Kaur Harleen, Katyal Nakul, Yelam Anudeep, Kumar Keerthana, Srivastava Hirsch, Govindarajan Raghav
Harleen Kaur, MBBS, Nakul Katyal, MD, Anudeep Yelam, MBBS, Keerthana Kumar, MBBS, and Raghav Govindarajan, MD, MSMA member since 2013, are in the Department of Neurology, University of Missouri School of Medicine.
Mo Med. 2019 Mar-Apr;116(2):154-159.
Malignant Hyperthermia (MH) is a life-threatening pharmacogenetic disorder which results from exposure to volatile anesthetic agents and depolarizing muscle relaxants. It manifests as a hypermetabolic response resulting in tachycardia, tachypnea, hyperthermia, hypercapnia, acidosis, muscle rigidity and rhabdomyolysis. An increase in the end-tidal carbon dioxide is one of the earliest diagnostic signs. Dantrolene sodium is effective in the management of MH, and should be available whenever general anesthesia is administered. This review also aims to highlight the genetics and pathology of MH, along with its association with various inherited myopathy syndromes like central core disease, multi-mini core disease, Native-American myopathy, and King-Denborough syndrome.
恶性高热(MH)是一种危及生命的药物遗传疾病,由接触挥发性麻醉剂和去极化肌松药引起。其表现为高代谢反应,导致心动过速、呼吸急促、高热、高碳酸血症、酸中毒、肌肉强直和横纹肌溶解。呼气末二氧化碳增加是最早的诊断体征之一。丹曲林钠对恶性高热的治疗有效,在进行全身麻醉时应随时可用。本综述还旨在强调恶性高热的遗传学和病理学,以及它与各种遗传性肌病综合征的关联,如中央轴空病、多微小轴空病、美洲原住民肌病和金-丹博罗综合征。