SCOR-LUNG BIOLOGY AND DISEASES IN INFANTS AND CHILDREN

支气管肺发育不良(BPD)已成为 新生儿重症监护病房的发病率。BPD是临床后果 功能性肺损伤对新生儿肺损伤的影响 没有及时、有序地恢复肺的完整性 修复过程。考虑到对BPD的关注，此SCOR的目标与 在细胞生长和分化水平上的肺发育， 肺损伤的防御机制，以及修复过程中 为应对肺损伤而启动的。该SCOR还解决了各种机制 通过哪些临床干预措施可以改变两个肺 损伤或修复过程作为一种策略来减少发病率和 BPD的严重程度。最后，在预期快速增长的成功 肺移植的应用将不可避免地扩大到终末期婴儿 在BPD阶段，本SCOR将尝试回答与 肺表面活性物质系统对移植肺性能的影响 肺表面活性物质系统在失神经肺中的作用 在发生排斥反应时使用表面活性物质治疗。中的具体项目 这项建议的SCOR涉及1)表皮生长因子信号的研究 肺发育中的转导，2)细胞外基质的作用 胎鼠肺上皮细胞生长和分化中的生物合成， 3)肺表面活性物质相关蛋白及相关C型蛋白的作用 凝集素在肺发育、损伤和修复中的作用 维生素A对肺上皮细胞分化的调节作用 P450基因表达的调控及其在氧化剂产生中的作用 高氧肺，以及6)表面活性物质治疗的生理效应 在人类早产儿身上。支持这些项目的资源包括 由五个核心提供：新生儿临床研究核心，动物 实验室核心，生物医学工程和信息学核心， 组织病理学和分子生物学核心，以及管理核心。

Bronchopulmonary dysplasia (BPD) has become the greatest contributor to morbidity in newborn intensive care units. BPD is the clinical consequence of lung injury in the human newborn infant that occurs when functional integrity of the lung is not restored in a timely and orderly manner by the repair process. With a focus on BPD in mind, the goals of this SCOR relate to lung development at the level of cell growth and differentiation, to mechanisms of defense against lung injury, and to repair processes that are initiated in response to lung injury. This SCOR also addresses mechanisms by which clinical interventions can be employed to modify either lung injury or the repair process as a strategy to reduce the incidence and severity of BPD. Finally, in anticipation that the rapidly growing success of lung transplantation will inevitably be extended to the infant with end- stage BPD, this SCOR will attempt to answer questions related to the surfactant system in the transplanted lung, both in regard to performance of the surfactant system in the denervated lung and in regard to the role of surfactant treatment during episodes of rejection. Specific projects in this proposed SCOR involve studies of 1) epidermal growth factor signal transduction in the developing lung, 2) the role of extracellular matrix biosynthesis in fetal rat lung epithelial cell growth and differentiation, 3) the role of pulmonary surfactant associated proteins and related C-type lectins in the lung development, injury and repair, 4) the effects of vitamin A on the regulation of lung epithelial differentiation, 5) the regulation of P450 gene expression and its role in oxidant production by the hyperoxic lung, and 6) the physiologic effects of surfactant treatment in human premature infants. Resources to support these projects are provided by five cores: the Newborn clinical Research Core, the Animal Laboratory Core, the Biomedical Engineering and Informatics Core, the Histopathology and Molecular Biology Core, and the Administrative Core.