Glomerular anionic charge in congenital nephrotic syndrome of the Finnish type
| Autor: | Harry Holthöfer, Päivi Ljungberg, Juhani Rapola, Hannu Jalanko, Christer Holmberg |
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| Rok vydání: | 1995 |
| Předmět: |
Anions
Pathology medicine.medical_specialty Ruthenium red Nephrotic Syndrome Kidney Glomerulus 030232 urology & nephrology Lamina Rara Externa Stain Basement Membrane 03 medical and health sciences chemistry.chemical_compound 0302 clinical medicine medicine Humans Congenital nephrotic syndrome 030304 developmental biology Basement membrane 0303 health sciences Kidney Chemistry Glomerular basement membrane Infant Cell Biology Anatomy medicine.disease Staining Microscopy Electron medicine.anatomical_structure Child Preschool Microscopy Electron Scanning |
| Zdroj: | The Histochemical journal. 27(7) |
| ISSN: | 0018-2214 |
| Popis: | Decrease of the anionic charge of the glomerular basement membrane and especially the reduced amount of heparan sulphate proteoglycan in the lamina rara externa has been suggested to be the basic pathogenetic defect in congenital nephrotic syndrome. In the present study the anionic charge of glomeruli was examined in the congenital nephrotic syndrome of the Finnish type and in controls using cationic stains (polyethyleneimine, Ruthenium Red) in electron microscopy. Chondroitinase and heparinase treatments were used to characterize further the anionic elements detected. Scanning electron microscopy (SEM) was used in addition to transmission electron microscopy (TEM) to examine the tridimensional structure and secondary changes of podocytes in this syndrome. The number (mean +/- SD) of polyethyleneimine granules per 1 micron length of lamina rara externa of the glomerular basement membrane was 24.9 +/- 4.5 in control and 23.2 +/- 4.3 [corrected] in congenital nephrotic syndrome subjects. The Ruthenium Red staining pattern was closely similar in syndrome and control kidneys. The granules evident after staining with either cationic stain were seen after chondroitinase but not after heparinase treatment in control as well as in syndrome patient kidney samples. No denuded areas of basement membrane in 42 glomeruli from four syndrome patients were found in SEM. In conclusion, the amount of anionic sites in the lamina rara externa as detected by either cationic stain was comparable to controls. These results do not support the hypothesis of decreased anionic sites in the lamina rara externa of the glomerular basement membrane in congenital nephrotic syndrome of the Finnish type. |
| Databáze: | OpenAIRE |
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