King Bernard F, Torres Vicente E, Brummer Marijn E, Chapman Arlene B, Bae Kyongtae T, Glockner James F, Arya Kraisthith, Felmlee Joel P, Grantham Jared J, Guay-Woodford Lisa M, Bennett William M, Klahr Saulo, Hirschman Gladys H, Kimmel Paul L, Thompson Paul A, Miller J Phillip
Department of Medicine (Renal Division), University of Alabama, Birmingham, Alabama, USA.
Kidney Int. 2003 Dec;64(6):2214-21. doi: 10.1046/j.1523-1755.2003.00326.x.
Autosomal-dominant polycystic kidney disease (ADPKD) is an inherited disorder characterized by renal cyst growth, early development of hypertension, and late occurrence of renal insufficiency. Despite evidence for the importance of nephroangiosclerosis in the progression of renal insufficiency in ADPKD, evaluation of renal blood flow (RBF) as a surrogate marker of disease severity has received little attention.
Flow phantoms and repeat RBF measurements assessed accuracy and reproducibility. One hundred twenty-seven ADPKD subjects with creatinine clearances >70 mL/min underwent measurements of RBF, total, and cyst renal volumes, and % cyst volumes by magnetic resonance (MR) and of glomerular filtration rate (GFR). Renal vascular resistance (RVR) was calculated. MR blood flow sequences utilized a two-dimensional cine phase-contrast breath-hold pulse sequence perpendicular to the renal arteries. Flow rates were calculated utilizing FLOW software. Volumetric analysis was performed using stereology and region-based thresholding.
Excellent accuracy and intraobserver and interobserver reproducibility were demonstrated. Anatomic (total kidney volume, total cyst volume, and % cyst volume), hemodynamic (RBF and RVR), and functional (GFR) parameters were strongly correlated. Left polycystic kidneys were larger and had more severe disease. Regression analysis showed that age, diagnosis of hypertension, anatomic parameters and hemodynamic parameters were significant predictors of GFR. Multiple linear regression analysis identified age and hemodynamic parameters only as separate predictors of GFR. Anatomic, hemodynamic, and functional parameters discriminated between normotensive and hypertensive subjects despite antihypertensive treatments.
Renal hemodynamic parameters measured by MR correlate with anatomic and functional indices of disease severity, are the strongest predictors of renal function, and deserve further consideration as an outcome measure in clinical trials to guide therapy in ADPKD.
常染色体显性多囊肾病（ADPKD）是一种遗传性疾病，其特征为肾囊肿生长、早期出现高血压以及晚期发生肾功能不全。尽管有证据表明肾血管硬化在ADPKD肾功能不全进展中具有重要性，但将肾血流量（RBF）作为疾病严重程度的替代标志物进行评估却很少受到关注。
通过血流模型和重复RBF测量来评估准确性和可重复性。127例肌酐清除率>70 mL/分钟的ADPKD患者接受了RBF、总肾体积和囊肿肾体积以及通过磁共振（MR）测量的囊肿体积百分比和肾小球滤过率（GFR）的测量。计算肾血管阻力（RVR）。MR血流序列采用垂直于肾动脉的二维电影相位对比屏气脉冲序列。使用FLOW软件计算流速。使用体视学和基于区域的阈值化进行体积分析。
证明了极佳的准确性以及观察者内和观察者间的可重复性。解剖学参数（总肾体积、总囊肿体积和囊肿体积百分比）、血流动力学参数（RBF和RVR）和功能参数（GFR）密切相关。左侧多囊肾更大且疾病更严重。回归分析表明，年龄、高血压诊断、解剖学参数和血流动力学参数是GFR的重要预测指标。多元线性回归分析仅将年龄和血流动力学参数确定为GFR的独立预测指标。尽管进行了抗高血压治疗，但解剖学、血流动力学和功能参数仍可区分血压正常和高血压患者。
通过MR测量的肾血流动力学参数与疾病严重程度的解剖学和功能指标相关，是肾功能的最强预测指标，在临床试验中作为指导ADPKD治疗的结局指标值得进一步考虑。
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