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血红素治疗急性卟啉病。几乎致命的复发早期缓解。

Hematin treatment of acute porphyria. Early remission of an almost fatal relapse.

作者信息

Peterson A, Bossenmaier I, Cardinal R, Watson C J

出版信息

JAMA. 1976 Feb 2;235(5):520-2.

PMID:946099
Abstract

Intravenous infusions of hematin in a young woman with acute porphyria in profound relapse was followed within 48 hours by remission of symptoms and rapid recovery. From a state of severe central and peripheral nervous-system involvement, the patient recovered so completely that she was able to leave the hospital in less than a month, with only a residual weakness of her arms. Serial studies of serum and urinary levles of porphyrin precursors and serum level of hematin provided highly important information about the effect of hematin on acute porphyria.

摘要

对一名处于深度复发期的急性卟啉症年轻女性静脉输注血红素,48小时内症状缓解并迅速康复。患者从严重的中枢和外周神经系统受累状态完全恢复,以至于不到一个月就能够出院,仅手臂残留轻度无力。对卟啉前体的血清和尿液水平以及血红素血清水平的系列研究提供了关于血红素对急性卟啉症影响的极其重要的信息。

相似文献

1
Hematin treatment of acute porphyria. Early remission of an almost fatal relapse.血红素治疗急性卟啉病。几乎致命的复发早期缓解。
JAMA. 1976 Feb 2;235(5):520-2.
2
[Hematin treatment of acute intermittent porphyria. A case report (author's transl)].[急性间歇性卟啉病的血红素治疗。病例报告(作者译)]
Rev Bras Pesqui Med Biol. 1979 Sep;12(4-5):325-33.
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[Acute attacks of hepatic porphyria. Treatment with hematin. 5 cases].[急性肝卟啉症发作。血红素治疗。5例]
Presse Med. 1986 Oct 4;15(33):1673-6.
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Treatment of acute hepatic porphyria with hematin.用血红素治疗急性肝卟啉病。
J Hepatol. 1988 Feb;6(1):1-7. doi: 10.1016/s0168-8278(88)80456-2.
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[Effect of hematin in acute porphyric relapse].[血红素对急性卟啉症复发的影响]
Rev Med Chil. 1979 Jan;107(1):38-41.
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Effects of hematin in hepatic porphyria. Further studies.血晶素对肝性卟啉症的影响。进一步研究。
Ann Intern Med. 1975 Jul;83(1):20-30. doi: 10.7326/0003-4819-83-1-20.
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[Treatment of acute intermittent porphyria with a new protein-bound lyophilized hematin].[用一种新型蛋白结合冻干血红素治疗急性间歇性卟啉病]
Dtsch Med Wochenschr. 1987 Aug 21;112(34):1302-5. doi: 10.1055/s-2008-1068240.
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Hematin and propranolol in acute intermittent porphyria. Full recovery from quadriplegic coma and respiratory failure.急性间歇性卟啉病中使用高铁血红素与普萘洛尔。四肢瘫性昏迷和呼吸衰竭完全康复。
Eur Neurol. 1979;18(5):289-94. doi: 10.1159/000115092.
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Postulated deficiency of hepatic heme and repair by hematin infusions in the "inducible" hepatic porphyrias.“可诱导性”肝性卟啉病中推测的肝血红素缺乏及血红素输注修复作用
Proc Natl Acad Sci U S A. 1977 May;74(5):2118-20. doi: 10.1073/pnas.74.5.2118.
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Prevention of acute porphyric attacks by intravenous haematin.静脉注射血红素预防急性卟啉症发作
Lancet. 1978 Sep 2;2(8088):492-4. doi: 10.1016/s0140-6736(78)92217-1.

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Therapy Follows Diagnosis: Old and New Approaches for the Treatment of Acute Porphyrias, What We Know and What We Should Know.
治疗遵循诊断:急性卟啉病治疗的新旧方法,我们所知道的和我们应该知道的。
Diagnostics (Basel). 2022 Jul 3;12(7):1618. doi: 10.3390/diagnostics12071618.
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A case of variegate porphyria in association with coeliac disease and bisphosphonate associated dental osteonecrosis.一例伴有乳糜泻和双膦酸盐相关颌骨坏死的混合型卟啉病病例。
J Clin Med Res. 2009 Dec;1(5):292-6. doi: 10.4021/jocmr2009.11.1270. Epub 2009 Dec 28.
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Brain 5-aminolaevulinate synthase. Developmental aspects and evidence for regulatory role.脑5-氨基酮戊酸合酶。发育方面及调节作用的证据。
Biochem J. 1981 Jun 15;196(3):811-7. doi: 10.1042/bj1960811.
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The "glucose effect" in acute hepatic porphyrias and in experimental porphyria.急性肝卟啉病及实验性卟啉病中的“葡萄糖效应”
Klin Wochenschr. 1981 Jul 1;59(13):727-35. doi: 10.1007/BF01721260.
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Delta-Aminolaevulinic acid and amino acid neurotransmitters.δ-氨基乙酰丙酸与氨基酸神经递质。
Mol Cell Biochem. 1981 Aug 11;38 Spec No(Pt 1):49-58. doi: 10.1007/BF00235687.
8
Postulated deficiency of hepatic heme and repair by hematin infusions in the "inducible" hepatic porphyrias.“可诱导性”肝性卟啉病中推测的肝血红素缺乏及血红素输注修复作用
Proc Natl Acad Sci U S A. 1977 May;74(5):2118-20. doi: 10.1073/pnas.74.5.2118.
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Hepatic heme metabolism and its control.肝脏血红素代谢及其调控
Yale J Biol Med. 1979 Jan-Feb;52(1):13-37.