临床病理讨论会课件

臨床病理討論會小兒科：盧俊維醫師放射科：吳金珠醫師

病理科：蕭正祥醫師A10y/ogirlChiefcomplaint:Chestdiscomfort,vomitinganddrycoughforonedayBriefHistoryGrowth&development:Weight:22kg(3rd-10thpercentile)Height:130cm(25-50thpercentile)Developmentmilestone:withinnormallimitPasthistoryHand-foot-mouthdiseasein1998FrequentURIandfeverduringchildhoodNodrugorfoodallergyPresentIllnessFeverandbilateralhandarthralgiaattackonce1monthagoChestdiscomfortandcoughsince9/11afternoon,2001VisitLMDandURIwastoldVomitingandchesttightnesson9/120AMand5AMPresentIllness9/12morning,visitLMDagain,ECGshowedarrhythmiaReferto亞東hospitalPresentIllnessFindingsat亞東hospitalClearconsciousness,ill-looking,pallorappearance,nocyanosis

IrregularheartbeatEKG:VPCbigeminyPresentIllnessEchocardiogramat亞東hospitalMultiplesmallVSDs,muscular

trabeculartype,atapexLVdyskinesia,LVEF60-70%MildTR,mildMRPresentIllnessManagementat亞東hospital

LidocaineivdripDopamine10mg/kg/minRefertoNTUH(2pm)PhysicalExaminationPhysicalfindingsatNTUHConsciousness:lethargic,acuteill-lookingT/P/R:37/140/25BP80/46SaO297%HEENT:paleconjunctiva

anictericscleramildcyanoticlipPhysicalExamination

Abdomen:nohepatomegaly

hypoactivebowelsoundExtremities:freelymovablecoldandcyanoticpoorcapillaryrefillingInitialLabDataCBC:WBCHbHctPlt

884012.7

37.2%160K

Seg82.4%,Lym13.8%,Eos0.1%BCS:BUNCreNaKClCa12.80.631414.51042.41

InitialLabDataVBG:pHpCO2pO2HCO3BE7.3647.427.326.9+1.4Cardiacenzyme:

CPK(U/L)CK-MBTroponinI(ng/ml)1040196.5

31.9CRP:0.53mg/dl

InitialLabDataEKG(9/12):

InitialLabDataEKG(9/12):

InitialLabDataEchocardiogram(9/12):LVenlargementLVEF45%MuscularVSDMildMR,TR,PR

CourseandTreatmentManagementForcardiogenicshock:Dopamine,Dobutamin,Primacor,LasixForventriculararrhythmia:

Amiodarone,Lidocaine,MgSO4Formyocarditis:IVIG,Considerextracorporealmembranousoxygenator(ECMO)supportCourseandTreatment9/125pm(3hrafteradmission)ProgressivehypotensionSuddenonsetofcoma,BPdrop(pulseless)EKG:ventriculartachycardiaStartCPR(40min)StartECMO,transfertoSICUEKG(9/12,5PM)Echocardiogram(9/13)CourseinSICUVTpersistentdespiteofcardioversion,Lidocaine,Amiodarone,MgSO49/12~9/17:ECMO5daysPoorLVfunctionPersistentlungedema(CXR,clinically)TnIslowlydecreaseA-lineflatten,nopulsatilewaveformEchocardiogram(9/17)CourseinSICU9/18,4amAcutethrombosisatLAcannulaandECMOcircuitpoorflowCPRfor30min.andemergentre-setECMOtubingCons.AfterCPR:E1M1VTLightreflex(+)CourseinSICU9/19,8am:grosshematuriaandECMOtubethrombosisresetECMOProgressivedilatedpupils,nolightreflex,suspectedhypoxicencephalopathyRemoveECMOon9/23(10thday)Labdata9/129/139/149/159/169/17TnI31.962.4>1007437.3CK104091242342126759138647026CK-MB196368687403207101Cre0.630.590.560.50.470.51Bil1.240.510.651.361.51.35LabDataLabDataSerologystudy;MycoplasmapneumoniaIgM:(9/12)positive,(9/21)negativeOthervirologystudy:allnegativeCoxsackieA,CoxsackieB1-B6,CMVIgG&IgM,Enterovirus70,InfluenzaA&BLabDataCulture:Throatswab(9/12):StaphylococcusaureusNasalswab(9/12):Staphylococcusaureus,

ViridansstreptococciBlood(9/19):StaphylococcusepidermidisDiscussionDiagnosticapproach:CauseofchestpaininchildrenIdiopathic:12-45%Costochondritis:9-22%Musculoskeletaltrauma:21%Cough,asthma,pneumonia:15-21%Psychogenicfactors:5-9%GIdisorders:4-7%Cardiacdisorders:0-4%DiagnosticapproachHx:cough,vomitingPE:hypotension

jugularvenousdistention

tachycardia

irregularheartbeatbasalräles

poorperipheralperfusionCardiovascularcompromise

DiagnosticapproachFlu-likeillness,arrhythmia,cardiovascularcompromiseAcutemyocarditishighlysuspectedD/D:Dilatedcardiomyopathy

AnomalousleftcoronaryarteryChronictachyarrhythmiaPericarditis

DiagnosticapproachEKG:VPCbigeminy,ventriculartachycardiaST-segmentchangeElevatedcardiacenzymeEchocardiogram:markedLVdyskinesiaEndomyocardialbiopsyLymphocyteinfiltrationMyocytedegeneration

AcutemyocarditisconfirmedClinicalclassificationofmyocarditisFulminantAcuteChronicactiveChronicpersistentInitialpresentationShock,severeLVdysfuntionCHFCHFNormalLVfunctionEndomyocardialbiopsyMultifocalactivemyocarditisActiveorborderlinemyocarditisActiveorborderlinemyocarditisActiveorborderlinemyocarditisNaturehistoryCompleterecoveryordeathIncompleterecoveryorDCMDCMNormalLVfunctionMyocarditis:anenigmaticdisease!DarksideofthemyocarditisInitialnon-specificsymptoms

DifficulttoestablishthediagnosisEtiologyhardtofindComplexityofpathogenesisOftenrefractorytoconventionaltreatmentDarksideofthemyocarditisInitialnon-specificsymptoms

Similartopatientswithsepsis,bronchiolitis,pneumonia,gastroenteritis,hepatitis,andrenalfailureetc.AggressivefluidresuscitationmayharmunstablepatientsRapidprogressioninfulminant

myocarditisDarksideofthemyocarditisDifficulttoestablishthediagnosisLimitedsensitivityandspecificityofchangesinCXR,ECG,cardiacenzyme(Troponinlevel:moresensitive)Echocardiogram:LVdysfunction,oftenregionalEndomyocardialbiopsy:asgoldstandard,butsensitivity3-63%DallascriteriaBorderlinemyocarditisActivemyocarditisAmJCadiovascPathol1987;1:3-14DarksideofthemyocarditisEtiologyhardtofindVIRALCAUSESEnterovirusCoxsackieACoxsackieB

EchovirusPoliovirusAdenovirusCytomegalovirusHerpesvirusInfluenzaAEpstein-BarrvirusVaricellaMumpsMeaslesParvovirusRabiesHepatitisB,CRubellaRubeolaRespiratorysyncytialvirusHumanimmunodeficiencyvirusRickettsialRickettsiaricketsiiRickettsiatsutsugamushiBacterialMeningococcusKlebsiellaLeptospiraMycoplasmaSalmonellaClostridiaTuberculosisBrucellaLegionellapneumophilasmallpoxStreptococcusProtozoalTrypanosomacruziToxoplasmosisAmebiasisOtherparasitesToxocaracanisSchistosomiasisHetereophyiasisCysticercosisEchinococcusViscerallarvamigransTrichinosisFungiandyeasts

ActinomycosisCoccidiodomycosisHistoplasmosisCandidaNONVIRALCAUSESDarksideofthemyocarditisEtiologyhardtofindToxicScorpionDiphtheriaDrugsSulfonamidesPhenylbutazoneCyclophosphamideNeomercazoleAcetazolamideAmphotericinBIndomethacinTetracyclineIsoniazidMethyldopaPhenytoinPenicillinHypersensitivity/Autoimmune

Rheumatoidarthritis

Rheumaticfever

UlcerativecolitisSystemiclupuserythematosusMixedconnectivetissuediseaseSclerodermaWhipple'sdiseaseOtherSarcoidosis

Kawasakidisease

CornstarchNONINFECTIOUSETIOLOGIESDarksideofthemyocarditisEtiologyhardtofindPediatrCardiol2001;22:34-9DarksideofthemyocarditisComplexityofpathogenesisNEJM2000;343:1388-98DarksideofthemyocarditisComplexityofpathogenesisFactorscontributingtohostsusceptibilityAutoantibodies:toadenosinenucleotidetranslocator,myosinExpressionofcelladhesionmolecules(ICAM-1)Expressionofcoxsackie-adenovirusreceptor(CAR)DarksideofthemyocarditisOftenrefractorytoconventionaltreatmentStandardtherapy:ACEinhibitor,inotropicagents,diuretics–oftennoteffectiveinfulminantmyocarditisImmunosuppression:IVIG,steroids,cyclosporin–stillcontroversialBrightsideofthemyocarditisGoodlongtermprognosisoffulminantmyocarditisImprovementofmechanicalsupport:LVAD,BVAD,ECMOBrightsideofthemyocarditisGoodlongtermprognosisoffulminantmyocarditisNEJM2000;342:690-5BrightsideofthemyocarditisGoodlongtermprognosisoffulminantmyocarditisBrightsideofthemyocarditisGoodlongtermprognosisoffulminantmyocarditisWhy?Differentviralagent?Diff