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杜兴氏肌营养不良症:重新审视一个古老的麻醉问题。

Duchenne muscular dystrophy: an old anesthesia problem revisited.

作者信息

Hayes Jason, Veyckemans Francis, Bissonnette Bruno

机构信息

The Hospital for Sick Children, Toronto, ON, Canada.

出版信息

Paediatr Anaesth. 2008 Feb;18(2):100-6. doi: 10.1111/j.1460-9592.2007.02302.x.

DOI:10.1111/j.1460-9592.2007.02302.x
PMID:18184239
Abstract

Patients with Duchenne and Becker muscular dystrophy suffer from a progressive deterioration in muscle secondary to a defect in the dystrophin gene. As such, they are susceptible to perioperative respiratory, cardiac and other complications, such as rhabdomyolysis. Inhalational anesthetic agents have been implicated as a cause of acute rhabdomyolysis that can resemble malignant hyperthermia (MH). This article reviews perioperative 'MH-like' reactions reported in muscular dystrophy patients and groups them into three categories according to clinical presentation. The etiology and underlying pathophysiological process responsible for these reactions is discussed and recommendations are proposed for the safe anesthetic management of these patients.

摘要

杜兴氏和贝克氏肌肉营养不良症患者由于肌营养不良蛋白基因缺陷,肌肉会逐渐恶化。因此,他们易发生围手术期呼吸、心脏及其他并发症,如横纹肌溶解症。吸入性麻醉剂被认为是导致急性横纹肌溶解症的原因之一,这种症状可能类似于恶性高热(MH)。本文回顾了肌肉营养不良症患者围手术期出现的“类MH”反应,并根据临床表现将其分为三类。讨论了这些反应的病因及潜在病理生理过程,并对这些患者的安全麻醉管理提出了建议。

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