Vera-Sempere F, Zamora I, Simón J M
Servicio de Anatomía Patológica, Hospital Universitario La Fe, Valencia.
Nefrologia. 2000 Sep-Oct;20(5):459-63.
Glomerulocystic kidney is a heterogeneous group of conditions morphologically characterised by multiple cortical cysts apparently originated from a cystic dilation of the filtration space with atrophy of the glomerular tufts. We report a case of glomerulocystic kidney affecting a 13-year-old boy who underwent renal transplantation for end-stage renal disease following a haemolytic-uraemic syndrome diagnosed nine years ago. The absence of other stigmas (urinary obstruction, extrarenal congenital abnormalities and family history of cystic kidney disease) suggest that our observation is apparently a sporadic and acquired glomerulocystic kidney following a haemolytic-uraemic syndrome, an infrequent association previously reported only twice. Our histological and immunohistochemical findings suggest that the cysts in this rare condition are really of glomerular origin but the pathogenesis of cyst development remains unknown.
肾小球囊性肾病是一组异质性疾病,其形态学特征为多个皮质囊肿,这些囊肿显然起源于滤过空间的囊性扩张,并伴有肾小球丛萎缩。我们报告一例肾小球囊性肾病,患者为一名13岁男孩,他在9年前被诊断为溶血性尿毒症综合征后,因终末期肾病接受了肾移植。由于不存在其他特征(尿路梗阻、肾外先天性异常和多囊肾病家族史),提示我们观察到的显然是一例溶血性尿毒症综合征后散发性获得性肾小球囊性肾病,这种罕见的关联此前仅报道过两次。我们的组织学和免疫组化结果表明,这种罕见疾病中的囊肿确实起源于肾小球,但囊肿形成的发病机制仍不清楚。