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  • 1
    Electronic Resource
    Electronic Resource
    An autopsy case of acute porphyria with a decrease of both uroporphyrinogen I synthetase and ferrochelatase activities (1984)
    Springer
    Acta neuropathologica 64 (1984), S. 6-11 
    Details
    ISSN: 1432-0533
    Keywords: Acute porphyria ; Porphyric neuropathy ; Axonal degeneration ; Uroporhyrinogen I synthetase ; Ferrochelatase
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary An autopsy case of a 37-year-old woman with acute porphyria is reported. The patient began to complain of severe menstrual pains, and later developed serious peripheral neuropathy and various autonomic nervous symptoms. The autopsy revealed a marked loss and degeneration of axons and myelin sheaths in the peripheral nervous system (PNS), and prominent central chromatolysis of the spinal anterior horn cells. The predominant process of the peripheral neuropathy appeared to be axonal degeneration. Biochemical analysis showed a marked increase of delta-aminolevulinic acid (ALA), porphobilinogen, uroporphyrin, and coproporphyrin in the urine, and an increase of coproporphyrin and protoporphyrin in the stools and blood. In the analysis of the enzymatic activities of the liver and bone narrow, the activity of ALA synthetase (ALA-S) was markedly increased, and the activities of both uroporphyrinogen I synthetase (URO-S) and ferrochelatase were decreased. It was characteristic in this case that the enzymatic abnormalities found in both acute intermittent porphyria (AIP) and variegate porphyria (VP) coexisted. Biochemical analysis of the sciatic nerve showed an increase of ALA-S activity and a decrease of both URO-S and ALA dehydrase activities. This was the first report that indicated the presence of abnormal activities of the heme biosynthetic enzymes in the peripheral nerves of porphyric patients. The possibility was discussed that these enzymatic abnormalities of the heme biosynthesis in the peripheral nerve itself might be strongly related to the pathogenesis of the porphyric neuropathy.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00695599
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    Paper (German National Licenses)
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  • 2
    Electronic Resource
    Electronic Resource
    Relation of clinical activity of rheumatoid arthritis to immune complexes, complement components and anti-immunoglobulins (1984)
    Springer
    Rheumatology international 4 (1984), S. 159-163 
    Details
    ISSN: 1437-160X
    Keywords: Rheumatoid arthritis ; Immune complexes ; Anti-immunoglobulin ; Complement
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Circulating immune complexes by fluid phase Clq binding assay, complement components and anti-immunoglobulin levels were studied in sera of 35 patients with rheumatoid arthritis (RA). In 23 of the 35 sera (65.7%), circulating immune complexes were positive, and the mean±SD of Clq binding activity (ClqBA), 44.5±19.4%, was significantly high compared to that of healthy persons, 17.4±8.2%. Antigenic determination of complement components revealed that Clq, C3, C5, C9, factor B and Cl esterase inhibitor (CIINH) were significantly high in sera of RA, but C4 and properdin were not. The disease activity correlated with ClqBA, IgG- and IgM-anti-immunoglobulins, C9 and serum IgG. On the other hand, ClqBA correlated with both IgG- and IgM-anti-immunoglobulin levels but not with complement components.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00541207
    Library Location Call Number Volume/Issue/Year Availability
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    Paper (German National Licenses)
    Fulltext
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