Nephrotic syndrome

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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.

Minimal change disease

General

  • Responds to steroids.

Microscopic

Features:

  • No changes on light microscopy.

EM

Features: Diffuse loss of foot processes.

Image:

Focal segmental glomerulosclerosis

General

  • Abbreviated FSGS.
  • 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]

  • Cellular.
  • Collapsing - poor prognosis.
  • Tip lesion - good prognosis.
  • Perihilar.
  • 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.