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Pathology General 2d933a13

A patient with fibronectin mesangial deposits was diagnosed having fibronectin nephropathy. Which of the following is NOT true about fibronectin nephropathy?

A
Autosomal recessive inheritance
B
PAS positivity is seen in glomerulus and mesangial basement membrane
C
No deposition of any immune complexes or complement components on immunofluorescence microscopy
D
Glomerular enlargement with fibronectin deposition in mesangium and basement membrane is characteristic
High-Yield Explanation
Fibronectin nephropathy: It is an autosomal dominant disease, presents with proteinuria and slowly progressive loss of renal function. The principle light microscopic change is glomerular enlargement and lobulation resulting from PAS and trichrome-positive mesangial deposits and mild mesangial proliferation. By immunofluorescence microscopy, the glomeruli do not consistently stain for immunoglobulin or complement components. The most consistent ultrastructural finding is large (giant), mesangial and subendothelial electron-dense deposits that mirror the location of the PAS-positive, fibronectin deposits. Ref: Heptinstall's Pathology of the Kidney By J. Charles Jennette, Robe H. Heptinstall, 2007, Page 931, 932, 933.

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