Glomerulonephritis with focal and segmental distribution of glomerular basement membrane antigen(s)

In this report, the authors describe a case of a 2-year, 11-month-old girl with glomerulonephritis and no family history of renal diseases and deafness. Immunofluorescent studies in the renal biopsy specimens with the use of anti-sera against human glomerular basement membrane (GBM) and P3 antigen (prepared from bovine GBM and inducible of Steblay's type nephritis in rats) demonstrated focal and segmental distribution of the GBM antigen(s). Electron microscopic examination revealed splitting and thinning of the GBM. Indirect immunofluorescence showed that there was no binding of Goodpasture's anti-GBM antibodies to the glomeruli. These findings are similar to those in patients with hereditary nephritis. The immunofluorescent examination of the fixation of the various anti-sera, including anti-types IV and V collagens, laminin, fibronectin, and actomyosin sera on the GBM, revealed normal reactivity. The abnormalities observed in this case may be a part of the spectrum of primary GBM defects.