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==Overview==
==Overview==


==Other Diagnostic Studies==
==Renal Biopsy==
* Kidney [[Needle aspiration biopsy|biopsy]]
A kidney biopsy with electron microscopy is recommended for all patients with FSGS for prognostic and therapeutic purposes. The following table shows the pathological classification of FSGS based on characteristic features. Electron microscopy generally shows podocyte foot process effacement.
 
Based on the proposed Columbia classification by D’Agati and colleagues<ref name="pmid14750104">{{cite journal| author=D'Agati VD, Fogo AB, Bruijn JA, Jennette JC| title=Pathologic classification of focal segmental glomerulosclerosis: a working proposal. | journal=Am J Kidney Dis | year= 2004| volume= 43 | issue= 2 | pages= 368-82 | pmid=14750104 | doi= | pmc= |url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14750104  }} </ref> in 2004, the classification of focal segmental glomerulosclerosis (FSGS) based on the morphology is as follows:
 
{| border="1" style="border-collapse:collapse; text-align:left;" cellpadding="5" align="center"
|+ '''''Pathological Classification of Focal Segmental Glomerulosclerosis<ref name="pmid14750104">{{cite journal| author=D'Agati VD, Fogo AB, Bruijn JA, Jennette JC|title=Pathologic classification of focal segmental glomerulosclerosis: a working proposal. |journal=Am J Kidney Dis | year= 2004 |volume= 43 | issue= 2 | pages= 368-82 | pmid=14750104 |doi= | pmc= |url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=14750104  }} </ref>'''''
| bgcolor="#d9ff54"|'''Variant''' || bgcolor="#d9ff54"|'''Location of Lesion'''||bgcolor="#d9ff54"|'''Distribution of Lesion'''|| bgcolor="#d9ff54"|'''Characteristic Features'''
|-
| bgcolor="#ececec"|'''Not Otherwise Specified (NOS)''' || Anywhere|| Segmental|| Capillary lumen abolished by the segmental increase in matrix.
|-
| bgcolor="#ececec"|'''Perihilar Variant''' || Perihilar||Segmental|| Presence of one or more glomeruli containing hyalinosis in the perihilar regions with or without sclerosis. Within each glomerulus, the segmental lesions must contain > 50% perihilar hyalinosis and/or sclerosis.
|-
| bgcolor="#ececec"|'''Cellular Variant''' || Anywhere|| Segmental|| Presence of one or more glomerulus with segmental hypercellularity of the capillary endothelium that blocks the capillary lumen, with or without foam cells and/or karryohexis.
|-
| bgcolor="#ececec"|'''Tip Variant''' || At tip domain|| Segmental|| One or more segmental lesions, that include tip domains. Lesions must have adhesions/confluence of podocytes with parietal or tubular cells. Tip domains are defined as 25% of tuft adjacent to the origin of the proximal tubule.  Sclerosing lesions shuld be <25% of tuft, while cellular lesions should be < 50% of tuft. No perihilar sclerosis should be observed.
|-
|bgcolor="#ececec"|'''Collapsing Variant''' || Anywhere|| Segmental or global|| One or more glomeruli with collapse with evidence of podocyte hypertrophy and hyperplasia.
|}
 
<sup><center>Adapted from D’Agati VD, Fogo AB, Bruijn JA, and Jennette JC. Pathological classification of focal segmental glomerulosclerosis. A working proposal. ''Am J of Kidney Dis''. 2004; 43(2):368-382.</center></sup>


==References==
==References==

Latest revision as of 19:56, 11 June 2018

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor-In-Chief:’’’ Manpreet Kaur, MD [2] Cafer Zorkun, M.D., Ph.D. [3]


Overview

Renal Biopsy

A kidney biopsy with electron microscopy is recommended for all patients with FSGS for prognostic and therapeutic purposes. The following table shows the pathological classification of FSGS based on characteristic features. Electron microscopy generally shows podocyte foot process effacement.

Based on the proposed Columbia classification by D’Agati and colleagues[1] in 2004, the classification of focal segmental glomerulosclerosis (FSGS) based on the morphology is as follows:

Pathological Classification of Focal Segmental Glomerulosclerosis[1]
Variant Location of Lesion Distribution of Lesion Characteristic Features
Not Otherwise Specified (NOS) Anywhere Segmental Capillary lumen abolished by the segmental increase in matrix.
Perihilar Variant Perihilar Segmental Presence of one or more glomeruli containing hyalinosis in the perihilar regions with or without sclerosis. Within each glomerulus, the segmental lesions must contain > 50% perihilar hyalinosis and/or sclerosis.
Cellular Variant Anywhere Segmental Presence of one or more glomerulus with segmental hypercellularity of the capillary endothelium that blocks the capillary lumen, with or without foam cells and/or karryohexis.
Tip Variant At tip domain Segmental One or more segmental lesions, that include tip domains. Lesions must have adhesions/confluence of podocytes with parietal or tubular cells. Tip domains are defined as 25% of tuft adjacent to the origin of the proximal tubule. Sclerosing lesions shuld be <25% of tuft, while cellular lesions should be < 50% of tuft. No perihilar sclerosis should be observed.
Collapsing Variant Anywhere Segmental or global One or more glomeruli with collapse with evidence of podocyte hypertrophy and hyperplasia.
Adapted from D’Agati VD, Fogo AB, Bruijn JA, and Jennette JC. Pathological classification of focal segmental glomerulosclerosis. A working proposal. Am J of Kidney Dis. 2004; 43(2):368-382.

References

  1. 1.0 1.1 D'Agati VD, Fogo AB, Bruijn JA, Jennette JC (2004). "Pathologic classification of focal segmental glomerulosclerosis: a working proposal". Am J Kidney Dis. 43 (2): 368–82. PMID 14750104.

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