Malignant hyperthermia

恶性高热
  • 文章类型: Case Reports
    恶性高热(MH)是由挥发性麻醉剂引发的潜在危及生命的药物遗传综合征,琥珀酰胆碱,以及剧烈运动等压力。
    作者描述了一例8岁男性,他在全身麻醉诱导后出现MH的典型症状,对症治疗由于丹曲林不可用而成功完成。
    MH的明确诊断可以基于在氟烷和咖啡因存在下的新鲜肌肉活检中的挛缩测试来进行。在没有肌肉活检和基因检测的情况下,MH的诊断可以根据MH评分进行。
    麻醉医师应该意识到,适当的对症管理也可以挽救患者的生命。此外,应大力倡导确保Danthroene的可用性,并进一步加强实验室设施以确认诊断,以促进将来的诊断和管理。
    UNASSIGNED: Malignant hyperthermia (MH) is a potentially life-threatening pharmacogenetic syndrome triggered by volatile anaesthetics, succinylcholine, and stress such as vigorous exercise.
    UNASSIGNED: The authors describe a case of an 8-year-old male who presented with classical symptoms of MH after induction of general anaesthesia and symptomatic treatment was done successfully due to the unavailability of Dantrolene.
    UNASSIGNED: Definitive diagnosis of MH can be done based on a contracture test in fresh muscle biopsy in the presence of halothane and caffeine. In the absence of muscle biopsy and genetic testing, diagnosis for MH can be done based on MH scoring.
    UNASSIGNED: Anesthesiologists should be made aware that proper symptomatic management can also save the life of a patient. Also, strong advocacy should be done to ensure the availability of Dantrolene and further strengthen lab facilities to confirm diagnosis to facilitate diagnosis and management in the future.
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  • 文章类型: Journal Article
    恶性高热(MH),以严重的肌阵鸣为特征,发热,心动过速,高血压,肌肉酶升高,和高碳酸血症,常发生于先天性畸形或遗传性疾病患者。尽管报道的发病率低至1:5000至1:100,000,但MH患者表现出迅速恶化和死亡率升高。因此,MH与大量围手术期风险相关。MH患者的成功治疗在很大程度上取决于早期诊断和及时有效治疗。该临床报告提供了新诊断为MH的患者的详细描述,该患者体温迅速升高,潮气末二氧化碳,上颌骨截骨术时的心率。抢救成功后,患者在术后恢复顺利,表明术中监测的重要性,早期诊断,有效治疗,和术后监测。该病例有望作为未来干预措施和医疗保健实践的参考,以管理其他MH患者。
    Malignant hyperthermia (MH), characterized by severe myoclonus, pyrexia, tachycardia, hypertension, elevated muscle enzymes, and hypercapnia, often occurs in patients with congenital deformities or genetic disorders. Although the reported incidence rate is as low as 1:5000 to 1:100,000, patients with MH exhibit rapid aggravation and an elevated mortality rate. Thus, MH is associated with substantial perioperative risk. Successful treatment of patients with MH largely depends on early diagnosis and timely effective treatment. This clinical report provides a detailed description of a patient with newly diagnosed MH who developed a rapid rise in body temperature, end-tidal carbon dioxide, and heart rate during maxillary osteotomy. After successful rescue, the patient recovered smoothly during the postoperative period, indicating the importance of intraoperative monitoring, early diagnosis, effective treatment, and postoperative monitoring. This case is expected to serve as a reference for future interventions and healthcare practices in managing other patients with MH.
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  • 文章类型: Case Reports
    麻醉医师一直处于解决围手术期患者安全问题的最前沿。因为麻醉没有直接的治疗益处,它的风险必须降到最低。有时手术很简单,但患者的病情使麻醉管理复杂化,增加并发症的风险。本报告描述了诊断为包涵体肌炎(IBM)的成年患者的麻醉管理,一种罕见的炎症性退行性肌病,最初表现为下肢和上肢运动功能下降,导致他卧床两年。由于他的病情进展,他最终出现了吞咽困难,因此他被安排做食管镜检查,环咽注射肉毒杆菌,和经皮内镜胃造瘘术.由于IBM患者存在对神经肌肉阻滞剂过度敏感和呼吸损害的风险,麻醉是由多学科团队方法领导的。围手术期管理以术前优化为中心,防止误吸,避免可能引发恶性高热的麻醉剂,预防术后肺部并发症。住院过程简单,患者在一天后出院。本报告强调了资源的改进,技术,和医疗保健服务,尤其是在麻醉中,帮助预防围手术期不良事件。
    Anesthesiologists have been at the forefront of initiatives addressing perioperative patient safety. As anesthesia has no direct therapeutic benefit, its risk must be minimized. At times the surgery is simple but the patient\'s condition complicates anesthetic management, increasing the risk for complications. This report describes the anesthetic management of an adult patient diagnosed with inclusion body myositis (IBM), a rare inflammatory degenerative myopathy, who initially presented with decreased motor function in both lower and upper extremities causing him to be bedbound for two years. Due to the progression of his disease, he eventually developed dysphagia, hence he was scheduled for esophagoscopy, cricopharyngeal Botox injection, and percutaneous endoscopic gastrostomy. As patients with IBM are at risk for exaggerated sensitivity to neuromuscular blockers and respiratory compromise, anesthesia was at the helm of a multidisciplinary team approach. The perioperative management centered on preoperative optimization, prevention of aspiration, avoidance of anesthetics that may trigger malignant hyperthermia, and prevention of postoperative pulmonary complication. The hospital course was uncomplicated and the patient was discharged well after one day. This report emphasizes how improvements in resources, technology, and healthcare delivery, especially in anesthesia, help prevent perioperative adverse events.
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  • 文章类型: Journal Article
    背景:恶性高热是由特定麻醉药物引发的潜在致命疾病,特别是琥珀酰胆碱(Suxamethonium)的去极化肌肉松弛剂。尽管经常使用琥珀酰胆碱与电惊厥治疗(ECT),目前还没有报道ECT后可能致命的恶性高热的病例.此外,在ECT的背景下,尚未概述琥珀酰胆碱给药与恶性高热发作之间的时间间隔。
    方法:我们介绍了一个79岁女性患有严重抑郁症的案例,在ECT会话期间,由于琥珀酰胆碱的使用而经历了严重的恶性高热。她出现了40.2摄氏度的高烧,140/min的心动过速,血压超过200mmHg的高血压,显著的肌肉僵硬,和意识受损。这些症状在ECT后两小时出现,发生在精神病房而不是手术室,并在不到24小时内达到顶峰。她服用了60毫克丹曲林,这迅速降低了肌肉的刚性。随后,她接受了两剂20毫克和60毫克丹曲林,这使她的发烧降至36.2°C,并在ECT后两天内完全缓解了肌肉僵硬。
    结论:这是首次报道的ECT后潜在致死性恶性高热的病例。此外,它突出了ECT手术后恶性高热的延迟发作,强调精神科医生即使在治疗后也要认识到其发病的必要性。鉴于恶性高热的潜在致命后果,它是至关重要的精神科医生密切监测术中和术后患者的生命体征和特征性的身体表现,及时识别任何症状的出现,并立即用丹曲林治疗。
    BACKGROUND: Malignant hyperthermia is a potentially lethal condition triggered by specific anesthetic drugs, especially a depolarizing muscle relaxant of succinylcholine (Suxamethonium). Despite the frequent use of succinylcholine with electroconvulsive therapy (ECT), there has been no reported case of potentially lethal malignant hyperthermia following ECT. In addition, the time interval between the administration of succinylcholine and the onset of malignant hyperthermia has not been outlined in the context of ECT.
    METHODS: We present the case of a 79-year-old woman suffering from severe depression, who experienced severe malignant hyperthermia due to succinylcholine administration during an ECT session. She presented with a high fever of 40.2 °C, tachycardia of 140/min, hypertension with a blood pressure exceeding 200 mmHg, significant muscle rigidity, and impaired consciousness. These symptoms emerged two hours after ECT, which occurred in a psychiatric ward rather than an operating room, and reached their peak in less than 24 h. She was given 60 mg of dantrolene, which quickly reduced the muscular rigidity. Subsequently, she received two additional doses of 20 mg and 60 mg of dantrolene, which brought her fever down to 36.2 °C and completely eased her muscle rigidity within two days after ECT.
    CONCLUSIONS: This is the first reported case of potentially lethal malignant hyperthermia after ECT. In addition, it highlights the delayed onset of malignant hyperthermia following an ECT procedure, emphasizing the necessity for psychiatrists to recognize its onset even after the treatment. In the light of potentially lethal consequences of malignant hyperthermia, it is critically important for psychiatrists to closely monitor both intraoperative and postoperative patient\'s vital signs and characteristic physical presentations, promptly identify any symptomatic emergence, and treat it immediately with dantrolene.
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  • 文章类型: Journal Article
    神经肌肉疾病患者在围手术期特别容易发生肺部和心脏并发症,或药物副作用。这些风险可能包括通气不足,吸入性肺炎,潜在心肌病恶化,心律失常,肾上腺功能不全,长时间的神经肌肉阻滞,与体温调节有关的问题,横纹肌溶解症,恶性高热,或长时间的机械通气。可以在每个围手术期实施干预以减轻这些风险。仔细的术前评估可能有助于识别风险因素,以便启动适当的干预措施。包括心脏病学咨询,肺功能检查,开始无创通气,或采取预防措施。重要的术中问题包括定位,气道和麻醉管理,和足够的通风。术后期间可能需要纠正电解质异常,用药物控制分泌物,手动或机械咳嗽辅助,避免重新插管的风险,明智的疼痛控制,和适当的药物管理。这项审查的目的是提高对这一弱势群体的特殊手术挑战的认识,并指导临床医生进行可能导致良好手术效果的各种评估和干预措施。
    Patients with neuromuscular diseases are particularly vulnerable in the perioperative period to the development of pulmonary and cardiac complications, or medication side effects. These risks could include hypoventilation, aspiration pneumonia, exacerbation of underlying cardiomyopathy, arrhythmias, adrenal insufficiency, prolonged neuromuscular blockade, issues related to thermoregulation, rhabdomyolysis, malignant hyperthermia, or prolonged mechanical ventilation. Interventions at each of the perioperative stages can be implemented to mitigate these risks. A careful pre-operative evaluation may help identify risk factors so that appropriate interventions are initiated, including cardiology consultation, pulmonary function tests, initiation of noninvasive ventilation, or implementation of preventive measures. Important intraoperative issues include positioning, airway and anesthetic management, and adequate ventilation. The postoperative period may require correction of electrolyte abnormalities, control of secretions with medications, manual or mechanical cough assistance, avoiding the risk of reintubation, judicious pain control, and appropriate medication management. The aim of this review is to increase awareness of the particular surgical challenges in this vulnerable population, and guide the clinician on the various evaluations and interventions that may result in a favorable surgical outcome.
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  • 文章类型: Case Reports
    由于恶性高热(MH)和麻醉诱导的横纹肌溶解症(AIR)的风险增加,对肌病患者的围手术期管理可能具有挑战性。然而,目前,没有证据表明副肿瘤坏死性肌病(PNM)的最佳麻醉管理(全静脉麻醉与挥发性麻醉剂)。这里,我报告了使用挥发性麻醉剂安全进行麻醉的情况。一名63岁的女性,患有与甲状腺乳头状癌相关的PNM,需要紧急甲状腺切除术。病人,先前诊断为抗3-羟基-3-甲基戊二酰辅酶A还原酶(HMGCR)抗体相关肌病,表现出进行性无力和吞咽困难,引起对PNM的怀疑。病人的呼吸状态受损,归因于大甲状腺肿压迫气管,需要紧急甲状腺切除术。麻醉管理考虑因素包括HMGCR-M对呼吸肌的潜在影响以及需要仔细计划以减轻术后并发症。患者接受了甲状腺全切除术,左中央舱间隙,气管造口术.手术进展顺利,细致的监测和调整麻醉剂,以保持血流动力学稳定。术后,病人恢复得很好,在3个月的随访中显示神经系统症状完全缓解.该病例强调了在甲状腺手术中识别副肿瘤综合征的重要性,并强调了麻醉医师面临的潜在挑战。尽管HMGCR-M坏死性肌病的麻醉药物缺乏既定的安全性数据,该案例证明了七氟醚和罗库溴铵的成功使用。
    Perioperative management of patients with myopathies can be challenging due to the increased risk of malignant hyperthermia (MH) and anesthesia-induced rhabdomyolysis (AIR). However, currently, there is no evidence regarding the optimal anesthetic management for paraneoplastic necrotizing myopathy (PNM) (total intravenous anesthetic vs. volatile anesthetics). Here, I report a case where anesthesia was administered safely using volatile anesthetics. A 63-year-old female presented with PNM associated with papillary thyroid carcinoma, necessitating urgent thyroidectomy. The patient, previously diagnosed with anti-3-hydroxy-3-methylglutaryl coenzyme A reductase (HMGCR) antibody-associated myopathy, exhibited progressive weakness and dysphagia, prompting suspicion of PNM. The patient\'s compromised respiratory status, attributed to tracheal compression by a large goiter, necessitated an urgent thyroidectomy. Anesthetic management considerations included the potential effect of HMGCR-M on respiratory muscles and the need for careful planning to mitigate postoperative complications. The patient underwent total thyroidectomy, left central compartment clearance, and tracheostomy. The surgery proceeded uneventfully, with meticulous monitoring and adjustment of anesthetic agents to maintain hemodynamic stability. Postoperatively, the patient recovered well, demonstrating complete resolution of neurological symptoms during a three-month follow-up. The case underscores the importance of recognizing paraneoplastic syndromes in the context of thyroid surgery and highlights potential challenges faced by anesthesiologists. Despite the lack of established safety data for anesthetic drugs in HMGCR-M necrotizing myopathy, the case demonstrates the successful use of sevoflurane and rocuronium.
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  • 文章类型: Case Reports
    背景:我们描述了1例中枢核心疾病患儿行支气管镜支气管肺泡灌洗的围手术期处理。避免触发剂(挥发性麻醉剂和琥珀酰胆碱)可能可以预防恶性高热(MH)的出现。重要的是要认识到潜在的并发症,并知道如何预防和管理患有这种疾病的患者。
    方法:一个5岁男孩(体重:8.8公斤;身高:63厘米)在间歇性发热(最高体温为39.3°C)和咳嗽五天后被送到儿科,加重1天,与此同时,他喉咙里有痰,但咳嗽不出来。该名儿童在一个月大的体检中被发现有运动障碍,然后遗传分析显示中枢核心疾病。在对症治疗的前提下,行支气管镜支气管肺泡灌洗治疗,以获得更好的治疗效果。
    结论:中枢核心疾病患者尤其是恶性高热,因此,在麻醉诱导前,有足够的预防措施来预防和治疗MH。麻醉医师需要制定足够的术前麻醉管理策略,以确保中央核心疾病患儿进行支气管镜支气管肺泡灌洗的安全性。患儿经抗炎、平喘治疗1周后出院。
    结论:我们总结了中枢核心疾病患者的麻醉预防措施和管理,同时对支气管镜下支气管肺泡灌洗的麻醉重点提出了一些建议。
    BACKGROUND: We described the perioperative management of a child patient with central core disease for bronchoscopy with bronchoalveolar lavage. It is safe to avoid triggering agents (volatile anesthetics and succinylcholine) probably in preventing this appearance of malignant hyperthermia (MH). It is important to recognize potential complications and know how to prevent and manage them in patients with this condition.
    METHODS: A 5-year-old boy (weight: 8.8 kg; height: 63 cm) presented to the pediatric department after five days of intermittent fever (highest body temperature is 39.3 °C) and cough, and aggravation 1 day, meanwhile he had phlegm in throat but he couldn\'t cough out. The child was found to have motor retardation at his one-month-old physical examination, then genetic analysis showed central core disease. Bronchoscopy with bronchoalveolar lavage was performed for better treatment under the premise of symptomatic treatment.
    CONCLUSIONS: The patients with central core disease are particularly to develop malignant hyperthermia, so adequate precautions are in place to prevent and treat MH before anesthetic induction. The anesthesiologists need to make adequate preoperative anesthesia management strategies to ensure the safety of the child with central core disease for bronchoscopy with bronchoalveolar lavage. The child was discharged from the hospital one week after anti-inflammatory and anti-asthmatic treatment.
    CONCLUSIONS: We summarized the anesthetic precautions and management in patients with central core disease, meanwhile we offered some suggestions about anesthetic focus on bronchoscopy with bronchoalveolar lavage.
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  • 文章类型: Journal Article
    背景:恶性热疗(MH)是一种罕见的,由挥发性麻醉剂和琥珀酰胆碱引发的骨骼肌细胞钙稳态危及生命的疾病,导致高代谢反应.致病性ryanodine受体1(RYR1)基因变异至关重要。易患MH的患者应避免引发剂,和全静脉麻醉(TIVA)是首选。雷马唑仑对疑似MH患者是安全的。
    方法:我们介绍了1例基因证实的MH无MH发展的患者中瑞马唑仑治疗的首例病例。一名72岁有MH家族史的男子接受了基于雷米咪唑的TIVA。在获得知情同意后,进行了肌肉活检和基因检测.术中和术后,患者无MH征象。表明RYR1通道释放钙的功能增强,基因检测揭示了RYR1的致病变体。
    结论:基于雷马唑仑的TIVA在确认诊断为MH的患者中是安全的。
    BACKGROUND: Malignant hyperthermia (MH) is a rare, life-threatening disorder of calcium homeostasis in skeletal muscle cells that is triggered by volatile anesthetics and succinylcholine, leading to a hypermetabolic reaction. The pathogenic ryanodine receptor 1 (RYR1) gene variant is critical. Patients susceptible to MH should avoid triggering agents, and total intravenous anesthesia (TIVA) is preferred. Remimazolam is safe in patients with suspected MH.
    METHODS: We present the first case of remimazolam treatment in a genetically confirmed patient with MH without MH development. A 72-year-old man with a family history of MH underwent remimazolam-based TIVA. After informed consent was obtained, a muscle biopsy and genetic testing were performed. Intraoperatively and postoperatively, the patient exhibited no signs of MH. An enhanced function of the RYR1 channel into releasing calcium was indicated, and the genetic testing revealed a pathogenic variant of RYR1.
    CONCLUSIONS: Remimazolam-based TIVA is safe in patients confirming the diagnosis of MH.
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  • 文章类型: Case Reports
    背景:恶性高热(MH)是一种罕见但严重的医学并发症,通常在全身麻醉或施用特定麻醉剂后出现。由于MH的频率不高,麻醉师通常缺乏足够的专业知识来识别和管理它,导致误诊和不当治疗。迫切需要通过利用相关工具来加强MH的诊断和管理。
    方法:在这种情况下,一名52岁的妇女在全身麻醉下接受了宫颈癌根治术,没有家族或重大病史。她在很短的时间内经历了潮气末二氧化碳(ETCO2)逐渐增加到最高75mmHg,体温从36.5升高到37.5°C,以及血气分析显示pH值为7.217。
    方法:麻醉师立即使用基于微信小程序的恶性高热国家远程应急系统(MH-NRES),得分为40分,这表明患者很可能患有MH。
    方法:我们立即停止七氟醚,并切换全静脉麻醉以维持全身麻醉,快速静脉输注丹曲林钠。
    结果:ETCO2和温度迅速下降到正常,随后手术成功完成,术后8天患者出院。
    结论:该经验可为MH-NRES的使用提供依据,提高麻醉医师处理术中MH的能力,增加患者的生存概率。
    BACKGROUND: Malignant hyperthermia (MH) is a rare yet serious medical complication that typically arises following general anesthesia or the administration of specific anesthetics. Due to the infrequency of MH, anesthesiologists often lack sufficient expertise in identifying and managing it, leading to misdiagnosis and inappropriate treatment. There is an urgent need to enhance the diagnosis and management of MH through the utilization of relevant tools.
    METHODS: In this case, a 52-year-old woman underwent radical cervical cancer surgery under general anesthesia, with no family or significant medical history. She experienced a gradual increase in end-tidal carbon dioxide (ETCO2) to a maximum of 75 mm Hg and a rise in body temperature from 36.5 to 37.5 °C in a very short period, as well as a blood gas analysis showing a pH of 7.217.
    METHODS: The anesthesiologist immediately used The WeChat applet-based National Remote Emergency System for Malignant Hyperthermia (MH-NRES), and the score was 40, which indicated that the patient was very likely to have MH.
    METHODS: We immediately discontinued sevoflurane and switched total intravenous anesthesia to maintain general anesthesia, with a rapid intravenous infusion of dantrolene sodium.
    RESULTS: The ETCO2 and the temperature quickly dropped to normal, followed by successful completion of the surgery, and the patient was discharged 8 days after surgery.
    CONCLUSIONS: The experience can provide a basis use of MH-NRES and improve the ability of anesthesiologists to deal with intraoperative MH as well as increase the survival probability of patients.
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  • 文章类型: Case Reports
    恶性高热是一种罕见但可能致命的疾病。我们介绍了2例2019年冠状病毒病(COVID-19)的年轻患者,需要因缺氧呼吸衰竭而插管,他们在插管后都出现了明显的高热,并怀疑患有恶性高热。然而,2例患者对保守治疗和丹曲林的反应不同.这些病例凸显了在管理COVID-19患者时插管并发症带来的挑战。
    Malignant hyperthermia is a rare but potentially fatal condition. We present 2 cases of young patients with coronavirus disease 2019 (COVID-19) requiring intubation for hypoxic respiratory failure who both developed significant hyperthermia post intubation and were suspected to have malignant hyperthermia. However, the 2 patients had different responses to conservative management and dantrolene. These cases highlight the increased challenge imposed by intubation complications when managing patients with COVID-19.
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