Laboratory for Molecular Diagnostics
Center for Nephrology and Metabolic Disorders

Fibronectin glomerulopathy

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.

Differentials

Glomerulopathy with fibronectin deposits type 1 is also a genetic disorder whose locus is not yet identified.

Symptoms

Proteinuria
Proteinuria is the first symptom in patients with fibronectin glomerulopathy.
Hematuria
Hematuria is microscopic and glomerular.
Hypertension
Hypertension accelerates with glomerular damage and renal failure.

Systematic

Hereditary glomerular disease
Fibronectin glomerulopathy
FN1
Glomerulonephritis
Glomerulosclerosis
Lipoprotein glomerulopathy
Myoclonus-nephropathy syndrome
Nephritic syndrome
Nephrotic syndrome

References:

1.

Niimi K et al. (2002) Fibronectin glomerulopathy with nephrotic syndrome in a 3-year-old male.

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2.

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.

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3.

Castelletti F et al. (2008) Mutations in FN1 cause glomerulopathy with fibronectin deposits.

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4.

Assmann KJ et al. (1995) Familial glomerulonephritis characterized by massive deposits of fibronectin.

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5.

Strøm EH et al. (1995) Glomerulopathy associated with predominant fibronectin deposits: a newly recognized hereditary disease.

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6.

OMIM.ORG article

Omim 601894 [^]
7.

Orphanet article

Orphanet ID 84090 [^]
Update: April 29, 2019