Abstract
BackgroundGlomerulocystic kidney disease is an uncommon type of cystic renal disease. It is characterized by cortical microsysts, which are represented by cystic dilatation of Bowman's spaces.Case presentationWe describe a case of glomerulocystic disease in a neonate and another in an abortus associated with tracheo-oesophageal fistula and megacystic-megaureter syndrome. The kidney on autopsy was sponge-like and revealed presence of cysts corresponding to dilatations of Bowman's space microscopically. In these two cases, the Glomerulocystic Kidney Disease in one case corresponded to a sporadic form and, in the other, to a syndromic, non-heritable form of glomerulocystic kidney disease.ConclusionThe associated anomalies in Glomerulocystic Kidney disease are well described in the literature. Two more new unrelated associations are described in this article.
Highlights
The associated anomalies in Glomerulocystic Kidney disease are well described in the literature
Glomerulocystic kidney disease (GCKD) was characterized as a distinct histopathological entity in 1976 by Taxy and Filmer, who described this as a heterogeneous group of disorders with a common link being a variable degree of cystic dilatation of Bowman's spaces [1]
Glomerular cysts have been observed in various renal cystic diseases such as autosomal dominant polycystic kidney disease (ADPKD), cystic renal dysplasia, autosomal recessive polycystic kidney disease (ADPKD), Zellweger's cerebral-renal-hepatic syndrome, and tuberous sclerosis [2]
Summary
Glomerulocystic kidney disease (GCKD) was characterized as a distinct histopathological entity in 1976 by Taxy and Filmer, who described this as a heterogeneous group of disorders with a common link being a variable degree of cystic dilatation of Bowman's spaces [1] These cortical cysts seen grossly are mostly located in the subcapsular area of the renal cortex. The attached umbilical cord measured 10 cm and on transverse section showed presence of three vessels Both the kidneys were normal sized with maintained reniform shape and a sponge-like appearance due to numerous, round, cystic spaces, which measured up to 2 mm (Fig 1a) and were evenly distributed all along the cortex on cut surface (Fig 1b). Neither cysts nor fibrosis were identified in any of these organs
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