CORE--CLINICAL

这份SCOR赠款申请中的每个项目都试图评估 肺活体模型与体外模型的关系 肺的形态发生与修复与肺病的发病机制 新生儿和成年人。我们的SCOR调查人员积极合作 在过去的五年中进行了大量的临床研究，现在寻求 建立临床核心组织采集、存储临床 数据、管理和注重相关性的科学互动 SCOR项目开发的基础科学成果与临床之间的关系 疾病。 中心将获取、编目和存储活组织和尸检组织以及 新生儿肺部疾病的临床资料包括 先天性畸形囊腺瘤样畸形 肺泡发育不良/腺泡发育不全、隔疝、表面活性物质 蛋白B缺乏症，脱屑性间质性肺炎，新生儿 肺泡蛋白沉积症和支气管肺发育不良。成人活组织检查 取自ARDS、IPF结节病和肺组织的组织 肺泡蛋白沉着症将被储存和维护，以便与 分子标记是在SCOR项目中开发的。这些组织 将用于评估生长因子、细胞因子的潜在作用 系统，以及在个别项目中研究的转录因子 在发育、损伤和恢复的肺中。组织样本将被 为免疫化学、原位杂交和形态观察做准备。脱氧核糖核酸 样本将被获取和存储，用于潜在的遗传分析 可能与临床相关的靶基因异常 疾病。临床样本和数据将被用来检验这一假设。 控制肺生长的因素和机制 分化也在肺损伤后和期间重现。 正常和不正常的修复过程。

Each of the projects in this SCOR grant application seeks to assess relationships between the in vivo and in vitro models of lung morphogenesis and repair and the pathogenesis of lung disease in neonates and adults. Our SCOR investigators have collaborated actively in numerous clinical studies during the past five years and now seek to establish a clinical core for tissue collection, storage of clinical data, management and for scientific interactions focused to correlations between basic science findings developed with SCOR projects and clinical disease. The Core will obtain, catalog and store biopsy and autopsy tissue and clinical data from infants with neonatal lung diseases including congenital malformations cystadenomatoid malformation, congenital alveolar hypoplasia/acinar hypoplasia, diaphragmatic hernia, surfactant protein B deficiency, desquamating interstitial pneumonitis, neonatal alveolar proteinosis and bronchopulmonary dysplasia. Adult biopsy tissues obtained from patients with ARDS, IPF sarcoidosis and pulmonary alveolar proteinosis will be stored and maintained for correlation with the molecular markers developed within the SCOR projects. These tissues will be used to assess the potential roles of growth factor, cytokine systems, and transcription factors studied in the individual projects in the developing, injured and recovering lung. Tissue samples will be prepared for immunochemistry, in situ hybridization and morphology. DNA samples will be obtained and stored for genetic analysis of potential target gene abnormalities that may be associated with the clinical disease. Clinical samples and data will be used to test the hypothesis that the factors and mechanisms controlling lung growth and differentiation are also recapitulated following lung injury and during normal and abnormal repair processes.