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[急性肝卟啉症发作。血红素治疗。5例]

[Acute attacks of hepatic porphyria. Treatment with hematin. 5 cases].

作者信息

Devars du Mayne J F, Deybach J C, Phung L, Likforman J, Tenhunen R, Cerf M, Nordmann Y

出版信息

Presse Med. 1986 Oct 4;15(33):1673-6.

PMID:2947113
Abstract

The effectiveness of 2 hematins administered by intravenous infusion was compared in acute intermittent porphyria. Judging from subjective symptoms (abdominal pain), clinical improvement was complete and constant. There was a rapid decrease in urinary excretion of porphyrins precursors, with a clearer response of delta-aminolevulinic acid than of porphobilinogen. Urine levels of uroporphyrin often returned to normal after two infusions. The drugs were very well tolerated. Provided it is administered early, before neurological complications develop, treatment with hematin completely relieves abdominal symptoms and suppresses most of the biochemical changes associated with hepatic porphyria.

摘要

在急性间歇性卟啉症中比较了两种通过静脉输注给药的血红素的有效性。从主观症状(腹痛)判断,临床改善是完全且持续的。卟啉前体的尿排泄量迅速下降,δ-氨基乙酰丙酸的反应比胆色素原更明显。经两次输注后,尿卟啉水平通常恢复正常。这些药物耐受性良好。如果在神经并发症出现之前尽早给药,血红素治疗可完全缓解腹部症状,并抑制与肝卟啉症相关的大多数生化变化。

相似文献

1
[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.
2
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.
3
[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.
4
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.
5
Intravenous heme-albumin in acute intermittent porphyria: evidence for repletion of hepatic hemoproteins and regulatory heme pools.急性间歇性卟啉病中静脉注射血红素白蛋白:补充肝脏血红素蛋白和调节性血红素池的证据
Am J Gastroenterol. 1991 Aug;86(8):1050-6.
6
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.
7
[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.
8
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.
9
[Hemin treatment in a case of porphyria variegata].[一例杂色卟啉病的高铁血红素治疗]
Dermatologica. 1982;165(6):640-6.
10
[Treatment of acute attacks of hepatic porphyrias with hematin].[用血红素治疗肝性卟啉病急性发作]
Ann Med Interne (Paris). 1989;140(2):83-5.

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1
Inhibition of the liver expression of arylalkylamine N-acetyltransferase increases the expression of angiogenic factors in cholangiocytes.抑制肝内芳香胺 N-乙酰基转移酶的表达可增加胆管细胞中血管生成因子的表达。
Hepatobiliary Surg Nutr. 2014 Feb;3(1):4-10. doi: 10.3978/j.issn.2304-3881.2014.01.02.
2
Haem arginate improves hepatic oxidative metabolism in variegate porphyria.精氨酸血红素可改善混合型卟啉病患者的肝脏氧化代谢。
Br J Clin Pharmacol. 1988 Dec;26(6):753-7. doi: 10.1111/j.1365-2125.1988.tb05315.x.