Abstract
Fibronectin glomerulopathy is an inherited non-immune-mediated glomerulopathy associated with the massive deposition of fibronectin. It presents with proteinuria, microscopic hematuria, and hypertension that lead to end-stage renal failure in the second to sixth decade of life. A 34-year-old male was referred with proteinuria (6 g/day), microscopic hematuria and hypertension (220/130 mmHg). Renal biopsy specimen showed massive deposits of fibronectin in the mesangium and subendothelial spaces. After 20 months of multidrug treatment his renal function is stable.
MeSH terms
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Adult
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Fibronectins / metabolism*
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Glomerular Mesangium / metabolism
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Glomerular Mesangium / ultrastructure
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Hematuria / drug therapy
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Hematuria / genetics
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Hematuria / pathology
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Humans
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Hypertension / drug therapy
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Hypertension / genetics
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Hypertension / pathology
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Kidney Diseases / drug therapy
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Kidney Diseases / genetics
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Kidney Diseases / metabolism
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Kidney Diseases / pathology*
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Kidney Glomerulus / metabolism
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Kidney Glomerulus / pathology*
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Male
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Microscopy, Electron, Transmission
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Proteinuria / drug therapy
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Proteinuria / genetics
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Proteinuria / pathology
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Treatment Outcome