Kanouzawa K, Tamura H, Matsumura O, Nagasawa R, Mitarai T, Isoda K, Yamanaka N
Fourth Department of Internal Medicine, Saitama Medical Center, Saitama Medical School, Japan.
Nihon Jinzo Gakkai Shi. 1994 Jun;36(6):762-8.
A 56-year-old female presented with end-stage renal disease. A CT scan of her kidneys demonstrated that the density of the renal parenchyma was quite low as compared with normal kidneys, and that corticomedullary demarcation was obscured. Magnetic resonance imaging (MRI) disclosed low intensity of the kidneys in T1-weighed images, and high intensity in T2-weighed images. In order to elucidate the etiology of her kidney disease, open renal biopsy was performed. The kidney surface was covered with numerous cysts with a diameter of less than 3 mm. Biopsy specimens from the cortical surface showed multiple cystic lesions. Serial sections of more than 200 slices of the biopsy material demonstrated that traces of collapsed glomeruli were present in most of the cysts. On electron microscopy, some epithelial cells lining the cysts were found to be round-shaped and contained a substantial amount of mitochondria, suggesting proximal tubules. These histological findings were compatible with glomerulocystic kidney disease (GCKD). An adult case of GCKD has rarely been reported, but the CT scan as well as MRI of the kidneys appeared to complement the diagnosis of GCKD. Although the cysts of GCKD have been considered to be dilatations of the Bowman's capsules, our observation suggested that part of the cells lining the cysts consisted of proximal tubular epithelium.
一名56岁女性因终末期肾病前来就诊。她的肾脏CT扫描显示，与正常肾脏相比，肾实质密度相当低，皮髓质分界不清。磁共振成像（MRI）显示，在T1加权图像中肾脏呈低信号强度，在T2加权图像中呈高信号强度。为了阐明其肾脏疾病的病因，进行了开放性肾活检。肾脏表面布满了无数直径小于3毫米的囊肿。皮质表面的活检标本显示有多个囊性病变。对活检材料的200多张连续切片进行检查发现，大多数囊肿中都存在塌陷肾小球的痕迹。电子显微镜检查发现，一些囊肿内衬上皮细胞呈圆形，含有大量线粒体，提示为近端小管。这些组织学表现符合肾小球囊性肾病（GCKD）。成人GCKD病例鲜有报道，但肾脏CT扫描及MRI似乎有助于GCKD的诊断。尽管GCKD的囊肿被认为是鲍曼囊的扩张，但我们的观察表明，囊肿内衬的部分细胞由近端小管上皮组成。
