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. 1971 Nov;68(11):2725–2729. doi: 10.1073/pnas.68.11.2725

Repression of the Overproduction of Porphyrin Precursors in Acute Intermittent Porphyria by Intravenous Infusions of Hematin

H L Bonkowsky 1,2, D P Tschudy 1,2, A Collins 1,2, J Doherty 1,2, I Bossenmaier 1,2, R Cardinal 1,2, C J Watson 1,2
PMCID: PMC389510  PMID: 5288250

Abstract

In a patient with a severe attack of acute intermittent porphyria, hematin given intravenously caused marked diminution of serum δ-aminolevulinic acid and porphobilinogen. The decline of aminolevulinate was more rapid than that of porphobilinoge. After 2 days of hematin administration, about 5 days were required for δ-aminolevulinic acid, and 11 days for porphobilinogen to return to the concentrations that were detected before treatment. Urinary excretion of both compounds also decreased after hematin administration. Considerable amounts of porphobilinogen were also found in the cerebrospinal fluid of the patient.

Keywords: δ-aminolevulinic acid, porphobilinogen, heme, cerebrospinal fluid

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

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