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Immunofluorescence: Negative Electron microscopy: Uniform effacement and “fusion” of foot processes of visceral epithelial cells. Selective Proteinuria. Membranous nephropathy. Major cause of nephrotic syndrome in adults. About 85% are truly idiopathic. Immune mediated. Role of C5b-9 (MAC)
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Immunofluorescence: Negative Electron microscopy: Uniform effacement and “fusion” of foot processes of visceral epithelial cells Selective Proteinuria
Membranous nephropathy Major cause of nephrotic syndrome in adults. About 85% are truly idiopathic. Immune mediated. Role of C5b-9 (MAC) Grossly kidney is swollen and pale. Microscopy: Uniform diffuse basement membrane thickening. Immunofluorescence: Uniform granular IgG along basement membrane EM: Dense irregular sub-epithelial deposits
Glomerular Diseases
Glomerular diseases Primary Secondary Acute diffuse proliferative GN Diabetes Crescentic GN (RPGN) Hypertension Membranous SLE Minimal change Amyloidosis Focal glomerular disease including FSGS and IgA Vasculitis Chronic glomerulonephritis Bacterial endocarditis Heriditary: Alport’s disease, Fabry’s disease
Primary glomerulonephritis Acute (diffuse) proliferative g n* Crescentic (RPGN)* Membrano-proliferative glomerulonephritis*# Focal proliferative g n and Ig A nephropathy *# Membranous glomerulonephritis # Minimal change disease # Focal segmental glomerulosclerosis # Chronic glomerulonephritis * = nephritic, # = nephrotic
Minimal change disease Most common cause of nephrotic syndrome in children Usually shows dramatic response to steroid treatment ? related to disorders of cell mediated (T cell) immunity Grossly kidney appears normal Microscopy: glomeruli, tubules and interstitium - normal except for occasional lipid vacuoles in tubules