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  • 1990-1994  (1)
  • 1980-1984  (1)
  • Henoch-Schönlein purpura  (1)
  • Lactic acidosis  (1)
  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Archives of dermatological research 282 (1990), S. 183-187 
    ISSN: 1432-069X
    Keywords: Henoch-Schönlein purpura ; Complement ; Cytolysis ; Endothelial cell damage
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The present study using direct immunofluorescence with monoclonal antibodies to C5b-9 complex-related antigens was undertaken to determine whether complement activation in Henoch-Schönlein purpura (HSP) causes assembly of the membrane attack complex of complement (MAC) in skin and nephritis lesions. The deposition of C5, C6, C7, C8, C9, and C5b-9 neoantigens was noted in the vascular walls of papillary dermis and/or subpapillary dermal plexus of the vessels in 11 out of 15 patients with HSP. Their presence in vessel walls indicates complement activation which leads to terminal complement activation. There were small deposits of S protein at the same sites in three of the 11 skin specimens. Thus, the majority of C5b-9 demonstrated in HSP skin was the cytolytically active C5b-9 complex, MAC. Granular deposits of C5b-9 related antigens without S protein were also found in the capillary walls and mesangium of the glomeruli of two out of four specimens from patients with HSP nephritis; in the other two S protein was colocalized with the deposition of C5b-9. The results of the present study indicate that complement activation leading to generation of MAC may possibly be involved in the pathogenesis of vascular injury in a significantly large number of skin lesions and of HSP nephritis.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-1076
    Keywords: Mitochondrial myopathic symdrome ; Lactic acidosis ; Endothelium ; Ultrastructural study
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The quadriceps femoris muscle and the muscularis mucosae of the rectum from two children with mitochondrial myopathic syndrome associated with lactic acidosis were studied by electron microscopy. Striking morphological abnormalities of mitochondria were noted not only in the skeletal but also in the smooth muscle cells. Endothelial cells of blood capillaries distributed in these affected muscles were so greatly swollen that the capillary lumen was almost occluded. In contrast, surface epithelial and glandular epithelial cells of the rectum contained normal mitochondria, and fenestrated capillaries in the propria mucosae remained intact. Long-term ischemia resulting from occlusive changes of the capillary wall may be responsible for the mitochondrial alterations of muscle cells.
    Type of Medium: Electronic Resource
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