卟啉症的麻醉意义

Milica Randjelović, M. Stojanovic, Jovan Z. Radeka, Milena N. Vasilijic, R. Jankovic
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引用次数: 0

摘要

卟啉症是一组与血红素生物合成缺陷有关的疾病,由合成途径中某些酶的功能障碍引起。红细胞生成性卟啉症表现为皮肤症状,不影响神经系统。肝性卟啉症急性发作,伴有轻度至重度神经内脏症状,病程剧烈,罕见但可能致命的结局。肝性卟啉症患者的麻醉管理具有挑战性,因为使用诱导或维持麻醉的药物可能引发或加重急性发作。根据实验室实验、临床研究、病例报告和经验,这些药物被标记为安全或不安全。在本文中,我们讨论潜在的病理生理,表现,治疗建议和麻醉相关的卟啉症。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Anesthetic implications in porphyrias
The porphyrias are a group of disorders related to deficient heme biosynthesis, caused by malfunction of certain enzymes in the synthesis pathway. Erythropoietic porphyrias present with cutaneous symptoms and do not affect the nervous system. Hepatic porphyrias develop acute attacks with mild to severe neurovisceral symptoms, dramatic course, and rare, but possibly lethal outcomes. Anesthetic management of patients suffering from hepatic porphyria is challenging regarding the possibility of triggering or worsening the acute attack with medications that induce or maintain anesthesia. The medications are labeled as safe or unsafe according to laboratory experiments, clinical studies, case reports and experience. In this paper, we discuss underlying pathophysiology, presentation, therapy recommendations and anesthetic implications related to porphyrias.
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