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Focal and segmental sclerosing glomerulopathy (-nephritis)

A pathomorphological study

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Summary

10 out of 44 renal biopsy specimens obtained from patients with focal and segmental sclerosing glomerulopathy (-nephritis) (focal sclerosing lesion, sclerose s. hyalinose segmentaire et focale) were examined with the electron microscope and 11 by immunofluorescence microscopy. Particularly ultrastructural alterations are described in detail. A nephrotic syndrome was observed in 33 (75%), proteinuria in 11 (25%), and erythrocyturia in 21 (48%) patients. As the characteristic glomerular lesion a progressive increase in mesangial matrix material (sclerosis) is found which in the beginning concerns only few glomeruli and within these only a portion of the capillary tuft. The remaining glomeruli histologically show minimal changes. Electron microscopy, however, reveals these glomeruli as pathologically altered, too, indicating that the underlying mechanism is a diffuse one. Progression of sclerosing processes finally results in complete sclerosis of more and more capillary loops and glomeruli, respectively. Lightmicroscopically identifiable PAS-positive hyaline deposits were present in about 80%, thus representing an important diagnostic tool. Electron dense deposits, identical with typical immune deposits, are a regular finding in sclerosing areas and very rarely occur in minimally changed glomeruli, too. Immunofluorescence microscopy reveals a corresponding segmental deposition pattern of mostly IgM and C3 globulins. These findings are discussed with regard to an immune pathogenesis of the focal and segmental lesion.

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Authors and Affiliations

  1. Institut für Cytobiologie und Cytopathologie der Universität Marburg, Federal Republic of Germany

    H. J. Rumpelt & W. Thoenes

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  1. H. J. Rumpelt
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  2. W. Thoenes
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Additional information

This conception was advanced by Dr. K. Thurau on the 1. Symposion of Nephrology Hannover 1973.

The authors are greatly indebted to Mrs. B. Agricola and Miss L. Meyer for their excellent technical assistance.

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Rumpelt, H.J., Thoenes, W. Focal and segmental sclerosing glomerulopathy (-nephritis). Virchows Arch. A Path. Anat. and Histol. 362, 265–282 (1974). https://doi.org/10.1007/BF00427076

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