Skip to main content
Log in

Split and extremely thin glomerular basement membranes in hereditary nephropathy (Alport's syndrome)

  • Published:
Virchows Archiv A Aims and scope Submit manuscript

Summary

Alterations of the glomerular basement membrane are the earliest ultrastructurally demonstrable lesions in the course of hereditary nephropathy. The basement membranes may be either focally or diffusely thickened. They may be (a) homogeneously broadened, (b) show a typically split lamina densa, or (c) show a focally or diffusely thinned lamina densa layer. The latter may be one third or one half of the normal thickness. These findings provide further evidence for the hypothesis of basement membrane alterations as the basic lesion in hereditary nephritis.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

References

  • Bergstrand, A., Bucht, H.: Anatomy of the Glomerulus as Observed in Biopsy Material From Young and Healthy Human Subjects. Z. Zellforsch. 48, 51–73 (1958)

    Google Scholar 

  • Bloom, P. M., Hartmann, J. F., Vernier, R. L.: An electron microscopic evaluation of the width of normal glomerular basement membrane in man at various ages. Anat. Rec. 133, 251–252 (1959)

    Google Scholar 

  • Buchem, F. S. P. van, Beetstra, A.: Hereditary renal disease associated with deafness-Alport's syndrome. Acta med. scnad. 179, 319–328 (1966)

    Google Scholar 

  • Churg, J., Grishman, E., Goldstein, M. H., Yunis, S. L., Porush, J. G.: Idiopathic nephrotic syndrome in adults. New Engl. J. Med. 272, 165–174 (1965)

    Google Scholar 

  • Churg, J., Sherman, R. L.: Pathologic Characteristics of hereditary nephritis. Arch. Path. 95, 374–379 (1973)

    Google Scholar 

  • David, H., Grossmann, P., Marx, I., Natusch, R.: Elektronenmikroskopische Befunde an der Niere beim Alport-Syndrom. Frankfurter Z. Path. 76, 12–20 (1966)

    Google Scholar 

  • Farquhar, M. G., Vernier, R. L., Good, R. A.: Studies on Familial Nephrosis. II. Glomerular Changes Observed with the Electron Microscope. Amer. J. Path. 33, 791–816 (1957)

    Google Scholar 

  • Farquhar, M. G., Vernier, R. L., Good, R. A.: An Electron Microscope Study of the Glomerulus in Nephrosis, Glomerulonephritis, and Lupus Erythematosus. J. exp. Med. 106, 649–660 (1958)

    Google Scholar 

  • Grünfeld, J.-P., Bois, E. P., Hinglais, N.: Progressive and nonprogressive hereditary chronic nephritis. Kidney Internat. 4, 216–228 (1973)

    Google Scholar 

  • Hinglais, N., Grünfeld, J.-P., Bois, E. P.: Characteristic ultrastructural lesions of the glomerular basement membrane in progressive hereditary nephritis (Alport's syndrome). Lab. Invest. 27, 473–487 (1972)

    Google Scholar 

  • Jørgensen, F., Bentzon, M. W.: The ultrastructure of the normal human glomerulus. Thickness of glomerular basement membrane. Lab. Invest. 18, 42–48 (1968)

    Google Scholar 

  • Kaufman, D. B., McIntosh, R. M., Smith, F. G., Vernier, R. L.: Diffuse familial nephropathy: A clinicopathological study. J. Pediat. 77, 37–47 (1970)

    Google Scholar 

  • Kinoshita, Y., Osawa, G., Morita, T., Kobayashi, N., Wada, J., Ebe, T., Watanabe, M., Murohashi, K., Murayama, M.: Hereditary chronic nephritis (Alport) complicated by nephrotic syndrome. Acta med. biol. (Niigata) 17, 101–117 (1969)

    Google Scholar 

  • Langer, K. H., Thoenes, W.: Alport-Syndrom — Licht- und elektronenmikroskopische Nierenbefunde im Frühstadium. Verh. dtsch. ges. Path. 55, 497–501 (1971)

    Google Scholar 

  • Osawa, G., Kimmelstiel, P., Seiling, V.: Thickness of glomerular basement membranes. Amer. J. clin. Path. 45, 7 (1966)

    Google Scholar 

  • Østerby Hansen, R.: A quantitative estimate of the peripheral glomerular basement membrane in recent juvenile diabetes. Diabetologica 1, 97–100, (1965)

    Google Scholar 

  • Spear, G. S.: Alport's syndrome: A consideration of pathogenesis. Clin. Nephrol. 1, 336–337 (1973)

    Google Scholar 

  • Spear, G. S., Slusser, R. J.: Alport's syndrome. Emphasizing Electron Microscopic studies of the Glomerulus. Amer. J. Path. 69, 213–220 (1972)

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Additional information

The excellent technical assistance of Mrs. B. Agricola is gratefully acknowledged. Expert photographic work was provided by Mrs. D. Wenz.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Rumpelt, H.J., Langer, K.H., Schärer, K. et al. Split and extremely thin glomerular basement membranes in hereditary nephropathy (Alport's syndrome). Virchows Arch. A Path. Anat. and Histol. 364, 225–233 (1974). https://doi.org/10.1007/BF00433075

Download citation

  • Received:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF00433075

Keywords

Navigation