Kidney nontumor
Other hereditary renal disease
Fibronectin glomerulopathy
Copyright: 2002-2020, PathologyOutlines.com, Inc.
PubMed Search: Fibronectin glomerulopathy [title]
Other hereditary renal disease
Fibronectin glomerulopathy
Author: Nikhil Sangle, M.D.
Topic Completed: 6 February 2017
Minor changes: 11 February 2020
Copyright: 2002-2020, PathologyOutlines.com, Inc.
PubMed Search: Fibronectin glomerulopathy [title]
Table of Contents
Definition / general | Case reports | Microscopic (histologic) description | Microscopic (histologic) images | Immunofluorescence description | Electron microscopy description | Electron microscopy imagesCite this page: Sangle N. Fibronectin glomerulopathy. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/kidneyfibronectinglomerulopathy.html. Accessed May 28th, 2020.
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
Images hosted on other servers:
Eosinophilic material in mesangial regions
Immunoelectron microscopy with anti-fibronectin
Glomeruli show massive deposits of a homogeneous structureless
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
Images hosted on other servers:
Enlargement of glomerular basement membrane and massive mesangial electron-dense deposits
