Difference between revisions of "Nephrotic syndrome"

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In children nephrotic syndrome is assumed to be ''minimal change disease''.  Biopsies are done only there is no response to steriods.
In children nephrotic syndrome is assumed to be ''minimal change disease''.  Biopsies are done only there is no response to steriods.


=Clinical definition=
Features:
*Anasarca (whole body - edema).
*Proteinuria (>3.5 g/24h).
*Hypercholesterolemia.
*Hypoalbuminemia.
=Overview=
Immune complex negative:
#[[MCD]].
#[[FSGS]].
Immune complex positive:
#[MN]].
#[[IgA nephropathy]].
=Specific entities=
==Minimal change disease==
==Minimal change disease==
*Abbreviated ''MCD''.
===General===
===General===
*Responds to steroids.
*Responds to steroids.
*Most common cause of nephrotic syndrome in children.


===Microscopic===
===Microscopic===
Line 13: Line 31:
===EM===
===EM===
Features:
Features:
Diffuse loss of foot processes.
*Diffuse loss of foot processes.
 
Note:
*Foot processes on urinary space side.


Image:
Image:
Line 19: Line 40:


==Focal segmental glomerulosclerosis==
==Focal segmental glomerulosclerosis==
*Abbreviated ''FSGS''.
===General===
===General===
*Abbreviated ''FSGS''.
*Presents as nephrotic syndrome.
*Presents as nephrotic syndrome.
*Does not respond to steroids (unlike MCD).
*Does not respond to steroids (unlike MCD).
Line 65: Line 86:
====Histologic classification====
====Histologic classification====
FSGS can be subdivided into the following subgroups:<ref name=pmid16518352>{{Cite journal  | last1 = Thomas | first1 = DB. | last2 = Franceschini | first2 = N. | last3 = Hogan | first3 = SL. | last4 = Ten Holder | first4 = S. | last5 = Jennette | first5 = CE. | last6 = Falk | first6 = RJ. | last7 = Jennette | first7 = JC. | title = Clinical and pathologic characteristics of focal segmental glomerulosclerosis pathologic variants. | journal = Kidney Int | volume = 69 | issue = 5 | pages = 920-6 | month = Mar | year = 2006 | doi = 10.1038/sj.ki.5000160 | PMID = 16518352 }}</ref>
FSGS can be subdivided into the following subgroups:<ref name=pmid16518352>{{Cite journal  | last1 = Thomas | first1 = DB. | last2 = Franceschini | first2 = N. | last3 = Hogan | first3 = SL. | last4 = Ten Holder | first4 = S. | last5 = Jennette | first5 = CE. | last6 = Falk | first6 = RJ. | last7 = Jennette | first7 = JC. | title = Clinical and pathologic characteristics of focal segmental glomerulosclerosis pathologic variants. | journal = Kidney Int | volume = 69 | issue = 5 | pages = 920-6 | month = Mar | year = 2006 | doi = 10.1038/sj.ki.5000160 | PMID = 16518352 }}</ref>
*Cellular.
{| class="wikitable"
*Collapsing - poor prognosis.
!Subtype
*Tip lesion - good prognosis.
!Comment
*Perihilar.
|-
*Not otherwise specified (NOS) - most common.
|Cellular
|abrupt severe onset
|-
|Collapsing  
|poor prognosis, viral/toxic etiology
|-
|Tip lesion  
|good prognosis
|-
|Perihilar
|reduced renal mass
|-
|Not otherwise specified (NOS)  
|most common
|}


===Stains===
===Stains===
Line 95: Line 130:
*[http://commons.wikimedia.org/wiki/File:Membranous_Nephropathy_Pathology_Diagram.svg MN - schematic (WC)].
*[http://commons.wikimedia.org/wiki/File:Membranous_Nephropathy_Pathology_Diagram.svg MN - schematic (WC)].


==See also==
=See also=
*[[Medical kidney diseases]].
*[[Medical kidney diseases]].
*[[Kidney tumours]].
*[[Kidney tumours]].


==References==
=References=
{{Reflist|1}}
{{Reflist|2}}


[[Category:Genitourinary pathology]]
[[Category:Genitourinary pathology]]

Revision as of 20:29, 29 October 2011

Nephrotic syndrome is a constellation of clinical findings seen in a number of medical kidney diseases. This article deals with them. An introduction to the medical kidney is in the medical kidney diseases article.

In children nephrotic syndrome is assumed to be minimal change disease. Biopsies are done only there is no response to steriods.

Clinical definition

Features:

  • Anasarca (whole body - edema).
  • Proteinuria (>3.5 g/24h).
  • Hypercholesterolemia.
  • Hypoalbuminemia.

Overview

Immune complex negative:

  1. MCD.
  2. FSGS.

Immune complex positive:

  1. [MN]].
  2. IgA nephropathy.

Specific entities

Minimal change disease

  • Abbreviated MCD.

General

  • Responds to steroids.
  • Most common cause of nephrotic syndrome in children.

Microscopic

Features:

  • No changes on light microscopy.

EM

Features:

  • Diffuse loss of foot processes.

Note:

  • Foot processes on urinary space side.

Image:

Focal segmental glomerulosclerosis

  • Abbreviated FSGS.

General

  • Presents as nephrotic syndrome.
  • Does not respond to steroids (unlike MCD).

Etiology

  • Primary.
    • May be familial.[1]
  • Secondary.[2]
    • HIV.
    • Drug use.
    • Reduced renal mass.

Primary vs. secondary:[3]

Feature Primary Secondary
Proteinuria (onset) sudden progressive
Albumnin low normal
Glomerulus size normal increased
Foot process effacement diffuse mild

Microscopic

Features:

  • Partial sclerosis of less than 50% of glomeruli.

Image:

Histologic classification

FSGS can be subdivided into the following subgroups:[4]

Subtype Comment
Cellular abrupt severe onset
Collapsing poor prognosis, viral/toxic etiology
Tip lesion good prognosis
Perihilar reduced renal mass
Not otherwise specified (NOS) most common

Stains

Features:[5]

  • PAS +ve crescents.

Membranous nephropathy

General

  • AKA membranous glomerulonephritis.
  • Presents as nephrotic syndrome.

Clinical DDx:[6]

  • Hepatitis B.
  • Hepatitis C.
  • Carcinoma.
  • NSAID toxicity.
  • SLE.
  • Idiopathic.

Microscopic

Features:

  • Subepithelial immune complex depositions, spike forming.

Image:

See also

References

  1. ↑ Sánchez de la Nieta MD, Arias LF, Alcázar R, et al. (2003). "[Familial focal and segmentary hyalinosis]" (in Spanish; Castilian). Nefrologia 23 (2): 172–6. PMID 12778884.
  2. ↑ URL: http://www.kidneypathology.com/English_version/Focal_segmental_GS.html. Accessed on: 11 February 2011.
  3. ↑ D'Agati, V. (Mar 2003). "Pathologic classification of focal segmental glomerulosclerosis.". Semin Nephrol 23 (2): 117-34. doi:10.1053/snep.2003.50012. PMID 12704572.
  4. ↑ Thomas, DB.; Franceschini, N.; Hogan, SL.; Ten Holder, S.; Jennette, CE.; Falk, RJ.; Jennette, JC. (Mar 2006). "Clinical and pathologic characteristics of focal segmental glomerulosclerosis pathologic variants.". Kidney Int 69 (5): 920-6. doi:10.1038/sj.ki.5000160. PMID 16518352.
  5. ↑ URL: http://www.kidneypathology.com/English_version/Focal_segmental_GS.html. Accessed on: 11 February 2011.
  6. ↑ Klatt, Edward C. (2006). Robbins and Cotran Atlas of Pathology (1st ed.). Saunders. pp. 241. ISBN 978-1416002741.
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