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. https://www.pathologyoutlines.com/topic/kidneyfibronectinglomerulopathy.html. Accessed December 4th, 2024.
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
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