膜性肾病合并Alport综合征

Membranous nephropathy associated with Alport syndrome

报道1例中年男性患者临床为肾病综合征伴血压升高,无听力及视力的下降,无肾脏病家族史;肾活检光镜及常规免疫荧光符合肾小球膜性病变,但肾间质见簇状分布的泡沫细胞,进一步行肾组织IV型胶原染色及电镜检查,最终明确合并Alport综合征。

A 38-year-old man presented with nephrotic syndrome and hypertension for five years. He had normal visual acuity and hearing, and denied hereditary nephropathy in his family. Renal biopsy showed non-typical merobranous nephropathy by the light microscope and immunofluoreseence. In addition ,there were several unusual clusters of foam cells in the renal interstitial area. Type IV collagen staining showed that the expression of the alpha3 was faint in the GBM （gloroerular basement membrane） and normal in TBM（tubular basement membrane）. Electron microscope indicated the splitting of the lamina densa of GBM,and subepithelial electron dense deposits. The final diagnosis was Alport syndrome associated with membranous nephropathy.