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[家族性高钾性周期性麻痹的麻醉]

[Anesthesia in familial hyperkalemic periodic paralysis].

作者信息

Thiel A, Wagner M, Behr R, Hempelmann G

机构信息

Abteilung für Anästhesiologie und Operative Intensivmedizin, Justus-Liebig-Universität Giessen.

出版信息

Anasthesiol Intensivmed Notfallmed Schmerzther. 1993 Apr;28(2):125-7. doi: 10.1055/s-2007-998891.

DOI:10.1055/s-2007-998891
PMID:8324104
Abstract

A 53-year-old woman was scheduled for elective surgery of a L4-L5 lumbar disc prolapse. Preoperatively, she reported of a hereditary muscle disease in her family which could be identified as familial hyperkalaemic periodic paralysis. In patients with familial periodic paralysis, only limited information is available in the current anaesthesiological literature. This is especially true of the hyperkalaemic form which was separated from the hypokalaemic form in 1957. Most patients suffering from periodic paralysis will develop myotonic symptoms in time, but evidence is lacking that the incidence of malignant hyperthermia (MH) is higher than in normals. However, abnormalities of the electrocardiogram (ECG) due to changes in the potassium serum levels are not unusual, and the anaesthesiologist must be aware of cardiac arrhythmias. In the present case, the patient was anaesthetized using fentanyl, midazolam, and vecuronium, and ventilated with 66% nitrous oxide in oxygen. Radial artery blood pressure, end-tidal CO2, and nasopharyngeal temperature were monitored continuously. Surgery was completed after 2 hours with no abnormalities in the intraoperative course. Recovery from anaesthesia was uneventful. During the postoperative follow-up for 1 week, further attacks of paralysis, intermittent sodium-potassium imbalance of the muscle cell membrane appears to be the primary pathogenetic factor. In this special disease, prevention of carbohydrate depletion and the avoidance of muscle relaxants are recommended in the anaesthesiological literature. However, a specified regimen of general anaesthesia has not yet been outlined. In our special case, the use of anaesthetics deemed to be safe in MH susceptible patients produced an uneventful perioperative course.(ABSTRACT TRUNCATED AT 250 WORDS)

摘要

一名53岁女性计划接受L4-L5腰椎间盘突出症的择期手术。术前,她报告家族中有遗传性肌肉疾病,可确诊为家族性高钾性周期性麻痹。在家族性周期性麻痹患者中,当前麻醉学文献中的信息有限。1957年从低钾性类型中分离出来的高钾性类型尤其如此。大多数周期性麻痹患者迟早会出现肌强直症状,但缺乏证据表明恶性高热(MH)的发生率高于正常人。然而,由于血清钾水平变化导致的心电图(ECG)异常并不罕见,麻醉医生必须意识到心律失常。在本病例中,患者使用芬太尼、咪达唑仑和维库溴铵进行麻醉,并以66%氧化亚氮和氧气进行通气。持续监测桡动脉血压、呼气末二氧化碳和鼻咽温度。手术2小时后完成,术中过程无异常。麻醉恢复顺利。在术后1周的随访中,未出现进一步的麻痹发作,肌肉细胞膜的间歇性钠钾失衡似乎是主要的致病因素。在这种特殊疾病中,麻醉学文献建议预防碳水化合物消耗并避免使用肌肉松弛剂。然而,尚未概述特定的全身麻醉方案。在我们的特殊病例中,使用被认为对MH易感患者安全的麻醉剂使围手术期过程顺利。(摘要截断于250字)

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