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PMID: 5288250 Published · ppublish English Journal Article

Repression of the overproduction of porphyrin precursors in acute intermittent porphyria by intravenous infusions of hematin.

Proceedings of the National Academy of Sciences of the United States of America ·Vol. 68 ·No. 11 ·1971-11-00 ·Pages 2725-9

Bonkowsky HL, Tschudy DP, Collins A, Doherty J, Bossenmaier I, Cardinal R, Watson CJ

Abstract

In a patient with a severe attack of acute intermittent porphyria, hematin given intravenously caused marked diminution of serum delta-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 delta-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.

MeSH Terms
Adult Amino Acids/antagonists & inhibitors,blood,cerebrospinal fluid,urine Female Heme/administration & dosage Humans Injections, Intravenous Levulinic Acids/antagonists & inhibitors,blood,cerebrospinal fluid,urine Porphyrias/drug therapy,metabolism Porphyrins/antagonists & inhibitors,blood,cerebrospinal fluid,urine Propionates/antagonists & inhibitors,blood,cerebrospinal fluid,urine Pyrroles/antagonists & inhibitors,blood,cerebrospinal fluid,urine Time Factors
Chemicals
Amino Acids Levulinic Acids Porphyrins Propionates Pyrroles Heme
Authors & Affiliations
7 authors, click to expand affiliations / ORCID
Bonkowsky H L
Tschudy D P
Collins A
Doherty J
Bossenmaier I
Cardinal R
Watson C J
References (25)
25 references, click to expand
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Article Info
Journal
Proceedings of the National Academy of Sciences of the United States of America
Abbr.
Proc Natl Acad Sci U S A
ISSN
0027-8424
DOI
10.1073/pnas.68.11.2725
Published
1971-11-00
Pages
2725-9
Language
English
Region
United States
NLM ID
7505876
PMCID
PMC389510
Subset
IM
External Links
PubMed source DOI: 10.1073/pnas.68.11.2725 PMC Full Text View journal details
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