CORE--CLINICAL

SCOR赠款申请中的每个项目都旨在评估 肺的体内外模型之间的关系 形态发生和修复以及肺疾病的发病机制 新生儿和成人。 我们的SCOR调查人员积极合作， 在过去的五年里，在许多临床研究中， 建立一个临床中心，用于组织收集、临床储存 数据、管理和科学互动，重点关注相关性 SCOR项目开发的基础科学发现与临床 疾病 核心将获取、分类和存储活检和尸检组织， 新生儿肺部疾病婴儿的临床数据，包括 先天性畸形 肺泡发育不全/腺泡发育不全，疝，表面活性剂 蛋白B缺乏，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。 成人活检 从患有ARDS、IPF结节病和肺结核的患者获得的组织 肺泡蛋白沉积症将被储存和维持， SCOR项目中开发的分子标记。这些组织 将用于评估生长因子、细胞因子 系统和转录因子的研究在个别项目 在发育、受伤和恢复中的肺部。组织样本将 制备用于免疫化学、原位杂交和形态学。DNA 将获得并储存样本，用于潜在的遗传分析。 靶基因异常可能与临床 疾病临床样本和数据将用于检验假设 控制肺生长的因素和机制， 分化也概括了肺损伤后， 正常和非正常维修过程。

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.