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1、CaseReportCASEAn8-day-oldboywasscheduledbilateralnasopharyngealtubeapplicationundergeneralanesthesiaPreoperativeexamination:Laboratorytests√X-ray√b-scanultrasound√ECG√Medicalhistory：scheduledforchoanalatresiaevaluationundergeneralanesthesiaoneweekagobodyexamin
2、ation：syndromicfacialappearance，micrognathia,low-setears,popeyedappearance,hypertelorism,palpebralfissures,cryptrochidism,clinodactyly,andbilateralhearingloss小颌畸形,低位耳,双眼突出,眼距过宽,睑裂,隐睾,小指内弯,双边听力损失CASEsevoflurane7-8%remifentanil2?gIntubationAnesthesialasted35minu
3、teswithoutmusclerelaxantExtubationTheprocedureendedwithoutanyproblemFollowup:temperatureincreasedto42.5∘C, heartrateincreasedto250/min,andrespiratorydistressdeveloped.Creatininephosphokinaselevelsreached 929IU/L,andhyperpotassemiadeveloped.Bloodgasanalysis rev
4、ealedhypoxemia(SO2<85%),respiratoryacidosis (PaCO2>60mmHg)andmetabolicacidosis(basedeficit> 10mEq/L)CASETreatmentDantrolenesodiumwasgivenorallybodytemperatureminimallydecreased respiratorydistresscontinuedIntubatedandmechanicalventilationDantrolensodium2.5mg/k
5、gwasgiven intravenouslywith6-hoursintervalsfor2daystemperaturedecreasedExtubatedandCPAPwasdone10-hoursperiodofintubationSpo2PACO2CaseReportDelayedOnsetMalignantHyperthermiaafterSevofluraneMalignantHyperthermiaMalignantHyperthermiaMalignantHyperthermiaHHHOWMali
6、gnantHyperthermia?Epidemiology?Highrisk？Clinicalfeatures？Laboratorytests？Pathophysiology?MalignantHyperthermia恶性高热（MalignantHyperthermia，MH）是目前所知的唯一可由常规麻醉用药引起围手术期死亡的遗传性疾病。它是一种亚临床肌肉病，即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（如氟烷、安氟醚、异氟醚等）和去极化肌松药（琥珀胆碱）后出现骨骼肌强直性收缩，产生大量能量，导致体温持
7、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。EpidemiologyEpidemiologyAudltFemaleForeignChildMaleChinaAgeGenderMortality201220062002Highrisk恶性高热家族病史中央轴空病特发性脊柱侧弯多小核疾病King综合征（表现为侏儒症,智力发育迟缓,肌肉骨骼异常）斜视、上睑下垂脐疝腹股沟疝在其它外科疾病中也有散在报道Clinicalfeatures突然发生的高碳酸血症；体温急剧升高，可
8、达45℃～46℃；骨骼肌僵直；咬肌强直（massetermusclerigidity，MMR）心动过速心律失常血压异常呼吸急促皮肤花斑特异性表现（极高特异性）（贯穿始终，无特异性）儿童接受七氟烷和琥珀胆碱诱导后发生MMR的概率为1%，发生MMR后利用血气改变确定MH的发生率为15%，而利用组织活检证实50%的MMR患者MH易感。（最早出现）Laborator
