Abstract
The pathogenesis of Alport's syndrome is not known; later histologic findings are controversial, and the site of early pathologic change in the kidney has been obscure. Ultrastructural studies of renal tissue from 8 affected patients revealed striking glomerular lesions, even in biopsies with few abnormalities by light microscopy. The most impressive lesions, present in all patients, were thickening of the basement membrane and splitting and splintering of the lamina densa, in a focal and local distribution. Flocculent basement membrane precipitate and extreme thinning of the lamina densa also occurred. Dense deposits were not seen. Focal-local sclerosis was frequently seen by light and electron microscopy. The cause of these lesions is unknown. Absence of dense deposits in glomeruli is consistent with previous immunofluorescent demonstrations of absent immunoglobulins. These studies suggest that glomerular basement membrane may be a site of early pathologic change in at least some patients with Alport's syndrome.
Full text
PDF
Images in this article
Selected References
These references are in PubMed. This may not be the complete list of references from this article.
- Antonovych T. T., Deasy P. F., Tina L. U., D'Albora J. B., Hollerman C. E., Calcagno P. L. Hereditary nephritis. Early clinical, functional, and morphological studies. Pediatr Res. 1969 Nov;3(6):545–556. doi: 10.1203/00006450-196911000-00004. [DOI] [PubMed] [Google Scholar]
- Arakawa M., Kimmelstiel P. Circumferential mesangial interposition. Lab Invest. 1969 Sep;21(3):276–284. [PubMed] [Google Scholar]
- David H., Grossmann P., Marx I., Natusch R. Elektronenmikroskopische Befunde an der Niere beim Alport-Syndrom. Frankf Z Pathol. 1966;76(1):12–20. [PubMed] [Google Scholar]
- Györkey F., Min K. W., Sincovics J. G., Györkey P. Systemic lupus erythematosus and myxovirus. N Engl J Med. 1969 Feb 6;280(6):333–333. doi: 10.1056/nejm196902062800620. [DOI] [PubMed] [Google Scholar]
- Kaufman D. B., McIntosh R. M., Smith F. G., Jr, Vernier R. L. Diffuse familial nephropathy: a clinicopathological study. J Pediatr. 1970 Jul;77(1):37–47. doi: 10.1016/s0022-3476(70)80042-7. [DOI] [PubMed] [Google Scholar]
- Krickstein H. I., Gloor F. J., Balogh K., Jr Renal pathology in hereditary nephritis with nerve deafness. Arch Pathol. 1966 Dec;82(6):506–517. [PubMed] [Google Scholar]
- Pincus T., Blacklow N. R., Grimley P. M., Bellanti J. A. Glomerular microtubules of systemic lupus erythematosus. Lancet. 1970 Nov 21;2(7682):1058–1061. doi: 10.1016/s0140-6736(70)90287-4. [DOI] [PubMed] [Google Scholar]
- REYERSBACH G. C., BUTLER A. M. Congenital hereditary hematuria. N Engl J Med. 1954 Sep 2;251(10):377–380. doi: 10.1056/NEJM195409022511003. [DOI] [PubMed] [Google Scholar]
- Spear G. S., Whitworth J. M., Konigsmark B. W. Hereditary nephritis with nerve deafness. Immunofluorescent studies on the kidney, with a consideration of discordant immunoglobulin-complement immunofluorescent reactions. Am J Med. 1970 Jul;49(1):52–63. doi: 10.1016/s0002-9343(70)80113-9. [DOI] [PubMed] [Google Scholar]