Hotta O, Sato M, Furuta T, Taguma Y
Department of Nephrology, Sendai Shakaihoken Hospital, Japan.
Clin Nephrol. 1999 Mar;51(3):177-80.
Glomerulocystic disease is an uncommon cystic renal condition characterized by cystic dilatation forming a glomerular cyst. The pathogenesis of this familial disease is unknown. We performed a serial section study using a biopsy specimen of a 16-year old female patient with glomerular cystic disease who had a family history of end stage renal failure. A total of 14 different glomeruli were analyzed, four of which exhibited a cystic appearance. Five glomerulotubular junctions were observed by serial sections, two of which had a stenotic appearance where glomerular cystic changes and periglomerular fibrosis were observed concomitantly. There were no such cystic glomerular changes in the other three glomeruli with non-stenotic glomerulo-tubular junctions. These findings suggest that the glomerular cystic lesion develops as a consequence of glomerulo-tubular junctional stenosis probably caused by periglomerular fibrosis.
肾小球囊性疾病是一种罕见的囊性肾脏疾病，其特征是形成肾小球囊肿的囊性扩张。这种家族性疾病的发病机制尚不清楚。我们对一名16岁患有肾小球囊性疾病且有终末期肾衰竭家族史的女性患者的活检标本进行了连续切片研究。总共分析了14个不同的肾小球，其中4个呈现出囊性外观。通过连续切片观察到5个肾小球肾小管连接部，其中2个呈现狭窄外观，同时观察到肾小球囊性改变和肾小球周围纤维化。在另外3个肾小球肾小管连接部无狭窄的肾小球中未观察到此类囊性肾小球改变。这些发现表明，肾小球囊性病变是由可能由肾小球周围纤维化引起的肾小球肾小管连接部狭窄所致。
