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  • 标题:Repression of the Overproduction of Porphyrin Precursors in Acute Intermittent Porphyria by Intravenous Infusions of Hematin
  • 本地全文:下载
  • 作者:H. L. Bonkowsky ; D. P. Tschudy ; A. Collins
  • 期刊名称:Proceedings of the National Academy of Sciences
  • 印刷版ISSN:0027-8424
  • 电子版ISSN:1091-6490
  • 出版年度:1971
  • 卷号:68
  • 期号:11
  • 页码:2725-2729
  • DOI:10.1073/pnas.68.11.2725
  • 语种:English
  • 出版社:The National Academy of Sciences of the United States of America
  • 摘要: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.
  • 关键词:δ-aminolevulinic acid ; porphobilinogen ; heme ; cerebrospinal fluid
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