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一名孕妇因急性肝卟啉症导致难治性癫痫持续状态:人工流产是唯一的治疗选择吗?

Refractory status epilepticus due to acute hepatic porphyria in a pregnant woman: induced abortion as the sole therapeutic option?

作者信息

Engelhardt K, Trinka E, Franz G, Unterberger I, Spiegel M, Beer R, Pfausler B, Kampfl A, Schmutzhard E

机构信息

Department of Neurology, University Hospital Innsbruck, Austria.

出版信息

Eur J Neurol. 2004 Oct;11(10):693-7. doi: 10.1111/j.1468-1331.2004.00876.x.

Abstract

A 22-years old, 55 kg female patient in the twelfth week of pregnancy developed neuropsychiatric syndromes and in the following status epilepticus. Raised porphyrines and porphyrine precursors were found in the patient's urine. Despite intravenous glucose infusions and appropriate medication no reduction in seizure-frequency and neuropsychiatric syndromes was observed. An abortion was induced. After the interruption and starting of haem arginate therapy, seizure activity stopped and porphyrine precursors returned to normal levels, and after 6 weeks the patient was discharged in excellent clinical condition. This report describes a status epilepticus caused by acute hepatic porphyria, triggered by pregnancy, in a 22-years old woman. To our knowledge this is the first report of induced abortion as successful treatment in acute hepatic porphyria induced status epilepticus.

摘要

一名22岁、体重55公斤的女性患者,在怀孕第12周时出现神经精神综合征,随后发生癫痫持续状态。在患者尿液中发现卟啉和卟啉前体升高。尽管静脉输注葡萄糖并给予适当药物治疗,但癫痫发作频率和神经精神综合征未见减轻。遂实施了人工流产。人工流产后开始进行血红素精氨酸治疗,癫痫活动停止,卟啉前体恢复到正常水平,6周后患者临床状况良好出院。本报告描述了一名22岁女性因妊娠引发急性肝卟啉病导致的癫痫持续状态。据我们所知,这是关于人工流产作为急性肝卟啉病诱发的癫痫持续状态成功治疗方法的首例报告。

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