POLYCYSTIC KIDNEY PROGRAM PROJECT

没有任何手段可以防止、逮捕或扭转无情和 进行性肾功能衰竭是成人多囊肾的临床特征 肾脏疾病(APKD)，一种对患者有重大贡献的实体 联邦资助的终末期肾脏的数量和规划成本 疾病计划。已经设计了一个计划项目，以确定 从而达到控制APKD肾功能衰竭的目的。八 各机构将参与开展十个项目。他们会 使用生化、显微镜、微生物学、内科和外科 肾脏结构和功能研究中的技术 CFWw小鼠(遗传性肾囊性疾病模型)和CD大鼠 致囊性化学物质(获得性肾囊性疾病的模型)，以及来自 受影响的人类，其肾脏在器官捐赠时可用 或者验尸。这些项目将解决与肾脏有关的六个问题 囊性疾病，从肾脏易感到终末期： 易患肾脏中的肾单位正常吗？为什么会形成包囊？做 囊泡功能正常吗？为什么会长出囊肿？囊性增长会导致肾功能衰竭吗？ 终末期肾功能能得到改善吗？的具体主题 检查包括肾脏的成分、代谢和顺应性。 肾小管基底膜；肾小管上皮细胞生长； 囊性肾单位的通畅性；囊性发育过程中宿主-微生物的相互作用； 抗菌药物对包囊的穿透作用；以及肿胀的影响 囊性变小对整体肾功能的影响。该项目将是 由三个核心支持：管理鼠群和器官 采购部。该计划的结果将确定哪些 如果APKD的肾功能不全，必须建立以下目标 成功控制：纠正导致酶缺陷的 有缺陷的基底膜产生，上皮细胞的控制 部分阻碍肾单位的增殖，控制肾单位的生长 压迫和破坏正常邻近肾脏的囊肿，或控制 导致包囊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.