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  • Rigidität der Erythrozyten  (1)
  • Uroporphyrinogen decarboxylase  (1)
  • 1
    Electronic Resource
    Electronic Resource
    Studies on the toxicology of hexachlorobenzene (1983)
    Springer
    Archives of toxicology 52 (1983), S. 13-22 
    Details
    ISSN: 1432-0738
    Keywords: Hexachlorobenzene ; Female rats ; Porphyrin accumulation ; Porphyrin elimination ; Uroporphyrinogen decarboxylase
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The time course of changes in the hepatic metabolism of porphyrins under the influence of hexachlorobenzene has been studied. For this purpose female Wistar rats were given 100 mg hexachlorobenzene/kg body weight every other day for 6 weeks. They were subsequently kept for a further 18 months under the same conditions as during treatment but without additional hexachlorobenzene. The disturbance of porphyrin biosynthesis in the liver caused by hexachlorobenzene occurs in four phases. The duration of the first two phases can be influenced in an arbitrary way because hexachlorobenzene is continually administered during their course. During the first phase an almost constant content of hexachlorobenzene and a gradual decrease of uroporphyrinogen decarboxylase activity is achieved. In the second phase a noticeable accumulation of porphyrins and a practically complete inhibition of decarboxylase activity are conspicuous. In the third phase, which occurs after hexachlorobenzene administration has been discontinued, a further accumulation of porphyrins and a continuing inhibition of uroporphyrinogen decarboxylase activity can be seen, even after extensive elimination of hexachlorobenzene. During the fourth phase a decrease in porphyrin content and a return of decarboxylase activity are clearly observable. On the basis of these results, it is necessary to reflect on the causal connection between the previously described individual biochemical changes in the liver, and the various phases of porphyria brought about by hexachlorobenzene. Indication is given of a process in the liver brought on primarily by hexachlorobenzene but able to sustain itself after elimination of the inducing agent.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00317978
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  • 2
    Electronic Resource
    Electronic Resource
    Porphyria erythropoetica congenita Günther und Chloroquin (1978)
    Springer
    Journal of molecular medicine 56 (1978), S. 623-624 
    Details
    ISSN: 1432-1440
    Keywords: Porphyria ; Morbus Günther ; Chloroquine ; Erythrocytes rigidity ; Porphyrie ; Morbus Günther ; Chloroquin ; Rigidität der Erythrozyten
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Bei einem 6jährigen Jungen mit Porphyria congenita Günther kam es unter Chloroquin in niedriger Dosierung zu einem vorübergehenden erheblichen Anstieg der Porphyrin-Ausscheidung mit dem Urin und vor allem zu einer weitgehenden Normalisierung der vorher pathologischen Rigidität der Erythrozyten. Da eine solche Rigidität der Erythrozyten bei der für diese Porphyrie typischen Hämolyse eine wesentliche Rolle spielen dürfte, könnte die Chloroquin-Anwendung therapeutisch wertvoll sein.
    Notes: Summary Treatment of a six-years old boy with porphyria congenita (Günther) by small amounts of chloroquine was followed by a sharp but transient increase of the urinary excretion of porphyrins. Moreover, a nearly complete normalization of the previously observed rigidity of the erythrocytes occurred. With respect to the possibility that this elevated rigidity plays an important role for the typical hemolysis connected with this porphyria, the application of chloroquine could be of therapeutic value for this disease.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01477011
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    Paper (German National Licenses)
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