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The Effect of Hematin in “Inducible” Hepatic Porphyria

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Diagnosis and Therapy of Porphyrias and Lead Intoxication
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Abstract

As discussed elsewhere in greater detail (15), those forms of hepatic porphyria in which various inducing,factors may precipitate a lifethreatening acute neurologic relapse, are appropriately grouped under the term “inducible.” This relates to induction of 6-aminolevulinic acid synthetase (ALA-S), the rate-limiting enzyme of porphyrin biosynthesis. The term embraces acute intermittent porphyria (AIP), porphyria variegata (PV), and hereditary coproporphyria (HCP), but porphyria cutanea tarda is not included, as it is doubtful whether ALA-S is significantly induced in this form (10); there may be induction, but at a very low level (5).

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References

  1. Bosch, E.P., Pierach, C.A., Bossenmaier, I., Cardinal, R., Thorson, M.: Effect of hematin in porphyric neuropathy. Neurology 27, 1053–1056 (1977)

    PubMed  CAS  Google Scholar 

  2. Burnham, B.F., Lascelles, J.: Control of porphyrin biosynthesis through a negative feedback mechanism. Biochem. J. 87, 462–472 (1963)

    PubMed  CAS  Google Scholar 

  3. Dhar, G.J., Bossenmaier, I., Petryka, Z.J., Cardinal, R., Watson, C.J.: Effects of hematin in hepatic porphyria. Further studies. Ann. Intern. Med. 83, 20–30 (1975)

    PubMed  CAS  Google Scholar 

  4. Granick, S.: The induction in vitro of the synthesis of δ-aminolevulinic acid synthetase in chemical porphyria: A response to certain drugs, sex hormones and foreign chemicals. J. Biol. Chem. 241, 1359–1375 (1966)

    PubMed  CAS  Google Scholar 

  5. Kaufman, L., Marver, H.S.: Biochemical defects in two types of human hepatic porphyria. N. Engl. J. Med. 283, 954–958 (1970)

    Article  PubMed  CAS  Google Scholar 

  6. Lamon, J., With, T.K., Redeker, A.G.: The Hoesch test: Bedside screening for urinary porphobilinogen in patients with suspected porphyria. Clin. Chem. 20, 1438–1440 (1974)

    PubMed  CAS  Google Scholar 

  7. Meyer, U.A., Schmid, R.: Intermittent acute porphyria: The enzymatic defect. In: Brain Dysfunction in Metabolic Disorders. Plum, F.(ed.).(Res. Publ. Assn. Nerv. Ment. Dis.). New York: Raven Press 1974, Vol. LIII, pp. 211–224

    Google Scholar 

  8. Peterson, A., Bossenmaier, I., Cardinal, R., Watson, C.J.: Hematin treatment of acute porphyria. Early remission of an almost fatal relapse. J.A.M.A. 235, 520–522 (1976)

    Article  PubMed  CAS  Google Scholar 

  9. Pierach, C.A., Cardinal, R., Bossenmaier, I., Watson, C.J.: Comparison of the Hoesch and the Watson-Schwartz tests for urinary porphobilinogen. Submitted to Clin. Chem. 23, 1666–1668 (1977)

    CAS  Google Scholar 

  10. Pimstone, N.R., Blekkenhorst, G., Eales, L.: Enzymatic defects in hepatic porphyria. Enzyme 16, 354–366 (1973)

    PubMed  CAS  Google Scholar 

  11. Schmid, R.: Discussion of paper by Sassa et al. , No. 6 in Program of Meeting of Assn. of Am. Phys., April 30, 1977, p. 130

    Google Scholar 

  12. Shanley, B.C., Neethling, A.C., Percy, V.A., Carstens, M.: Neurochemical aspects of porphyria. Studies on the possible neurotoxicity of delta aminolaevulinic acid. S. Afr. Med. J. 49, 576–580 (1975)

    PubMed  CAS  Google Scholar 

  13. Taddeini, L., Watson, C.J.: The clinical porphyrias. Sem. Hematol. 5, 335–369 (1968)

    CAS  Google Scholar 

  14. Tschudy, D.P., Valsamis, M., Magnussen, C.R.: Acute intermittent porphyria: Clinical and selected research aspects. Ann. Intern. Med. 83, 851–864 (1975)

    PubMed  CAS  Google Scholar 

  15. Watson, C.J., Pierach, C.A., Bossenmaier, I., Cardinal, R.: Use of hematin in the acute attack of the “inducible” hepatic porphyrias. Adv. Intern. Med. 23, 265-286 (1978). Stollerman, G.H. (ed.). Year Book Medical Publishers Inc.

    Google Scholar 

  16. Watson, C.J., Taddeini, L., Bossenmaier, I.: Present status of the Ehrlich aldehyde reaction for urinaryporphobilinogen. J.A.M.A. 190, 501–504 (1964)

    PubMed  CAS  Google Scholar 

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Watson, C.J., Pierach, C.A., Bossenmaier, I., Cardinal, R. (1978). The Effect of Hematin in “Inducible” Hepatic Porphyria. In: Doss, M. (eds) Diagnosis and Therapy of Porphyrias and Lead Intoxication. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-67002-2_14

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  • DOI: https://doi.org/10.1007/978-3-642-67002-2_14

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-540-08863-9

  • Online ISBN: 978-3-642-67002-2

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