Anesthetic management in MAO-A and MAO-B deficiency: a case report
Anesthetic management in MAO-A and MAO-B deficiency: a case report
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MAO-A 和 MAO-B 缺乏症的麻醉管理：病例报告
DOI：
10.1007/s00540-020-02808-5
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发表时间：
2020
影响因子：
2.8
通讯作者：
Mizuta Kentaro
中科院分区：
文献类型：
--
作者：
Hoshijima Hiroshi;Takeuchi Risa;Kikuchi Kimiharu;Mizuta Kentaro
单胺氧化酶（MAO）缺乏症是一种X连锁遗传性疾病，其特征是X染色体上MAO- a和/或MAO- b的自发缺失。在这里，我们描述了第一个报告的病例患者与MAO-A和MAO-B缺乏症管理全麻牙科治疗。病人在接受牙科治疗时只有11岁。2岁时经基因检测诊断为MAO-A和MAO-B缺乏症。患者未给予预用药，并建立了无创血压监测、脉搏血氧仪和心电图的标准监测。我们还预先准备了一个心脏转复除颤器。丙泊酚46 mg （2 mg/kg）全身麻醉后，分别通过泵给药罗库溴铵10 mg （0.4 mg/kg）和瑞芬太尼0.30 μg/kg/min。经气管插管无并发症。持续输注瑞芬太尼0.15 ~ 0.2 μg/kg/min，异丙酚5.0 ~ 7.0 mg/kg，维持麻醉平稳。新鲜气流包括氧气和空气。在整个过程中，潮汐末二氧化碳浓度维持在35毫米汞柱左右。我们给予糖马德92 mg （4 mg/kg）用于逆转神经肌肉阻断，并拔管。我们在这位MAO-A和MAO-B缺乏症患者中取得了成功的麻醉管理，没有任何明显的致死性心律失常的临床体征。
Monoamine oxidase (MAO) deficiency is an X-linked hereditary disease characterized by spontaneous deletion of MAO-A and/or MAO-B on the X chromosome. Here, we describe the first reported case of a patient with MAO-A and MAO-B deficiency managed under general anesthesia in dental treatment. The patient was aged 11 years old when he was scheduled for dental treatment. He was diagnosed with MAO-A and MAO-B deficiency on genetic testing at 2 years of age. He was not given premedication, and standard monitoring with noninvasive blood pressure monitoring, pulse oximetry, and ECG was instituted. We also preemptively prepared a cardioverter-defibrillator. General anesthesia was induced with propofol 46 mg (2 mg/kg), then rocuronium 10 mg (0.4 mg/kg) and remifentanil 0.30 μg/kg/min were administered via separate infusion pumps. Orotracheal intubation was performed without complications. Anesthesia was maintained uneventfully with a continuous infusion of remifentanil 0.15–0.2 μg/kg/min and propofol 5.0–7.0 mg/kg. Fresh gas flow included oxygen and air. End-tidal CO2concentration was maintained at around 35 mmHg throughout the procedure. We administered sugammadex 92 mg (4 mg/kg) for reversal of neuromuscular blockade and the patient was extubated. We achieved successful anesthetic management without any appreciable clinical signs of fatal arrhythmias in this patient with MAO-A and MAO-B deficiency.
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