POLYCYSTIC KIDNEY PROGRAM PROJECT

没有任何手段可以阻止、逮捕或扭转这场无情的、 进行性肾衰竭，临床上以成人多囊性病变为特征 肾脏疾病（APKD）是一种显著影响患者 联邦政府资助的终末期肾移植的数量和方案费用 疾病计划。 已设计了一个方案项目， 从而达到控制APKD肾功能衰竭的目的。 八 各院校将参与十个项目的实施。 他们将 使用生物化学、显微镜、微生物、医学和外科学 肾的结构和功能的研究技术， CFWw小鼠（遗传性肾囊肿病模型）和CD大鼠喂食 致囊化学物质（获得性肾囊肿模型），以及来自 受影响的人，其肾脏在器官捐赠时可用 或者尸检 这些项目将解决有关肾脏的六个问题， 囊性疾病，从易感肾脏到终末期肾脏的病变： 在易感肾中，肾单位正常吗？ 为什么会形成囊肿？ 做 囊肿功能？ 为什么囊肿会生长？ 肾囊肿会导致肾功能衰竭吗？ 终末期肾功能可以改善吗？ 的具体议题 研究包括肾脏的组成、代谢和顺应性。 肾小管基底膜;肾小管上皮生长;肾小管上皮细胞。 囊性肾单位的开放性;囊肿发育中宿主-微生物的相互作用; 抗微生物剂对囊肿的渗透;以及扩大的影响 以及囊肿大小减小对整体肾功能的影响。 该项目将 由三个核心支持：给药小鼠集落和器官 采购等 该计划的结果将确定哪些 如果要治疗APKD的肾衰竭，必须建立以下目标 成功控制：纠正导致 基底膜产生缺陷，上皮细胞的控制 部分阻碍肾单位的增殖， 囊肿压迫并破坏正常的邻近肾脏，或控制 导致囊肿形成或扩大的微生物。

No means exist to prevent, arrest, or reverse the relentless and progressive renal failure that clincally characterizes adult polycystic kidney disease (APKD), an entity that contributes significantly to patient numbers and programmatic costs in the federally sponsored endstage renal disease program. A Program Project has been designed to identify avenues by which the control of renal failure in APKD might be achieved. Eight institutions will be involved in the conduct of ten projects. They will employ biochemical, microscopical, microbiological, medical, and surgical techniques in the study of the structure and function of kidneys from the CFWw mouse (a model of heritable renal cystic disease) and the CD rat fed cystogenic chemicals (a model of acquired renal cystic disease), and from affected humans, whose kidneys become available at times of organ donation or autopsy. The projects will address six questions concerning renal cystic disease, spanning the lesion from predisposed to endstage kidney: In the predisposed kidney are nephrons normal? Why do cysts form? Do cysts function? why do cysts grow? Does cyst growth cause renal failure? Can function in the endstage kidney be improved? Specific topics of investigation include the composition, metabolism, and compliance of renal tubular basement membrane; the growth of renal tubular epithelium; the patency of cystic nephrons; host-microbe interaction in cyst development; the penetration of cysts by antimicrobials; and the impacts of enlargement and reduction in cyst size on overall renal function. The Project will be supported by three cores: Administration Mouse Colony, and Organ Procurement. The results of this program will identify which of the following goals must be established if the renal failure of APKD is to be successfully controlled: correction of the enzymatic defect that leads to faulty basement membrane production, control of the epithelial proliferation that partially obstructs nephrons, control of the growth of cysts that compress and destroy normal adjacent kidney, or control of microbes that cause cysts to form or expand.