Thirty-three patients with chronic hereditary nephritis, obtained from 23 unrelated families, were evaluated with respect to clinicopathologic features. Renal tissue was examined by light microscopy in 25 cases, immunofluorescence in 19 cases, and electron microscopy in 16 cases. The light microscopic findings varied, and foam cells were present in only 4 cases. Immunofluorescence was negative in all but 4 cases, and in these the immunomicroscopic pattern was compatible with the findings of end stage glomeruli and hyaline arteriolar sclerosis. Although electron microscopy uniformly showed marked thinning or splitting of the glomerular basement membrane, parallel splitting of the glomerular basement membrane with interposition of electron dense granular particles was seen in only 8 cases. Association of glomerular basement membrane splitting with granular particles was observed in 4 of 6 patients with IgA nephropathy, in 2 patients with benign familial hematuria, and in a normal kidney donor. Eleven patients, 7 men and 4 women, had chronic renal failure requiring dialysis. Of 5 patients who received renal allografts, 3 are alive, with post-transplant survival ranging from 24 to 70 mth. The other 2 died of septicemia.
ASJC Scopus subject areas
- Pathology and Forensic Medicine