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

起源于肺小管晚期至成熟肺泡化时期的肺部疾病 而介于生存能力极限和成年早期之间的肺功能峰值仍然是发病率的主要原因。 和死亡率。发现治疗这些疾病的新疗法的研究受到获得这些疾病的稀缺性的限制 在这一年龄段内对正常和患病的人肺的影响。拥有作为原始人类组织核心的 (HTC)为肺发育计划分子图谱(LUNgMAP)创建了 罗彻斯特大学医学中心肺部疾病调查(BRINDL) 提交此申请以响应RFA-HL-19-021，以继续担任LongMAP的HTC 第二阶段(LUNGMAP II)。BRINDL现在包含200多个全肺集合，其中50个在历史上是正常的 组织病理学，从妊娠中后期到成年，以及另外70例特定的 疾病。URMC-HTC将继续负责充分识别和管理组织来源 为了实现建立开放获取的、全面的、晚期分子图谱的总体计划目标 肺处于发育成熟阶段。作为HTC，URMC将收集、处理、存放并分发到 四个LongMAP研究中心(RCS)，最终是更大的研究社区，人类肺 以支持时间和空间的形式表示妊娠晚期到成年期早期的样本 发育过程中功能或解剖学定义的细胞类型的分子图谱特征 阿龙。根据RFA，市建局-HTC将支持将LongMAP扩展到最多25岁，并 包括新生儿和儿童罕见肺部疾病以及正常的肺部发育。因为 以标准化的方式和AS恢复高质量的儿童肺的关注和复杂性 尽可能少的缺血诱导的人工制品，以及满足所有法律和道德标准的需要，我们 继续选择主要通过器官共享联合网络的要素开展工作 联邦签约器官采购和移植网络(OPTN)和两个非营利性组织 被转介到适合研究的不可移植捐赠器官的组织，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和家庭合作得很好，这些家庭在 怀孕或疾病预计是致命的。此外，我们还与儿科肺科中心有联系 在全国各地寻找罕见肺部疾病样本的潜在来源。我们的目标是提供一个 额外的250个肺集捐赠，或至少进行活组织检查，以纳入LUNGMAP BRINDL 生物储存库。我们的URMC-HTC将与西雅图儿童研究所合作，准备 捐赠器官和组织，以满足LUNGMAP第二期RCS的需要。

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.