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Kidney nontumor
Other hereditary renal disease
Fibronectin glomerulopathy

Nikhil Sangle, M.D.

Topic Completed: 6 February 2017

Revised: 26 February 2019

Copyright: 2002-2019, PathologyOutlines.com, Inc.

PubMed Search: Fibronectin glomerulopathy [title]

Nikhil Sangle, M.D.
Page views in 2018: 122
Page views in 2019 to date: 166
Table of Contents
Definition / general | Case reports | Microscopic (histologic) description | Microscopic (histologic) images | Immunofluorescence description | Electron microscopy description | Electron microscopy images
Cite this page: Sangle N. Fibronectin glomerulopathy. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/kidneyfibronectinglomerulopathy.html. Accessed November 19th, 2019.
Definition / general
  • Massive fibronectin deposition in glomeruli due to autosomal dominant, non sex-linked disorder with 1q32 abnormality (Am J Hum Genet 1998;63:1724, OMIM #601894)
  • Proteinuria, often nephrotic syndrome, microhematuria, hypertension and progressive loss of renal function
  • May recur after renal transplant
Case reports
  • 3 year old boy with microhematuria and hypertension (Pediatr Nephrol 2002;17:363)
  • 34 year old man with microhematuria and hypertension (Ultrastruct Pathol 2010;34:240)
  • 41 year old man with nephrotic syndrome (Int J Clin Exp Pathol 2009;3:210)
  • Nephrol Dial Transplant 1998;13:2417
    • Microscopic (histologic) description
    • Lobular accentuation of glomeruli with minimal hypercellularity (Nihon Jinzo Gakkai Shi 1999;41:49)
    • Marked enlargement of mesangium and subendothelial space due to massive deposition of fibronectin and fibulin (PAS+, Congo red negative homogenous substance, Mod Pathol 2012;25:709), causing obliteration of capillary lumens
    Microscopic (histologic) images

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    Eosinophilic material in mesangial regions

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    Immunoelectron microscopy with anti-fibronectin

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    Glomeruli show massive deposits of a homogeneous structureless

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    Massive flooding of the glomerulus with fibronectin

    Immunofluorescence description
    • Strongly positive for plasma fibronectin (not cell-derived fibronectin), scanty immunoglobulin or complement deposition
    Electron microscopy description
    • Fibronectin has dense granular appearance with 12 - 16 nm fibrils
    Electron microscopy images

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    Enlargement of glomerular basement membrane and massive mesangial electron-dense deposits

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