Kidney biopsies from probate GN29 (A–G) and
his mother, GN29M (H–O), showed remarkably
similar light, immunofluorescence, and ultrastructural findings. The
characteristic histological lesion consisted of mesangial hypercellularity with
thickened, eosinophil-rich segments of GBM (A and H).
The affected glomerular segments were PAS positive and reacted to trichrome and
Jones methenamine-silver stain (B, C, and
I–K). The main immunofluorescence
findings were prominent and diffuse C3 deposits, which were granular in some
glomerular areas (D and L). IgG was absent from these
deposits (F and M), although local deposits of IgM were
observed in GN29 (E). Both biopsies showed similar ultrastructural
alterations (G, N, and O) consisting of the
presence of ribbon-like, osmiophilic deposits in the GBM (arrows); these
electron-dense deposits were also evident in the mesangial matrix (asterisks).
Original magnification, ×400
(A–C, H, and
I); ×200 (D–F);
×1,600 (G); ×600 (J and
K); ×100 (L and M);
×2,950 (N); ×8,900 (O).