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  • 1990-1994
  • 1980-1984  (2)
  • Copper deficiency  (1)
  • Lactic acidosis  (1)
  • 1
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 134 (1980), S. 121-124 
    ISSN: 1432-1076
    Keywords: Copper deficiency ; Erythrocyte superoxide dismutase
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract In a patient with typical copper deficiency, we found superoxide dismutase (SOD, 1.15.1.1) activity and copper in erythrocytes to be decreased to 52% and 46% of age-matched controls, respectively. However, these were not so markedly depleted as plasma copper (17%) or ceruloplasmin (20%). After copper replacement therapy, erythrocyte copper and SOD activity gradually returned to the control levels. Although certain abnormalities reported in copper deficiency in animals were expected, osmotic fragility of erythrocytes of the patient was normal and damage of the liver and heart were not a feature as far as could be determined by electrocardiography and routine laboratory examinations. Probably a decrease of SOD to this extent is not severe enough to lead to superoxide-induced damage.
    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
    Library Location Call Number Volume/Issue/Year Availability
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