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  • 1
    ISSN: 1432-2307
    Keywords: Anionic sites ; Heparan sulfate proteoglycans
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Samoyed hereditary glomerulopathy (SHG) is an X-linked dominant disease characterized by proteinuria and renal failure in affected male dogs. Electron microscopic examination of glomerular capillary basement membranes (GCBM) shows widespread multilaminar splitting of the lamina densa, identical to that in Alport's syndrome. Anionic sites in GCBM of three affected males and five unaffected dogs were labeled using polyethyleneimine to determine whether proteinuria was associated with an alteration in their number. No significant differences were noted in the number of anionic sites in the lamina rara externa, whereas small but statistically significant increases were seen in the number of sites in the lamina rara interna of affected males. In the lamina densa, affected males showed a striking increase in anionic sites, particularly in regions of GCBM which were split. Thus, although proteinuria in some glomerular diseases has been attributed to a reduction in anionic sites in GCBM, this was not so in SHG.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-2307
    Keywords: Hereditary nephritis ; Goodpasture antigen ; Basement membrane
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Samoyed hereditary glomerulopathy (SHG) in dogs has been employed as a model for human hereditary nephritis (HN), since affected dogs and patients show splitting of glomerular capillary basement membranes by electron microscopy (EM) and absent staining of glomerular capillaries for Goodpasture antigen (GPA) by immunofluorescence (IF). EM and IF were used to examine basement membranes (BM) in skin, lung, choroid plexus, lens, retina, and inner ear in SHG. By EM, BM in these tissues appeared similar in affected male, carrier female, and unaffected dogs. By IF, GPA could be detected only in lens capsule, internal limiting membrane of retina and basilar membrane of inner ear of unaffected and carrier female dogs, but not in affected male dogs. However, eye abnormalities and hearing loss were not present in any dogs, in contrast to their frequent occurrence in human HN. Our findings on extra-renal BM in SHG suggest that GPA is not required to maintain normal vision or hearing in affected male dogs and permit a greater understanding of the pathogenesis of human HN.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-198X
    Keywords: Hemolytic-uremic syndrome ; Plasma therapy ; Oncotic pressure ; Tubulo-interstitial disease
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Two children with prototypic hemolytic-uremic syndrome had prolonged acute dialysis-dependent renal failure (74 and 84 days) associated with a state of hyperproteinemia induced by extensive infusion of fresh frozen plasma (283 and 307 units). We believe that the hyperproteinemia prolonged the duration of renal failure. Following cessation of plasma therapy, the hyperproteinemic state reversed, the degree of proteinuria decreased and renal function quickly recovered. Although the pathophysiological mechanism requires further evaluation, we speculate that an alteration in the colloid oncotic pressure and/or aggravation of tubulointerstitial injury due to overload-proteinuria may have increased the duration of renal failure.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1432-198X
    Keywords: Cyclosporin A ; Persistent renal transplant rejection ; Azathioprine ; Renal function
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The effect on renal function and growth of switching from azathioprine to cyclosporin A (CyA) was prospectively evaluated in ten children with persistent renal transplant rejection. Progression of renal insufficiency during CyA therapy was compared with that before using CyA. Prednisone administration decreased after CyA was introduced and although growth retardation persisted, height velocity improved significantly. Renal function stabilized in seven patients treated with CyA for a variable time period, and four of these children remain off dialysis 0.44–1.42 years later. Renal biopsies were obtained in seven children when they were converted from azathioprine to CyA. The response to CyA could not be predicted from renal morphology or clinical features.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1573-6865
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Summary Immunohistochemical staining of tuberculoid and lepromatous leprosy skin lesions was performed using various rabbit antisera. Macrophages in both stained with serum containing antibodies against lysozyme and alpha-1-antitrypsin, while macrophages in lepromatous leprosy also reacted with other antibodies. An immunoglobulin fraction of positive serum stained following pepsin digestion, indicating that reactivity was not Fc dependent. Positive serum contained antibody againstMycobacterium butyricum, which caused macrophage staining, since affinity-purified antibody did not stain and absorption withM. butyricum removed staining. Staining was also produced by serum of subjects with leprosy or a positive tuberculin test. By immunoblotting, the anti-mycobacterial antibody was directed against surface components ofM. butyricum of molecular weights 20 000–70 000. Electron microscopy showedM. leprae in phagolysosomes of macrophages, while immunoelectron microscopy demonstrated labelling along bacterial cell membranes. Therefore, macrophages in lepromatous leprosy skin lesions stain because they containM. leprae, which reacts with antibody to eitherM. leprae, M. tuberculosis or atypical mycobacteria in human serum and with antibody toM. butyricum in serum from rabbits immunized with various antigens and Freund's complete adjuvant. These results indicate that immunohistochemical studies on leprosy are misleading if performed using intact polyclonal immune sera rather than affinity purified or monoclonal antibodies.
    Type of Medium: Electronic Resource
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