Kansas Polycystic Kidney Imaging Program

堪萨斯州多囊肾成像计划

基本信息

批准号：
8710152
负责人：
Alan S Yu
金额：
$ 27.13万
依托单位：
UNIVERSITY OF KANSAS MEDICAL CENTER
依托单位国家：
美国
项目类别：
财政年份：
2000
资助国家：
美国
起止时间：
2000-02-01 至 2016-05-31
项目状态：
已结题

来源：
https://reporter.nih.gov/project-details/8710152
关键词：
Address Affect Age Alabama Ancillary Study Autosomal Dominant Polycystic Kidney Biological Biological Markers Characteristics Clinic Clinical Clinical Data Clinical Trials Computer Retrieval of Information on Scientific Projects Database Counseling Cyst Cystic kidney DNA Data Data Analyses Development Diet Disease Progression End stage renal failure Family member Funding Genetic Genotype Goals Growth Hepatic Cyst Image Individual Intake Intervention Intervention Studies Kansas Kidney Kidney Diseases Kidney Failure Life Expectancy Liver Magnetic Resonance Magnetic Resonance Imaging Measurement Methods Modeling Morbidity - disease rate National Institute of Diabetes and Digestive and Kidney Diseases Nature Other Genetics Participant Patient Outcomes Assessments Patients Pattern Phenotype Pilot Projects Polycystic Kidney Diseases Population Predictive Value Principal Investigator Proteomics Quality of life Renal Blood Flow Renal function Research Personnel Resources Risk Risk Factors Role Sampling Sensitivity and Specificity Severities Severity of illness Sodium Surrogate Markers Testing Universities Washington base follow-up genetic risk factor improved kidney imaging modifiable risk programs repository serological marker sex urinary

项目摘要

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.

描述（由申请人提供）：常染色体显性多囊肾病 (ADPKD) 是发病的主要原因，也是世界上 ESRD 的第四大原因，影响超过 500,000 名美国公民。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 年联合创建了多囊肾病放射学研究联盟 (CRISPI)，并于 2006 年将匹兹堡大学纳入其中以取代华盛顿大学对于 CRISP II。 CRISPI 和 II 的主要目标是：建立基于磁共振的准确、可靠和可重复的肾总体积 (TKV)、肝囊肿体积 (LCV)、肾血流量 (RBF) 以及囊肿生长和扩张模式的测量。基于 200 名 CRISP l/ll 参与者 7.3 年的纵向随访，我们现在可以： 1) 在 TKV 与定性（患者报告的结果）和定量（肾功能不全）终点之间建立明确的关系； 2) 确定与 TKV 和 LCV 相关的潜在可改变危险因素，以进行干预研究。 TKV 最终可能在临床试验中用作疾病进展的替代标志物。 CRISPIN 的目标扩展了 CRlSPI/ll 的观察结果。 CRISP III 的总体目标是开发和增强最能预测 ADPKD 肾功能不全的预测模型。具体来说，目标 1：扩展 TKV 和 LCV 的连续量化，以开发和测试预测肾功能不全风险的新模型。目标 2：确定年龄和性别调整后的 RBF 测量值预测 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。目标 3：开发方法来量化基线时肾囊肿数量、体积和地形对后续 TKV 和 GFR 进程以及发生肾功能不全风险的影响。目标 4：扩展和分析 NIDDK 存储库中收集的 CRISP 生物样本，以改进基因型/表型和生物标志物研究，并促进辅助研究。目标 5：测试强化饮食咨询在改变 CRISP 中观察到的固定钠摄入模式的可行性和有效性，并确定这种变化是否会改变 TKV 变化率。