估其作为学龄前CF患者临床试验结局指标的效用。本研究将使用标准化设备和检测程序，严格培训研究人员，并由专家小组对所有原始数据进行持续质量控制审查。 为了获得并记录学龄前肺功能检测所需的专业知识，在学龄前肺功能研究中招募任何受试者之前，每家研究中心将在单独的“认证方案”中招募未患有CF的学龄前受试者。

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. With the awareness that cystic fibrosis (CF) lung disease begins early in life and often prior to the onset of symptoms, there is increasing recognition of the need for multicenter clinical trials in young CF patients. Unfortunately, clinical investigations in this critical young population have been hindered by the lack of appropriate outcome measures, particularly measures of lung function. The proposed study is a prospective, longitudinal, descriptive, multi-center investigation of three promising lung function measures (spirometry, inductance plethymography (IP) and forced oscillometry (FO)) in preschool aged children with CF and in healthy control subjects, with the objective of assessing their utility as outcome measures for clinical trials in preschool CF patients. The study will utilize standardized equipment and testing procedures, rigorous training of study personnel, and ongoing quality control review of all primary data by an expert panel. In order to gain and document the required expertise in preschool lung function testing, each site will enroll preschool subjects without CF in a separate ¿certification protocol¿ prior to enrolling any subjects in the preschool lung function study.