The Department of Radiology, 200 Lothrop Street, Ste. 4895, University of Pittsburgh, Pittsburgh, PA 15213, USA.
Nat Rev Nephrol. 2010 Feb;6(2):96-106. doi: 10.1038/nrneph.2009.214.
Autosomal dominant polycystic kidney disease (ADPKD) is characterized by the unrelenting enlargement of innumerable cysts derived from renal tubules. This cystic growth often leads to a grotesque renal enlargement. Relatively early in life, the cysts trigger secondary complications including pain, hypertension and gross hematuria; renal insufficiency is usually not detected until the fifth or sixth decade of life. Therapies targeted to molecular and pathophysiological abnormalities slow cyst growth and protect renal function in animal models of the disease. Unfortunately, the translation of these treatments into clinical trials is hampered since glomerular filtration rate, the usual biomarker of renal disease progression, does not decrease substantially until extensive and irreversible damage to noncystic parenchyma occurs. Ultrasonography, CT and MRI have been used for many years to quantify the increase in renal volume in patients with ADPKD. Imaging with these techniques has also been used to accurately quantify the rate of increased kidney and total cyst volume in patients. In this Review we discuss the overwhelming evidence in support of the view that imaging is an invaluable tool to monitor the onset and progression of ADPKD and is well-suited to gauge the response of this disease to targeted therapy before renal function begins to decline.
常染色体显性多囊肾病（ADPKD）的特征是无数源自肾小管的囊肿不断增大。这种囊性生长通常会导致肾脏严重增大。在生命的相对早期，囊肿会引发继发性并发症，包括疼痛、高血压和肉眼血尿；直到生命的第五或第六个十年，通常才会发现肾功能不全。针对疾病的分子和病理生理异常的治疗方法可以减缓囊肿的生长并保护肾功能，在动物模型中已经得到证实。不幸的是，这些治疗方法在临床试验中的转化受到阻碍，因为肾小球滤过率（通常是肾功能进展的生物标志物）在非囊性实质发生广泛和不可逆转的损伤之前不会显著降低。多年来，超声、CT 和 MRI 一直用于量化 ADPKD 患者肾脏体积的增加。这些技术的影像学检查也被用于准确量化患者肾脏和总囊肿体积增加的速度。在这篇综述中，我们讨论了压倒性的证据支持这样一种观点，即影像学是监测 ADPKD 发病和进展的非常有价值的工具，非常适合在肾功能开始下降之前评估针对这种疾病的靶向治疗的反应。
