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一名患有卡恩斯-塞尔综合征和夏科-马里-图思病的患者因室上性心动过速消融术:病例报告

A Patient With Kearns Sayre Syndrome and Charcot-Marie-Tooth for Supraventricular Tachycardia Ablation: A Case Report.

作者信息

Warncke Kristen A, Marshall Julie M

机构信息

From the Department of Anesthesiology and Perioperative Medicine, University of Missouri, Columbia, Missouri.

出版信息

A A Pract. 2021 Jun 2;15(6):e01488. doi: 10.1213/XAA.0000000000001488.

Abstract

Kearns Sayre syndrome (KSS) is a rare mitochondrial myopathy that is associated with progressive impaired ventilatory drive, heart block, and peripheral neuropathy. Charcot-Marie-Tooth disease (CMT) is a rare chronic motor and sensory peripheral neuropathy which includes muscle weakness and restrictive pulmonary impairment. Patients with either condition having anesthesia can have postoperative respiratory failure. We report a 52-year-old man diagnosed with variants of KSS and CMT presenting for supraventricular tachycardia (SVT) ablation. This is the first report of successful perioperative management of a general anesthetic in a patient with both KSS and CMT.

摘要

卡恩斯-塞尔综合征(KSS)是一种罕见的线粒体肌病,与进行性通气驱动受损、心脏传导阻滞和周围神经病变有关。夏科-马里-图斯病(CMT)是一种罕见的慢性运动和感觉性周围神经病变,包括肌肉无力和限制性肺功能损害。患有这两种疾病之一且接受麻醉的患者可能会出现术后呼吸衰竭。我们报告了一名52岁男性,诊断为KSS和CMT变异型,因室上性心动过速(SVT)消融术前来就诊。这是首例成功对同时患有KSS和CMT的患者进行全身麻醉围手术期管理的报告。

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