Rüffert H, Wehner M, Deutrich C, Olthoff D
Klinik für Anästhesiologie und Intensivtherapie, Universitätsklinikum Leipzig AöR, Liebigstr. 20, 04103 Leipzig.
Anaesthesist. 2007 Sep;56(9):923-9. doi: 10.1007/s00101-007-1218-5.
The term "malignant hyperthermia" (MH), regarded as the typical anaesthetic disease, refers to a clinical syndrome of varying intensity (from abortive courses to fulminant crises) and develops only under exposure of certain triggering substances or mechanisms. MH is caused by a defect in the ryanodine receptor subtype 1, which can often be proved genetically. Furthermore, it may also be generated by other mechanisms which disturb the membranous integrity of skeletal muscle cells (e.g. some myotonias, muscular dystrophies, malformation syndromes). Hyperthermia is only one of a number of life-threatening symptoms that may occur during a fulminant crisis, which ultimately results from an excessive release of calcium into the cytoplasm of muscle cells. Due to a current good knowledge about classical triggers, symptoms and therapeutic interventions, a clinical MH presentation may successfully be treated in the perioperative period. However, it appears to be likely that there are unreported cases outside hospitals since atypical courses or alternative MH triggers (e.g. alcohol, drugs, physical stress) may impair the correct diagnosis. In contrast severe hyperthermia can also arise from other drug-induced diseases, e.g. the neuroleptic malignant syndrome or the serotonin syndrome.
“恶性高热”(MH)这一术语被视为典型的麻醉疾病,指的是一种强度各异的临床综合征(从顿挫型病程到暴发型危象),且仅在接触某些触发物质或机制时才会发生。MH 由 1 型兰尼碱受体缺陷引起,这种缺陷往往可通过基因检测证实。此外,它也可能由其他干扰骨骼肌细胞膜完整性的机制引发(如某些肌强直、肌肉萎缩症、畸形综合征)。高热只是暴发型危象期间可能出现的众多危及生命的症状之一,而这最终是由于钙过度释放到肌肉细胞胞质中所致。鉴于目前对经典触发因素、症状及治疗干预措施有充分了解,临床 MH 表现可在围手术期得到成功治疗。然而,由于非典型病程或其他 MH 触发因素(如酒精、药物、身体应激)可能影响正确诊断,医院外可能存在未报告的病例。相比之下,严重高热也可能由其他药物诱发的疾病引起,如抗精神病药恶性综合征或血清素综合征。