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[一名患有杜氏肌营养不良症的患者接受法洛四联症根治性修复手术的麻醉管理]

[Anesthetic management of a patient with Duchenne's muscular dystrophy undergoing radical repair for tetralogy of Fallot].

作者信息

Taguchi A, Takada K, Horiuchi F, Suzuki H

机构信息

Department of Anesthesiology, Tokyo Women's Medical College.

出版信息

Masui. 1997 Dec;46(12):1594-8.

PMID:9455083
Abstract

This report describes a 13-month-old-girl with Duchenne's muscular dystrophy (DMD) who had radical repair for tetralogy of Fallot safely. Patients with DMD are considered to be at risk of malignant hyperthermia (MH). Drugs for induction and maintenance were chosen from a list of agents rarely associated with MH. To wash out the inhalation anesthetics from the equipment, oxygen was circulated continuously for 24 hours. Dantrolene sodium was kept readily available in case of MH occurrence. Differential diagnosis during surgery is difficult in term of the episodes of MH and complications of cardiac surgery, as cardiac surgery is also associated with tachycardia, tachyarrhythmias, metabolic asidosis and red colored urine, which are frequently accompanied by MH. Although increased levels of CK, GOT, LDH and myoglobin strongly support the diagnosis of MH, such evidence can only be confirmed after operation. Fortunately, these factors recovered to the normal range without treatment by dantrolene sodium. During the cardiac surgery, treatment of MH may be delayed due to its late confirmation.

摘要

本报告描述了一名患有杜氏肌营养不良症(DMD)的13个月大女孩,她接受了法洛四联症根治术,手术过程安全。患有DMD的患者被认为有恶性高热(MH)风险。诱导和维持用药从与MH关联极少的药物清单中选择。为从设备中清除吸入麻醉剂,氧气持续循环24小时。准备好丹曲林钠以备发生MH时使用。由于心脏手术也会出现心动过速、快速性心律失常、代谢性酸中毒和红色尿液,这些情况常与MH伴随出现,所以在手术过程中,就MH发作和心脏手术并发症进行鉴别诊断很困难。尽管肌酸激酶(CK)、谷草转氨酶(GOT)、乳酸脱氢酶(LDH)和肌红蛋白水平升高强烈支持MH的诊断,但此类证据只能在术后得到证实。幸运的是,这些指标未经丹曲林钠治疗便恢复到了正常范围。在心脏手术期间,由于MH确诊较晚,其治疗可能会延迟。

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