[Glomerulocystic kidney disease and hemolytic-uremic syndrome: clinicopathological case]

Abstract

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.