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用血红素治疗急性肝卟啉病。

Treatment of acute hepatic porphyria with hematin.

作者信息

Bissell D M

机构信息

Liver Core Center, University of California, San Francisco.

出版信息

J Hepatol. 1988 Feb;6(1):1-7. doi: 10.1016/s0168-8278(88)80456-2.

DOI:10.1016/s0168-8278(88)80456-2
PMID:3346530
Abstract

The efficacy of hematin has been evaluated in eight patients with acute intermittent porphyria: six with acute attacks and two with chronic subacute symptoms. Hematin suppressed the chemical signs of porphyria in all patients and the symptoms in those with acute attacks. The clinical response to hematin occurred uniformly on the third or fourth day of treatment, suggesting that a stereotypic response to this form of therapy can be defined. Hematin had no effect on chronic subacute symptoms. The indications for hematin therapy, its rationale and the details of its administration are reviewed.

摘要

已对8例急性间歇性卟啉病患者的血红素疗效进行了评估:6例为急性发作,2例为慢性亚急性症状。血红素抑制了所有患者的卟啉病化学指标以及急性发作患者的症状。治疗第三天或第四天,患者对血红素均出现一致的临床反应,这表明可以确定对这种治疗形式的典型反应。血红素对慢性亚急性症状无效。本文对血红素治疗的适应症、理论依据及其给药细节进行了综述。

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