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[妊娠期急性间歇性卟啉症:葡萄糖还是血红素治疗?]

[Acute intermittent porphyria in pregnancy: glucose or hematin therapy?].

作者信息

Isenschmid M, König C, Fässli C, Haenel A, Hänggi W, Schneider H

机构信息

Abteilung Gynäkologie und Geburtshilfe, Kantonsspital Fribourg.

出版信息

Schweiz Med Wochenschr. 1992 Nov 14;122(46):1741-5.

PMID:1448679
Abstract

The symptoms and management of acute intermittent porphyria (AIP) during pregnancy and delivery are presented on the basis of three case reports. Two patients became asymptomatic after infusion of a concentrated glucose solution (600 ml 50% glucose = 300 g glucose per day), while the third patient needed treatment with hematin (1 mg/kg body weight, 6 infusions at intervals of 12 hours) because of deterioration of psychic state in spite of glucose infusion. Whenever an attack of AIP occurs, concentrated glucose solution should be administrated as a first measure accompanied by an analgesic and possibly a neuroleptic agent after elimination of precipitating factors. However, if symptoms persist for 48-96 hours or the psychic and/or neurological state of the patient further deteriorates, administration of 1-3 mg hematin per kg body weight can be recommended.

摘要

基于三例病例报告,介绍了妊娠期和分娩期急性间歇性卟啉病(AIP)的症状及处理方法。两名患者在输注浓缩葡萄糖溶液(600毫升50%葡萄糖,即每天300克葡萄糖)后症状消失,而第三名患者尽管输注了葡萄糖,但由于精神状态恶化,需要用血红素治疗(1毫克/千克体重,每隔12小时输注6次)。每当AIP发作时,应首先采取输注浓缩葡萄糖溶液的措施,并在消除诱发因素后,给予止痛剂,可能还需给予抗精神病药物。然而,如果症状持续48 - 96小时,或者患者的精神和/或神经状态进一步恶化,则建议每千克体重给予1 - 3毫克血红素。

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