Biorepository for INvestigation of Diseases of the Lung (BRINDL) - Phase II

起源于晚期小管期至成熟肺泡化之间的时期的肺部疾病 在生存极限和成年早期之间的肺功能峰值仍然是发病的主要原因 and mortality.研究发现新的治疗这些疾病的方法是有限的罕见的访问 正常和患病的人类肺部的差异作为原始的人体组织核心 (HTC)肺发育计划的分子图谱（LungMAP），创建了BioRepository 肺部疾病调查（BRINDL），罗切斯特大学医学中心（URMC） 提交本申请以响应RFA-HL-19-021，继续担任LungMAP的HTC 第2阶段（LungMAP II）。BRINDL现在包含200多个人类全肺组，50个正常的历史， 组织病理学检查，涵盖妊娠中晚期至成年年龄，以及另外70+例具有特定 疾病URMC-HTC将继续负责充分识别和管理组织来源 为了实现总体计划目标，建立一个开放获取的，全面的，晚期分子图谱- 肺发育成熟期。作为HTC，URMC将收集、处理、存款并分发给 四个LungMAP研究中心（RC），最终是更大的研究社区，人类肺 代表妊娠晚期到成年早期的样本，其形式将支持时间和空间 发育中功能或解剖学上定义的细胞类型的分子谱的表征 肺。根据RFA，URMC-HTC将支持将LungMAP扩展至25岁， 包括新生儿和儿科罕见肺部疾病以及正常肺部发育。因为 以标准化的方式恢复高质量的儿科肺的关注和复杂性， 尽可能少的缺血引起的伪影，以及满足所有法律的和道德标准的需要，我们 继续选择主要通过器官共享联合网络的组成部分开展工作， 联邦政府签约的器官采购和移植网络（OPTN），以及两个非营利组织 组织向谁是转介非移植捐赠器官适合研究，国家 疾病研究交流中心（NDRI）和国际医学促进研究所 （IIAM）。NDRI和IIAM与OPTN以及在一次紧急情况下直接与他们联系的家庭合作良好 怀孕或疾病可能致命。此外，我们还与儿科肺中心建立了联系， 以确定罕见肺病样本的潜在来源。我们的目标是提供一个 额外250例肺集捐献，或至少活检，以纳入LungMAP BRINDL 生物储藏室我们的URMC-HTC与西雅图儿童研究所合作， 捐赠器官和组织，以满足LungMAP II期RC的需求。

Lung disease originating in the period between the late canalicular stage to mature alveolarization and peak lung function, between limits of viability and early adulthood, remains a major cause of morbidity and mortality. Research to discover novel treatments for these disorders is limited by the rarity of access to normal and diseased human lung across this age span. Having, as the original Human Tissue Core (HTC) for the Molecular Atlas of Lung Development Program (LungMAP), created the BioRepository for INvestigation of Diseases of the Lung (BRINDL), the University of Rochester Medical Center (URMC) submits this application in response to RFA-HL-19-021 to continue to serve as the HTC for LungMAP Phase 2 (LungMAP II). BRINDL now contains over 200 human full lung sets, 50 normal by history and histopathology, spanning mid-late gestation to adult age, as well as an additional 70+ with specified disease. It will continue to be URMC-HTC responsibility to sufficiently identify and manage tissue sources to meet the overall Program goal to build an open-access, comprehensive, molecular atlas of the late- stage developing and maturing lung. As the HTC, URMC will collect, process, deposit, and distribute to the four LungMAP Research Centers (RCs), and ultimately the greater research community, human lung samples representing late gestation through early adulthood in forms that will support temporal and spatial characterization of molecular profiles of functionally or anatomically defined cell types in the developing lung. Per the RFA, the URMC-HTC will support the expansion of LungMAP to up to 25 years of age and to include neonatal and pediatric rare lung disease as well as normal lung development. Because of the concern and complexity in recovering high-quality pediatric lungs in a standardized manner and with as little ischemia-induced artifact as possible, and the need to meet all legal and ethical standards, we continue to choose to work primarily through elements of the United Network of Organ Sharing, the federally contracted Organ Procurement and Transplantation Network (OPTN), and two non-profit organizations to whom are referred non-transplantable donated organs suitable for research, The National Disease Research Interchange (NDRI) and the International Institute for the Advancement of Medicine (IIAM). NDRI and IIAM work well with the OPTN and with families who approach them directly during a pregnancy or illness expected to be lethal. In addition, we have contacts with Pediatric Pulmonary Centers around the country to identify potential sources for rare lung disease samples. Our goal is to provide an additional 250 lung set donations, or at minimum biopsies, for inclusion in the LungMAP BRINDL biorepository. Our URMC-HTC, in collaboration with Seattle Children’s Research Institute, will prepare the donated organs and tissues to meet the needs of the LungMAP Phase II RCs.