Kern S, Zimmerhackl L B, Hildebrandt F, Ermisch-Omran B, Uhl M
Department of Diagnostic Radiology, University Hospital Freiburg, Germany.
Pediatr Radiol. 2000 Mar;30(3):156-60. doi: 10.1007/s002470050035.
To describe the appearance of autosomal recessive polycystic kidney disease (ARPKD) on MRI and RARE-MR urography.
Seven boys and one girl (aged 3 months to 14 years, median 2.5 years) were evaluated. Images were obtained with 0.23-T and 1.5-T MR systems using T1-weighted (T1-W) spin-echo, T2-weighted (T2-W) turbo-spin-echo and RARE-MR-urography sequences. Signal intensities, morphological appearance of the affected kidneys and, specifically, the picture of the urinary tract on RARE-MR-urography were evaluated.
All children showed kidney enlargement, reniform but humpy kidney shape, homogeneously grainy renal parenchyma, normal renal pelvis and normal calyces. Signal intensity was hyperintense in T2-W images in all cases. In six cases (n = 7), T1-W images were hypointense. On RARE-MR urography a hyperintense, linear radial pattern was seen in the cortex and medulla which represents the characteristic microcystic dilatation of collecting ducts in ARPKD. Three boys and the girl presented with a few circumscribed small subcapsular cysts.
In order to confirm the diagnosis of ARPKD, RARE-MR urography seems to be a non-invasive imaging tool that shows directly the microcystic dilated water-filled collecting ducts.
描述常染色体隐性多囊肾病（ARPKD）在MRI及快速自旋回波磁共振尿路造影（RARE-MR urography）上的表现。
对7名男孩和1名女孩（年龄3个月至14岁，中位年龄2.5岁）进行评估。使用0.23-T和1.5-T MR系统，采用T1加权（T1-W）自旋回波、T2加权（T2-W）快速自旋回波及RARE-MR尿路造影序列获取图像。评估信号强度、患肾的形态外观，特别是RARE-MR尿路造影上的尿路图像。
所有患儿均表现为肾脏增大，肾形但表面凹凸不平，肾实质呈均匀颗粒状，肾盂及肾盏正常。所有病例在T2-W图像上信号强度均为高信号。7例中有6例在T1-W图像上呈低信号。在RARE-MR尿路造影上，皮质和髓质可见高信号的线性放射状图案，代表ARPKD中集合管特征性的微囊性扩张。3名男孩及该女孩出现少数边界清楚的小囊下囊肿。
为确诊ARPKD，RARE-MR尿路造影似乎是一种无创成像工具，可直接显示微囊性扩张的充满水的集合管。
