Kansas Polycystic Kidney Imaging Program (CRISPII)

常染色体显性遗传性多囊肾病(ADPKD)是致残性疾病的主要原因，也是 世界第四大终末期肾功能衰竭原因，影响50多万美国公民和 全球范围内还有数百万人。阿拉巴马大学、埃默里大学、堪萨斯大学的研究人员 梅奥诊所和华盛顿大学圣路易斯分校于2000年共同创建了 多囊肾病的放射学研究这次调查的主要目的是 目的：(1)开发和测试监测肾脏变化的成像技术的准确性和重复性 (2)建立和维护一个统一、准确的囊性病变数据库。 收集到的信息，以及(3)保存和提供这些数据，以便于规划和 在不久的将来实施临床适当的干预措施。CRISPII的目标是扩展 对Crispi的观察是为了：1)明确地将肾/囊肿率与 扩大以及定性和定量的终点；2)提供疾病进展的标志 (肾脏体积)足够灵敏和准确，可作为临床试验的主要结果标志 旨在阻止疾病的进展；3)开发和测试其他疾病进展的生物标志物。 具体目标是：目标1：扩大Crispi的初步观察范围，以确定 定量(肾脏体积和肝肾囊肿体积)或定性(囊肿分布和 结构参数预测肾功能不全。目标2：扩大初步观察范围 Crispi确定年龄和性别调整后的MR测量肾血流量的程度 技术可以预测肾脏的生长速度，而肾脏的血流量和体积则可以预测肾脏的生长速度。 ADPKD患者功能下降。目标3：详尽分析活体数据库和存储的生物样本 派生自Crispi和CRISPII扩展，以开发和测试量化和监控的新指标 疾病的发展。扩大单核细胞趋化蛋白-1(MCP-1)排泄分析以确定 尿单核细胞趋化蛋白-1的绝对排泄量和排泄率的变化是否与特定的 病态事件(例如肉眼血尿、新发高血压、尿路感染、肾结石、非特异性 肾痛和肾功能恶化(肾小球滤过率下降或蛋白尿增加)。

Autosomal dominant polycystic kidney disease (ADPKD) is a major cause of disabling morbidity and is the fourth leading cause of end-stage renal failure in the world, affecting more than 500,000 U.S. citizens and millions more worldwide. Researchers at the University of Alabama, Emory University, University of Kansas, Mayo Clinic and Washington University St. Louis joined together in 2000 to create the Consortium for Radiologic Studies of Polycystic Kidney Disease (CRISPI). The primary objectives of this investigation were to: (1) to develop and test the accuracy and reproducibility of imaging techniques to monitor changes in renal cyst size and parenchymal involvement, (2) to establish and maintain a database of uniformly and accurately collected information, and (3) to maintain and make available such data to facilitate the planning and implementation of clinically appropriate interventions in the near future. The goals of CRISPII are to extend the observations of CRISPI in order to: 1) draw unequivocal linkage between the rate of kidney/cyst enlargement and qualitative and quantitative end-points; 2) to provide a marker of disease progression (kidney volume) sensitive and accurate enough to be used as a primary outcome marker in clinical trials aiming to forestall disease progression; 3) to develop and test other biomarkers of disease progression. The specific aims are: Aim 1: Extend the preliminary observations of CRISPI to ascertain the extent to which quantitative (kidney volume and hepatic and kidney cyst volume) or qualitative (cyst distribution and character) structural parameters predict renal insufficiency. Aim 2: Extend the preliminary observations of CRISPI to ascertain the extent to which age and sex-adjusted measurements of renal blood flow by MR technology predict the rate of renal growth; and, renal blood flow and kidney volume predict the rate of renal function decline in ADPKD. Aim 3: Exhaustively analyze the living database and stored biologic samples derived from CRISPI and the CRISPII extension to develop and test new metrics to quantify and monitor disease progression. Extend the analysis of monocyte chemotactic protein-1 (MCP-1) excretion to determine if absolute levels of urinary MCP-1 excretion and changes in the rates of excretion bear relation to specific morbid events (e.g. gross hematuria, new onset hypertension urinary tract infection, renal stone, nonspecific renal pain, and worsening renal function (declining GFR or increased albuminuria).