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

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.