Kidney Institute, University of Kansas Medical Center, 3901 Rainbow Boulevard, Kansas City, KS 66160, USA.
Clin J Am Soc Nephrol. 2010 May;5(5):889-96. doi: 10.2215/CJN.00550110. Epub 2010 Apr 1.
In autosomal dominant polycystic kidney disease, cysts derived from tubules are detected at birth by ultrasound (threshold for detection >7.0 mm); thus, fetal cyst growth rates must exceed 2300%/yr. In adults, the combined renal cyst component enlarges at approximately 12%/yr by growth of individual cysts. To explore this discrepancy, the growth rates of individual cysts were determined in adult polycystic kidneys.
DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: Diameter, volume, and growth rates of individual cysts were measured by magnetic resonance in 30 individual cysts in three adult patients over a span of 3 years. Results were confirmed in 22 cysts measured in five patients by computed tomography over a span of 11 years.
Mean cyst diameters were 20.4 +/- 9.9 mm (range 7.1 to 40.5 mm) at baseline and 25.8 +/- 15.6 mm (range 7.8 to 49.6 mm) after 3 years. Mean cyst volumes, determined by manual segmentation and summation of magnetic resonance cross sections, were 8.7 +/- 12.9 cm(3) (0.3 to 43.3 cm(3)) and 24.2 +/- 66.3 cm(3) (0.3 to 364.8 cm(3)) after 3 years. Mean cyst growth rates ranged from 6.9 to 23.9%/yr; the maximum growth rate was 71.1%/yr, far less than required to develop a 7-mm diameter cyst in utero. Results were similar in 22 cysts examined by computed tomography.
It was concluded that renal cysts detected by ultrasound in newborns must have grown at exuberant rates in utero; thereafter, expansion appears to proceed at much slower rates.
常染色体显性多囊肾病中,通过超声在出生时即可检测到源自肾小管的囊肿(检测阈值>7.0mm);因此,胎儿囊肿生长速度必须超过 2300%/年。在成年人中,通过单个囊肿的生长,多囊肾的肾囊肿复合成分以约 12%/年的速度增大。为了探索这一差异,本研究在成人多囊肾中确定了单个囊肿的生长速度。
设计、设置、参与者和测量:通过磁共振在 3 名成年患者的 30 个单个囊肿中测量直径、体积和生长速度,跨度为 3 年。在 11 年跨度中,通过计算机断层扫描在 5 名患者的 22 个囊肿中测量了结果。
基线时平均囊肿直径为 20.4 ± 9.9mm(范围 7.1 至 40.5mm),3 年后为 25.8 ± 15.6mm(范围 7.8 至 49.6mm)。通过磁共振横断面手动分割和求和确定的平均囊肿体积分别为 8.7 ± 12.9cm3(0.3 至 43.3cm3)和 24.2 ± 66.3cm3(0.3 至 364.8cm3),3 年后。平均囊肿生长速度范围为 6.9%/年至 23.9%/年;最大生长速度为 71.1%/年,远低于在子宫内形成 7mm 直径囊肿所需的速度。通过计算机断层扫描检查的 22 个囊肿的结果相似。
本研究得出结论,新生儿通过超声检测到的肾囊肿在子宫内必须以旺盛的速度生长;此后,扩张似乎以慢得多的速度进行。