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CRISP III - Kansas Polycystic Kidney Imaging Program Supplemental Request

CRISP III - 堪萨斯州多囊肾成像计划补充请求

基本信息

批准号：
9269449
负责人：
Alan S Yu
金额：
$ 6万
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：
2016
资助国家：
美国
起止时间：
2016-06-01 至 2017-05-30
项目状态：
已结题

来源：
https://reporter.nih.gov/project-details/9269449
关键词：
Address Affect Age 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 Genetic study Genotype Goals Growth Hepatic Cyst Image Individual Instruction 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 predictive marker programs rate of change repository serological marker sex urinary

项目摘要

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 Alabanna, 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 ncluded 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 nsufficiency 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. RELEVANCE (See instructions): The results of these studies will impact the lives of patients with ADPKD. Developing predictive markers of disease severity early, prior to loss of renal insufficiency will result in increased life expectancy and improved quality of life in patients with ADPKD.

常染色体显性多囊肾病 (ADPKD) 是发病的主要原因，也是第四大疾病是全球终末期肾病 (ESRD) 的一个病因，影响着超过 50 万美国公民。大学的研究人员阿拉巴纳、埃默里大学、堪萨斯大学、梅奥诊所和华盛顿大学联手 2000 年创建多囊肾放射学研究联盟 (CRISPI)，并于 2006 年 CRISP II 包括匹兹堡大学代替华盛顿大学。主要目标 CRISPI 和 II 的目标是：建立基于磁共振的准确、可靠和可重复的测量肾总体积 (TKV)、肝囊肿体积 (LCV)、肾血流量 (RBF) 和模式囊肿生长和扩张。基于对 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 和定性（患者报告的结果）之间建立明确的关系和定量（肾功能不全）终点；以及 2) 识别潜在的可改变的风险因素与 TKV 和 LCV 联合进行干预研究。 TKV 最终可能用作替代标记临床试验中疾病进展的情况。 CRISPIN 的目标扩展了 CRlSPI/ll 的观察结果。这 CRISP III 的总体目标是开发和增强能够最好地预测肾脏疾病的预测模型 ADPKD 不足。具体来说，目标 1：扩展 TKV 和 LCV 的系列量化以开发和测试预测肾功能不全风险的新模型。目标 2：确定在多大程度上年龄和性别调整后的 RBF 测量值可以预测 TKV 的变化率，并确定 RBF 和 TKV 独立预测发生肾功能不全的风险。目标 3：开发量化方法基线时肾囊肿数量、体积和形态对后续 TKV 疗程的影响 GFR 和发生肾功能不全的风险。目标 4：扩展和分析 CRISP 生物样本收集在 NIDDK 存储库中，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修改固定饮食习惯方面的可行性和有效性 CRISP 中观察到的钠摄入模式，并确定这种变化是否会改变 TKV 变化率。相关性（参见说明）：这些研究的结果将影响 ADPKD 患者的生活。开发预测标记在肾功能不全消失之前及早发现疾病严重程度将导致预期寿命增加并改善 ADPKD 患者的生活质量。