Polycystic Kidney Disease: Innovative Imaging to Assess Progression (PCC)

多囊肾病：评估进展的创新影像学 (PCC)

• 批准号: 7342835
• 负责人: Vicente E Torres
• 金额: $ 36.95万
• 依托单位: MAYO CLINIC ROCHESTER
• 依托单位国家: 美国
• 财政年份: 2000
• 资助国家: 美国
• 起止时间: 2000-02-01 至 2010-12-31
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/7342835
• 关键词: Affect; Age; Alabama; Albuminuria; Autosomal Dominant Polycystic Kidney; Blood Pressure; Characteristics; Clinic; Clinical; Clinical Trials; Computer Retrieval of Information on Scientific Projects Database; Cyst; Cystic kidney; Data; Databases; Disease Progression; End Point; End stage renal failure; Event; Excretory function; Family member; Future; Genetic; Genotype; Goals; Growth; Hematuria; Hepatic; Hour; Hypertension; Image; Imaging Techniques; Individual; Intervention; Investigation; Kansas; Kidney; Kidney Calculi; Kidney Failure; Life; Magnetic Resonance Imaging; Measurement; Measures; Metric; Monitor; Monocyte Chemoattractant Protein-1; Morbidity - disease rate; Outcome; Pain; Participant; Pattern; Phenotype; Polycystic Kidney Diseases; Rate; Renal Blood Flow; Renal function; Renin-Angiotensin-Aldosterone System; Reproducibility; Research Personnel; Sampling; Severity of illness; Technology; Testing; Time; Universities; Urinary tract infection; Ursidae Family; Washington; innovation; sex; size; urinary

DESCRIPTION (provided by applicant): 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 (CRISP-I). The primary objectives of this investigation were to: (1) Develop and test the accuracy and reproducibility of imaging techniques to monitor changes in renal cyst size and parenchymal involvement. (2) Establish and maintain a database of uniformly and accurately collected information. (3) Maintain and make available such data to facilitate the planning and implementation of clinically appropriate interventions in the near future. The goals of CRISP-I I 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) 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) Develop and test other bio-markers of disease progression. The specific aims are: Aim 1: Extend the preliminary observations of CRISP-I 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 CRISP-I 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 CRISP-I and the CRISP-II extension to develop and test new metrics to quantify and monitor disease progression, and collect DMA samples and clinical information from CRISP family members known to have ADPKD for use in future studies to examine genotype-phenotype correlations and to identify genetic modifiers.

描述（由申请人提供）：常染色体显性多囊性肾脏疾病（ADPKD）是造成发病率的主要原因，并且是世界上终末期肾衰竭的第四个主要原因，影响了50万美国公民和全球范围内数百万。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圣路易斯大学的研究人员于2000年一起创建了多囊肾脏病放射学研究联盟（CRISP-I）。这项研究的主要目标是：（1）发展和测试成像技术的准确性和可重复性，以监测肾脏囊肿大小和实质受累的变化。 （2）建立并维护一个统一，准确收集的信息的数据库。 （3）维护并提供此类数据，以促进在不久的将来计划和实施临床适当的干预措施。 Crisp-i的目标是将Crispi的观察结果扩展到：1）在肾脏/囊肿扩大的速度与定性和定量终点之间取得明确的联系。 2）在旨在防止疾病进展的临床试验中，提供了敏感和准确的疾病进展（肾脏体积）的标志。 3）开发和测试其他疾病进展的生物标志物。具体目的是：目标1：扩展Crisp-I的初步观察结果，以确定定量（肾脏体积和肝和肾脏囊肿体积）或定性（囊肿分布和特征）结构参数的程度可预测肾功能不全。目标2：扩展Crisp-I的初步观察结果，以确定MR技术对肾脏血流的年龄和性别调整后的测量程度预测了肾脏生长的速度；而且，肾脏流量和肾脏体积预测ADPKD的肾功能下降率。 AIM 3：详尽分析源自Crisp-I和Crisp-II扩展的生物数据库，并存储生物学样本，以开发和测试新的指标，以量化和监测疾病的进展，并收集来自CRIRP家族成员的DMA样本和临床信息，这些家族成员已被众所周知的ADPKD用于使用ADPKD，可在未来的研究中用于检查基因型 - 型号型号Corelations Corelations Corelations Corelations coreltenty Modifiers center Generatife Modifiers。