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用于肌肉活检以检测恶性高热易感性的麻醉

[Anesthesia for muscle biopsy to test susceptibility to malignant hyperthermia].

作者信息

Silva Helga Cristina Almeida da, Onari Elton Shinji, de Castro Isac, Perez Marcelo Vaz, Hortensi Alexandre, Amaral José Luiz Gomes do

机构信息

Universidade Federal de São Paulo (Unifesp) Escola Paulista de Medicina (EPM), Disciplina Anestesiologia, Dor e Terapia Intensiva Centro de Estudo, Diagnóstico e Investigação de Hipertermia Maligna (Cedhima), São Paulo, SP, Brasil.

Universidade Federal de São Paulo (Unifesp) Escola Paulista de Medicina (EPM), Disciplina Anestesiologia, Dor e Terapia Intensiva Centro de Estudo, Diagnóstico e Investigação de Hipertermia Maligna (Cedhima), São Paulo, SP, Brasil.

出版信息

Braz J Anesthesiol. 2019 Jul-Aug;69(4):335-341. doi: 10.1016/j.bjan.2019.02.001. Epub 2019 Apr 30.

Abstract

INTRODUCTION

Malignant hyperthermia is an autosomal dominant pharmacogenetic disorder, characterized by hypermetabolic crisis triggered by halogenated anesthetics and/or succinylcholine. The standard method for diagnosing malignant hyperthermia susceptibility is the in vitro muscle contracture test in response to halothane-caffeine, which requires muscle biopsy under anesthesia. We describe a series of anesthetic procedures without triggering agents in malignant hyperthermia, comparing peripheral nerve block and subarachnoid anesthesia.

METHOD

We assessed the anesthetic record charts of 69 patients suspected of malignant hyperthermia susceptibility who underwent muscle biopsy for in vitro muscle contracture in the period of 7 years. Demographic data, indication for malignant hyperthermia investigation, in vitro muscle contracture test results, and surgery/anesthesia/recovery data were analyzed.

RESULTS

Sample with 34 ± 13.7 years, 60.9% women, 65.2% of in vitro muscle contracture test positive. Techniques used: peripheral nerve blocks — lateral femoral and femoral cutaneous, latency 65 ± 41 min — (47.8%); subarachnoid anesthesia (49.3%), and total venous anesthesia (1.4%). There was 39.4% failure of peripheral nerve block and 11.8% of subarachnoid anesthesia. Adverse events (8.7%) occurred only with subarachnoid blockade (bradycardia, nausea, and transient neurological syndrome). All patients remained in the post-anesthesia care unit until discharge. Age and weight were significantly higher in patients with blockade failure (ROC cut-off point of 23.5 years and 59.5 kg) and blockade failure was more frequent in the presence of increased idiopathic creatine kinase.

CONCLUSION

Anesthesia with non-triggering agents has been shown to be safe in patients with malignant hyperthermia susceptibility. Variables such as age, weight, and history of increased idiopathic creatine kinase may be useful in selecting the anesthetic technique for this group of patients.

摘要

引言

恶性高热是一种常染色体显性遗传药理学疾病,其特征为由卤化麻醉剂和/或琥珀酰胆碱引发的高代谢危机。诊断恶性高热易感性的标准方法是对氟烷-咖啡因作出反应的体外肌肉挛缩试验,这需要在麻醉状态下进行肌肉活检。我们描述了一系列在恶性高热患者中不使用触发剂的麻醉程序,比较了外周神经阻滞和蛛网膜下腔麻醉。

方法

我们评估了69例疑似恶性高热易感性患者的麻醉记录图表,这些患者在7年期间接受了肌肉活检以进行体外肌肉挛缩试验。分析了人口统计学数据、恶性高热调查指征、体外肌肉挛缩试验结果以及手术/麻醉/恢复数据。

结果

样本年龄为34±13.7岁,女性占60.9%,65.2%的体外肌肉挛缩试验呈阳性。使用的技术:外周神经阻滞——股外侧皮神经和股神经阻滞,潜伏期65±41分钟——(47.8%);蛛网膜下腔麻醉(49.3%),以及全静脉麻醉(1.4%)。外周神经阻滞失败率为39.4%,蛛网膜下腔麻醉失败率为11.8%。不良事件(8.7%)仅发生在蛛网膜下腔阻滞时(心动过缓、恶心和短暂性神经综合征)。所有患者均在麻醉后护理单元留观直至出院。阻滞失败患者的年龄和体重显著更高(ROC截止点为23.5岁和59.5千克),并且在特发性肌酸激酶升高的情况下阻滞失败更为常见。

结论

对于恶性高热易感性患者,已证明使用非触发剂进行麻醉是安全的。年龄、体重和特发性肌酸激酶升高病史等变量可能有助于为该组患者选择麻醉技术。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d872/9391910/24aa85833f35/gr1.jpg

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