Familial glomerular nephritis with fibronectin deposits is an autosomal dominant disorder which is characterized by enlarged glomeruli with mesangial and subendothelial fibrillary deposits (fibronectin). The clinical picture includes proteinuria, glomerular hematuria, and hypertension. The glomerular damage eventually leads to end stage renal disease which is reached in the second to fifth decade.
Glomerulopathy with fibronectin deposits type 1 is also a genetic disorder whose locus is not yet identified.
Proteinuria is the first symptom in patients with fibronectin glomerulopathy.
Hematuria is microscopic and glomerular.
Hypertension accelerates with glomerular damage and renal failure.
|Hereditary glomerular disease|
Niimi K et al. (2002) Fibronectin glomerulopathy with nephrotic syndrome in a 3-year-old male.[^]
Mazzucco G et al. (1992) Glomerulonephritis with organized deposits: a mesangiopathic, not immune complex-mediated disease? A pathologic study of two cases in the same family.[^]
Castelletti F et al. (2008) Mutations in FN1 cause glomerulopathy with fibronectin deposits.[^]
Assmann KJ et al. (1995) Familial glomerulonephritis characterized by massive deposits of fibronectin.[^]
Strøm EH et al. (1995) Glomerulopathy associated with predominant fibronectin deposits: a newly recognized hereditary disease.[^]
OMIM.ORG articleOmim 601894 [^]
Orphanet articleOrphanet ID 84090 [^]