[3 cases of Alport's syndrome with different clinical and morphologic manifestations]

Of 350 renal biopsies referred to the Pathologic Anatomy Department Academy of Medicine in Lódź between 1982 and 1986 the authors recognized three cases of Alport's syndrome. The diagnosis was made on the basis of light microscopy (HE, PTAH, silver staining according to Jones, PAS with alcian blue) and immunofluorescence reactions with the sera against IgA, IgG, IgM and fraction C3 of the complement. In two cases electron microscopy was performed additionally. In two patients clinical data pointed to the diagnosis of Alport's syndrome (hematuria, partial deafness, mental retardation, progressive nephropathy). In the third patient Alport's syndrome was not suspected clinically and the diagnosis was established only on the basis of electron microscopic studies. Light microscopy detected cellular proliferation and increase of the mesangium matrix as well as foci of foamy cells in the renal stroma in one case. Immunofluorescence studies were not significant for the diagnosis whereas electron microscopy examinations were decisive. We found characteristic diffused or focal widening of basal membranes of the glomerular vascular loops with the dense lamina splitting, as well as focal thinning of the basal membranes. The intensity and extent of submicroscopic changes varied in the cases described.