Histology of Alport syndrome -a) Foamy cells in interstitiumb) Foamy cells in tubular epithelial cellsc) Thickening of GBM > 100 nmd) Thinning of GBM < 100 nme) Intimal proliferation
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Description: Histological characteristics of Alport’s syndrome:
• Diffuse basement membrane thinning • Vascular sclerosis
• Foam cells in interstitium • Tubular atrophy
• In advanced stage there is focal or global glomerulosclerosis • Interstitial fibrosis
On electron microscopy (diagnostic of this disorder), GBM shows
• Irregular foci of thickening alternating with thinning
• Pronounced splitting and lamination of lamina densa (basket weave appearance).
• Info: Immunohistochemistry shows failure to stain a-3, 4, 5 collagen.
Category:
Pathology
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