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Glomerulocystic kidney presenting as an incidental finding in an adult male with unilateral non-functioning kidney.

Mubarak M, Shakeel S, Iqbal Kazi J - J Renal Inj Prev (2014)

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Affiliation: Department of Histopathology, Sindh Institute of Urology and Transplantation (SIUT) Karachi, Pakistan.

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On gross examination, there was gross dilatation of the pelvicalyceal system with thinned out cortex (Figure 1A)... A single small stone was found impacted in the upper ureter causing obstruction... D) High-power view showing cystic glomeruli with small shrunken glomerular tufts... The term glomerulocystic kidney (GCK) denotes a kidney with more than 5% cystic glomeruli... The cystic glomeruli or glomerular cysts, in turn, are arbitrarily defined as Bowman’s space dilatation greater than 2 to 3 times normal size... It is to be noted that GCK is not a single disease but rather it represents a phenotype that includes several diseases... The glomerular cysts are found in a variety of disorders, both hereditary and acquired, with equally varied clinical manifestations... Recent clinicopathological and molecular genetic studies have shed light on the etiopathogenesis of the disorder and its appropriate classification into clinically relevant categories... GCK is more common in males (62%) and in children (72%) as compared with adults... The condition can be asymmetric, unilateral or segmental in distribution... The present case most likely represents a unilateral disease associated with urinary obstruction and corresponds to type IV (obstructive GCK) as proposed by Lennerz et al.... The negative family history, absence of syndromic features, apparent unilaterality of the disease in an obstructed kidney and the adult onset all further corroborate this categorization... In conclusion, the diagnosis of GCK should not be considered the end result.

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Mentions: Right nephrectomy was performed. Preoperatively, gross hydronephrosis with thin cortex was observed. On gross examination, there was gross dilatation of the pelvicalyceal system with thinned out cortex (Figure 1A). A single small stone was found impacted in the upper ureter causing obstruction. Histopathological evaluation revealed marked hydronephrosis and changes of chronic non-specific pyelonephritis. In addition, the majority of the glomeruli showed cystically dilated Bowman’s spaces to more than 2-3 times normal size with small capillary tufts (Figure 1B, C). Many glomeruli were also globally sclerosed (Figure 1D). The postoperative course was uneventful and the patient is maintaining normal renal function three years after the surgical intervention.


Glomerulocystic kidney presenting as an incidental finding in an adult male with unilateral non-functioning kidney.

Mubarak M, Shakeel S, Iqbal Kazi J - J Renal Inj Prev (2014)

© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4301388&req=5

Mentions: Right nephrectomy was performed. Preoperatively, gross hydronephrosis with thin cortex was observed. On gross examination, there was gross dilatation of the pelvicalyceal system with thinned out cortex (Figure 1A). A single small stone was found impacted in the upper ureter causing obstruction. Histopathological evaluation revealed marked hydronephrosis and changes of chronic non-specific pyelonephritis. In addition, the majority of the glomeruli showed cystically dilated Bowman’s spaces to more than 2-3 times normal size with small capillary tufts (Figure 1B, C). Many glomeruli were also globally sclerosed (Figure 1D). The postoperative course was uneventful and the patient is maintaining normal renal function three years after the surgical intervention.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Histopathology, Sindh Institute of Urology and Transplantation (SIUT) Karachi, Pakistan.

AUTOMATICALLY GENERATED EXCERPT
Please rate it.

On gross examination, there was gross dilatation of the pelvicalyceal system with thinned out cortex (Figure 1A)... A single small stone was found impacted in the upper ureter causing obstruction... D) High-power view showing cystic glomeruli with small shrunken glomerular tufts... The term glomerulocystic kidney (GCK) denotes a kidney with more than 5% cystic glomeruli... The cystic glomeruli or glomerular cysts, in turn, are arbitrarily defined as Bowman’s space dilatation greater than 2 to 3 times normal size... It is to be noted that GCK is not a single disease but rather it represents a phenotype that includes several diseases... The glomerular cysts are found in a variety of disorders, both hereditary and acquired, with equally varied clinical manifestations... Recent clinicopathological and molecular genetic studies have shed light on the etiopathogenesis of the disorder and its appropriate classification into clinically relevant categories... GCK is more common in males (62%) and in children (72%) as compared with adults... The condition can be asymmetric, unilateral or segmental in distribution... The present case most likely represents a unilateral disease associated with urinary obstruction and corresponds to type IV (obstructive GCK) as proposed by Lennerz et al.... The negative family history, absence of syndromic features, apparent unilaterality of the disease in an obstructed kidney and the adult onset all further corroborate this categorization... In conclusion, the diagnosis of GCK should not be considered the end result.

No MeSH data available.