Consortium for Radiologic Imaging Studies in Polycystic Kidney Disease (CRISP)

常染色体显性遗传性多囊肾病(ADPKD)是发病率的主要原因，是第四位 ESRD是世界上导致ESRD的主要原因，影响着50多万美国公民。该研究所的研究人员 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 2000年联合成立了多囊肾病放射学研究联盟(CRISP I)，并在2006年将匹兹堡大学取代华盛顿大学列入CRISP II。 CRISP I和II的主要目标是：建立准确、可靠和可重现的磁力 基于磁共振的总肾体积(TKV)、肝囊肿体积(LCV)、肾血流量的测量 (RBF)，以及包囊生长和扩张的模式。基于200年7.3年的纵向跟踪调查 清晰的I/I I参与者，我们现在可以1)在TKV和定性之间建立明确的关系 (患者报告的结果)和量化(肾功能不全)终点；以及2)确定潜在的 与TKV和LCV相关的可修改的危险因素进行干预。将军澳最终可能会被用作 临床试验中疾病进展的替代标记。CRISP III的目标扩展了对 清新的L/11。CRISP III的总体目标是开发和增强最佳预测的预测模型 ADPKD合并肾功能不全。具体地说，目标1；扩展TKV和LCV的序列量化以开发 并测试预测发生肾功能不全风险的新模型。目标2：确定 哪些年龄和性别调整的RBF测量可以预测TKV的变化率，并确定RBF TKV独立预测发生肾功能不全的风险。目标3：制定量化方法 基线时肾囊肿数、体积和地形图对TKV后续病程的影响 肾小球滤过率和发生肾功能不全的风险。目标4：扩展和分析CRISP生物学 样本，以改善基因/表型和生物标记物研究。目标5.确定强化饮食是否 在一小群干净利落的参与者中进行咨询和干预，成功地改变了相对 在CRISP I中观察到固定的钠摄取模式，并降低多囊肾的生长速度。 相关性(请参阅说明)： 这些研究的结果将影响ADPKD患者的生活。发展预测标记物 病情严重程度越早，肾功能不全消失之前，治疗/药物疗效越好。 发展并导致ADPKD患者预期寿命的延长和生活质量的改善。

Autosomal dominant polycystic kidney disease (ADPKD) is a major cause of morbidity and is 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 (CRISP I) and In 2006 included the University of Pittsburgh in place of Washington University for CRISP II. The primary objectives of CRISP I 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 follow-up in 200 CRISP I/I I 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 to intervene upon. TKV ultimately may be used as a surrogate marker of disease progression in clinical trials. The goals of CRISP III extend the observations of CRISP l/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 to improve genotype/phenotype and biomarker studies. Aim 5. Determine whether intensive dietary counseling and intervention in a small group of CRISP participants is successful in modifying the relatively fixed pattern of sodium intake observed in CRISP I and reducing the rate of growth of the polycystic kidneys. 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 efficiency of theray/drug development and result in increased life expectancy and improved quality of life in patients with ADPKD.