Fig. 2
From: Co-existence of Alport syndrome and C3 glomerulonephritis in a proband with family history
The pathological features of kidney and skin samples in the proband (III:1), his mother (II:1), and His affected aunt’s daughter (III:2). Light micrograph: the proband shows segmental sclerosis (a, PAS × 200), crescent formation (b, MA × 200), diffuse and global mild-moderate mesangial proliferation (c, HE × 200), and interstitial fibrosis/tubular atrophy containing lipid-laden foam cells (d, MA × 200). Immunofluorescent stain: the proband reveals partial loss for the collagen IV a5 chain in GBM and complete absence in Bowman’s capsule and distal tubule basement membrane (e, IF × 400), compared with control (f, IF × 400). A skin biopsy of his mother demonstrates segmental absence of the collagen IV a5 chain within the epidermal basement membrane (g, IF × 200), compared with control (h, IF × 200). Electron micrograph: segmental uneven thickness with unsmooth spiculation of the dense layer in the GBM is found in the proband (i, × 6000), and uneven thickness with a laminated appearance is shown in his aunt’s daughter (j, × 15,000). Remarkably, a marked C3 staining is shown in the proband to locate along the mesangium by immunofluorescence analysis (k, IF × 200) with the corresponding electron-dense deposits under the electron microscopy (l, × 10,000)