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Glomerulocystic Kidney Disease and its rare associations: an autopsy report of two unrelated cases

BACKGROUND: Glomerulocystic 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 PRESENTATION: We describe a case of glomerulocystic disease in a neonate and another in an abor...

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Detalles Bibliográficos
Autores principales: Gupta, Kirti, Vankalakunti, Mahesha, Sachdeva, Man Updesh Singh
Formato: Texto
Lenguaje:English
Publicado: BioMed Central 2007
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1863412/
https://www.ncbi.nlm.nih.gov/pubmed/17459157
http://dx.doi.org/10.1186/1746-1596-2-12
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author Gupta, Kirti
Vankalakunti, Mahesha
Sachdeva, Man Updesh Singh
author_facet Gupta, Kirti
Vankalakunti, Mahesha
Sachdeva, Man Updesh Singh
author_sort Gupta, Kirti
collection PubMed
description BACKGROUND: Glomerulocystic 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 PRESENTATION: We 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. CONCLUSION: The associated anomalies in Glomerulocystic Kidney disease are well described in the literature. Two more new unrelated associations are described in this article.
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spelling pubmed-18634122007-05-03 Glomerulocystic Kidney Disease and its rare associations: an autopsy report of two unrelated cases Gupta, Kirti Vankalakunti, Mahesha Sachdeva, Man Updesh Singh Diagn Pathol Case Report BACKGROUND: Glomerulocystic 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 PRESENTATION: We 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. CONCLUSION: The associated anomalies in Glomerulocystic Kidney disease are well described in the literature. Two more new unrelated associations are described in this article. BioMed Central 2007-04-25 /pmc/articles/PMC1863412/ /pubmed/17459157 http://dx.doi.org/10.1186/1746-1596-2-12 Text en Copyright © 2007 Gupta et al; licensee BioMed Central Ltd. http://creativecommons.org/licenses/by/2.0 This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( (http://creativecommons.org/licenses/by/2.0) ), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Gupta, Kirti
Vankalakunti, Mahesha
Sachdeva, Man Updesh Singh
Glomerulocystic Kidney Disease and its rare associations: an autopsy report of two unrelated cases
title Glomerulocystic Kidney Disease and its rare associations: an autopsy report of two unrelated cases
title_full Glomerulocystic Kidney Disease and its rare associations: an autopsy report of two unrelated cases
title_fullStr Glomerulocystic Kidney Disease and its rare associations: an autopsy report of two unrelated cases
title_full_unstemmed Glomerulocystic Kidney Disease and its rare associations: an autopsy report of two unrelated cases
title_short Glomerulocystic Kidney Disease and its rare associations: an autopsy report of two unrelated cases
title_sort glomerulocystic kidney disease and its rare associations: an autopsy report of two unrelated cases
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1863412/
https://www.ncbi.nlm.nih.gov/pubmed/17459157
http://dx.doi.org/10.1186/1746-1596-2-12
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