Kansas Polycystic Kidney Imaging Program

描述(申请人提供)：常染色体显性遗传性多囊肾病(ADPKD)是世界上ESRD的主要致病原因和第四大致病原因，影响着50多万美国公民。阿拉巴马大学、埃默里大学、堪萨斯大学、梅奥诊所和华盛顿大学的研究人员于2000年联合成立了多囊肾病放射学研究联盟(Crispi)，并于2006年将匹兹堡大学取代华盛顿大学用于CRISP II。Crispi和II的主要目标是：建立基于磁共振的准确、可靠和可重复性的肾脏总体积(TKV)、肝囊肿体积(LCV)、肾血流(RBF)以及囊肿生长和扩张模式的测量。基于对200名CRISE L/11参与者7.3年的纵向随访，我们现在可以：1)建立TKV与定性(患者报告的结果)和定量(肾功能不全)终点之间的明确关系；以及2)为干预性研究识别与TKV和LCV相关的潜在可改变的危险因素。在临床试验中，TKV最终可能被用作疾病进展的替代标记物。Crispin的目标扩展了CRlSPI/11的观测范围。CRISP III的首要目标是开发和增强最好地预测ADPKD肾功能不全的预测模型。具体地说，目标1：扩展TKV和LCV的连续量化，以开发和测试预测发生肾功能不全风险的新模型。目的2：确定年龄和性别调整的RBF测量预测TKV变化率的程度，并确定RBF和TKV是否独立预测发生肾功能不全的风险。目的3：建立量化基线时肾囊肿数量、体积和地形图对TKV和GFR后续病程的影响以及发生肾功能不全风险的方法。目的4：扩展和分析NIDDK库中收集的脆性生物样本，以改进基因/表型和生物标记物研究，并促进辅助研究。目的：测试强化膳食咨询改变CRISP中固定的钠摄入量模式的可行性和有效性，并确定这种改变是否改变了TKV的变化率。

DESCRIPTION (provided by applicant): Autosomal dominant polycystic kidney disease (ADPKD) is a major cause of morbidity and the fourth leading cause of ESRD in the world, affecting more than 500,000 U.S. citizens. Researchers at the University of Alabama, Emory University, University of Kansas, Mayo Clinic and Washington University joined together in 2000 to create the Consortium for Radiologic Studies of Polycystic Kidney Disease (CRISPI) and in 2006 included the University of Pittsburgh in place of Washington University for CRISP II. The primary objectives of CRISPI and II were to: establish accurate, reliable and reproducible magnetic resonance based measurements of total kidney volume (TKV), liver cyst volume (LCV), renal blood flow (RBF), and patterns of cyst growth and expansion. Based on 7.3 years of longitudinal followup in 200 CRISP l/ll participants, we can now: 1) establish an unequivocal relationship between TKV and qualitative (patient reported outcomes) and quantitative (renal insufficiency) end-points; as well as 2) identify potential modifiable risk factors associating with TKV and LCV for intervention studies. TKV ultimately may be used as a surrogate marker of disease progression in clinical trials. The goals of CRISPIN extend the observations of CRlSPI/ll. The overarching Aim for CRISP III is to develop and enhance prediction models that best predict renal insufficiency in ADPKD. Specifically, Aim 1: Extend the serial quantification of TKV and LCV to develop and test new models for predicting the risk of developing renal insufficiency. Aim 2: Determine the extent to which age and sex-adjusted measurements of RBF predict the rate of change in TKV and determine if RBF and TKV independently predict the risk of developing renal insufficiency. Aim 3: Develop methods to quantify the influence of renal cyst number, volume, and topography at baseline on the subsequent course of TKV and GFR and the risk of developing renal insufficiency. Aim 4: Expand and analyze CRISP biological samples collected in NIDDK repositories to improve genotype/phenotype and biomarker studies, and facilitate ancillary studies. Aim 5: Test the feasibility and efficacy of intensive dietary counseling in modifying the fixed pattern of sodium intake observed in CRISP and determine if this change alters the rate of TKV change.