恶性高热-教学文稿.ppt

单击此处编辑母版标题样式,编辑母版文本样式,第二级,第三级,第四级,第五级,*,*,恶性高热 2017,概述,恶性高热（,Malignant Hyperthermia,，,MH,）是一个基因性临床病理综合征，患者平时无异常表现，在全麻过程中接触挥发性吸入麻醉药和去极化肌松药（琥珀酰胆碱）后出现骨骼肌强直性收缩，产生大量能量，导致体温持续快速增高，在没有特异性治疗药物的情况下，一般的临床降温措施难以控制体温的增高，最终可导致患者死亡。,在一般人群中，预计,MH,发作的发生率为每,100,000,次麻醉剂给予,1,例,Exposure of an individual who has a genetic susceptibility(ryanodine receptor,RYR1,or dihydropyridine receptor,DHP,mutation)to an anesthetic triggering agent(ie,volatile inhalational anesthetic agent,succinylcholine,or both)may result in malignant hyperthermia.This reaction is caused by an altered calcium balance between the lumen of the sarcoplasmic reticulum(SR)and the sarcoplasm.Normally,muscle cell depolarization is sensed by the DHP receptor,which is thought to signal RYR1 opening by a direct physical connection.In malignant hyperthermia,accumulation of abnormally high levels of calcium in the sarcoplasm causes uncontrolled anaerobic and aerobic metabolism and sustained muscle cell contraction.This results in the clinical manifestations of respiratory acidosis,metabolic acidosis,muscle rigidity,and hyperthermia.If the process continues unabated,adenosine triphosphate(ATP)depletion eventually causes widespread muscle fiber hypoxia(cell death,rhabdomyolysis),which manifests clinically as hyperkalemia and myoglobinuria and an increase in creatine kinase.Dantrolene sodium binds to RYR1,causing it to favor the closed state,thereby reversing the uninhibited flow of calcium into the sarcoplasm.,Larach MG,Gronert GA,Allen GC,et al.Clinical presentation,treatment,and complications of malignant hyperthermia in North America from 1987 to 2006.Anesth Analg 2010;110:498.,发病机制,骨骼肌细胞膜发育缺陷,诱发药物,肌细胞浆内钙离子浓度迅速增高，使肌肉挛缩,产热急剧增加，体温迅速升高,酸中毒,高血钾,低血氧,心律失常,死亡（,MOF,，,DIC,）,诱发药物,吸入麻醉药：乙醚、氟烷、安氟烷、异氟烷、地氟烷和七氟烷。,去极化肌松药：琥珀胆碱,其它有过报道的药物：氯胺酮、利多卡因和氟哌啶醇。,Typical order of appearance of clinical signs of malignant hyperthermia,Masseter spasm(in some cases),Hypercarbia,Sinus tachycardia,Generalized muscular rigidity,Tachypnea,Cyanosis,Rapidly increasing temperature,Elevated temperature,Sweating,Ventricular tachycardia,Cola-colored urine,Ventricular fibrillation,Excessive bleeding,Larach MG,Gronert GA,Allen GC,et al.Clinical presentation,treatment,and complications of malignant hyperthermia in North America from 1987 to 2006.Anesth Analg 2010;110:498.,高热,高钾血症相关的心电图改变,心室异位起搏,/,二联率,室性心动过速,/,心室颤动,肌红蛋白尿,出血过多,高碳酸血症,窦性心动过速,咬肌强直,(MMR),全身肌肉强直,体征,Clinical sign,Percentage with sign,Hypercarbia,92.2,Sinus tachycardia,72.9,Rapidly increasing temperature,64.7,Elevated temperature,52.2,Generalized muscular rigidity,40.8,Tachypnea,27.1,Masseter spasm,26.7,Sweating,17.6,Cola-colored urine,13.7,Cyanosis,9.4,Ventricular tachycardia,3.5,Excessive bleeding,2.7,Ventricular fibrillation,2.4,Larach MG,Gronert GA,Allen GC,et al.Clinical presentation,treatment,and complications of malignant hyperthermia in North America from 1987 to 2006.Anesth Analg 2010;110:498.,实验室检查,Laboratory study,Typical value in MH,Patient conditions,Creatine kinase,20,000 international units,With succinylcholine,10,000 international units,Without succinylcholine,Urine myoglobin,60 mcg/L,Serum myoglobin,170 mcg/L,Serum K,6 mEq/L,Without renal failure,PaCO,2,60 mmHg,During controlled ventilation,65 mmHg,During spontaneous ventilation,Arterial pH,10L/min,）进行过度通气（分钟通气量达正常值的,23倍）,病情汇报，寻求帮助,换用非诱发药物维持麻醉（全凭静脉麻醉,TIVA,）,告知外科医生，终止或推迟手术,处理,丹曲林：,尽快获取足够丹曲林（,3650,瓶）,静脉注射丹曲林首剂量,2.5mg/kg,，,随后再静脉内单次快速给予,1mg/kg,，并重复该剂量直到急性,MH,的体征消退。,（每瓶丹曲林,20mg,以,60ml,灭菌注射用水溶解。注意：,禁用生理盐水或葡萄糖溶液溶解丹曲林,）,根据病情发展，每,4-6,小时静注或静滴追加丹曲林,1mg/kg,用药时间至少不短于,24,小时，直至体温退烧或血,CK,下降，心血管系统功能稳定。,最后被观察到的急性,MH,体征出现后持续使用,24-48,小时,。,支持治疗,监测和治疗高钾血症,(,即钙、碳酸氢盐、胰岛素,-,葡萄糖,),监测和治疗酸中毒；考虑碳酸氢盐。,持续监测核心温度,(,如，食管、鼓室、直肠体温计探头,),（,38.5,以下,）,留置导尿管，监测尿液颜色和尿量,（,CK,值通常会在,MH,发病大约,14,小时后达到峰值,）,监测各肌肉筋膜室以预防急性筋膜室综合征,采取措施来防止肌红蛋白尿引起的肾衰竭,(,即补液、利尿剂、碳酸氢盐,),监控,DIC,死亡率,常规使用,ETCO,2,监测和能获取,丹曲林,治疗,，死亡率,1%-17%,。,未接受体温监测的患者死亡的可能性至少是接受了核心体温监测的患者的,2,倍。,所有的死亡均发生于体温的峰值为,38.9,或更高的患者中。,增加,MH,患者发生心搏骤停和死亡的风险的其他因素包括高龄、共存疾病、肌肉发达体型,(,如年轻男性,),和发生了,DIC,此课件下载可自行编辑修改，仅供参考！感谢您的支持，我们努力做得更好！谢谢,