1、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：蕭正祥醫師临床病理讨论会课件1A 10 y/o girlChief complaint:Chest discomfort,vomiting and dry cough for one day临床病理讨论会课件2Brief HistoryGrowth&development:Weight:22 kg(3rd-10th percentile)Height:130 cm(25-50th percentile)Development milestone:within normal limitPast historyHand-foot-mouth
2、 disease in 1998Frequent URI and fever during childhoodNo drug or food allergy临床病理讨论会课件3Brief HistoryFamily history:Her sister had fever and URI recently.临床病理讨论会课件4Present IllnessFever and bilateral hand arthralgia attack once 1 month agoChest discomfort and cough since 9/11 afternoon,2001Visit LMD
3、and URI was toldVomiting and chest tightness on 9/12 0 AM and 5 AM临床病理讨论会课件5Present Illness9/12 morning,visit LMD again,ECG showed arrhythmiaRefer to 亞東 hospital临床病理讨论会课件6Present IllnessFindings at 亞東 hospital Clear consciousness,ill-looking,pallor appearance,no cyanosis Irregular heart beat EKG:VPC
4、 bigeminy临床病理讨论会课件7Present IllnessLab.findings at 亞東 hospital WBC 9000/mm3,Hb 13.5 g/dl BUN 11 mg/dl,Cre 0.6 mg/dl GOT 25 U/L,CK 665 U/L,CK-MB 175 U/L临床病理讨论会课件8Present IllnessEchocardiogram at 亞東 hospital Multiple small VSDs,muscular trabecular type,at apex LV dyskinesia,LVEF 60-70%Mild TR,mild MR临床
5、病理讨论会课件9Present IllnessManagement at 亞東 hospital Lidocaine iv drip Dopamine 10 mg/kg/min Refer to NTUH(2pm)临床病理讨论会课件10Physical ExaminationPhysical findings at NTUH Consciousness:lethargic,acute ill-looking T/P/R:37/140/25 BP 80/46 SaO2 97%HEENT:pale conjunctiva anicteric sclera mild cyanotic lip临床病理
6、讨论会课件11Physical Examination Neck:jugular venous engorgement Chest:bilateral basal rles Heart:irregularly irregular beats,distant heart sound no murmur临床病理讨论会课件12Physical Examination Abdomen:no hepatomegaly hypoactive bowel sound Extremities:freely movable cold and cyanotic poor capillary refilling临床
7、病理讨论会课件13Initial Lab DataCBC:WBC Hb Hct Plt 8840 12.7 37.2%160 K Seg 82.4%,Lym 13.8%,Eos 0.1%BCS:BUN Cre Na K Cl Ca 12.8 0.63 141 4.5 104 2.41 临床病理讨论会课件14Initial Lab DataVBG:pH pCO2 pO2 HCO3 BE 7.36 47.4 27.3 26.9 +1.4Cardiac enzyme:CPK(U/L)CK-MB Troponin I(ng/ml)1040 196.5 31.9CRP:0.53 mg/dl 临床病理讨论
8、会课件15Initial Lab DataEKG(9/12):临床病理讨论会课件16Initial Lab DataEKG(9/12):临床病理讨论会课件17Initial Lab DataEKG(9/12):临床病理讨论会课件18Initial Lab DataEchocardiogram(9/12):LV enlargementLVEF 45%Muscular VSDMild MR,TR,PR 临床病理讨论会课件19Echocardiogram(9/12)临床病理讨论会课件20Course and TreatmentManagementFor cardiogenic shock:Dopam
9、ine,Dobutamin,Primacor,LasixFor ventricular arrhythmia:Amiodarone,Lidocaine,MgSO4For myocarditis:IVIG,Consider extracorporeal membranous oxygenator(ECMO)support临床病理讨论会课件21Course and Treatment9/12 5pm(3 hr after admission)Progressive hypotensionSudden onset of coma,BP drop(pulseless)EKG:ventricular t
10、achycardiaStart CPR(40 min)Start ECMO,transfer to SICU临床病理讨论会课件22EKG(9/12,5 PM)临床病理讨论会课件23Course in SICUECMO settingV-A ECMO:15 Fr Rt femoral artery,19 Fr Rt femoral vein by cutdownFlow:2000 ml/minMean BP:70 mmHgUrine output:1.72 ml/kg/hr临床病理讨论会课件24Echocardiogram(9/13)临床病理讨论会课件25Course in SICUVT per
11、sistent despite of cardioversion,Lidocaine,Amiodarone,MgSO4 9/12 9/17:ECMO 5 daysPoor LV functionPersistent lung edema(CXR,clinically)TnI slowly decreaseA-line flatten,no pulsatile wave form临床病理讨论会课件26Course in SICUEndomyocardial biopsy(9/14)Mild to moderate perivascular and interstitial lymphocyte
12、infiltrationFoci of myocyte degeneration Interstitial edemaNo giant cell Compatible with acute myocarditis临床病理讨论会课件27Course in SICULA drain(9/17):To decompress LV,avoid thrombosisLA dome cannulation connecting to FV cannula ECMO FALAP:22 mmHg 10 mmHg临床病理讨论会课件28Echocardiogram(9/17)临床病理讨论会课件29Course i
13、n SICU9/18,4am Acute thrombosis at LA cannula and ECMO circuit poor flowCPR for 30 min.and emergent re-set ECMO tubing Cons.After CPR:E1M1VTLight reflex(+)临床病理讨论会课件30Course in SICU9/19,8am:gross hematuria and ECMO tube thrombosis reset ECMOProgressive dilated pupils,no light reflex,suspected hypoxic
14、 encephalopathyRemove ECMO on 9/23(10th day)临床病理讨论会课件31Lab data9/129/139/149/159/169/17TnI31.962.41007437.3CK104091242342126759138647026CK-MB196368687403207101Cre0.630.590.560.50.470.51Bil1.240.510.651.361.51.35临床病理讨论会课件32Lab DataDATE9/229/209/189/169/149/12Troponin I(ng/ml)120100806040200DATE9/229/
15、209/189/169/149/12U/L8006004002000GOTCK-MB临床病理讨论会课件33Lab DataSerology study;Mycoplasma pneumonia IgM:(9/12)positive,(9/21)negativeOther virology study:all negative Coxsackie A,Coxsackie B1-B6,CMV IgG&IgM,Enterovirus 70,Influenza A&B临床病理讨论会课件34Lab DataCulture:Throat swab(9/12):Staphylococcus aureusNa
16、sal swab(9/12):Staphylococcus aureus,Viridans streptococciBlood(9/19):Staphylococcus epidermidis临床病理讨论会课件35DiscussionDiagnostic approach:Cause of chest pain in childrenIdiopathic:12-45%Costochondritis:9-22%Musculoskeletal trauma:21%Cough,asthma,pneumonia:15-21%Psychogenic factors:5-9%GI disorders:4-
17、7%Cardiac disorders:0-4%临床病理讨论会课件36Diagnostic approachHx:cough,vomitingPE:hypotension jugular venous distention tachycardia irregular heart beat basal rles poor peripheral perfusion Cardiovascular compromise 临床病理讨论会课件37Diagnostic approachFlu-like illness,arrhythmia,cardiovascular compromise Acute my
18、ocarditis highly suspectedD/D:Dilated cardiomyopathy Anomalous left coronary artery Chronic tachyarrhythmia Pericarditis 临床病理讨论会课件38Diagnostic approachEKG:VPC bigeminy,ventricular tachycardiaST-segment changeElevated cardiac enzymeEchocardiogram:marked LV dyskinesiaEndomyocardial biopsyLymphocyte in
19、filtrationMyocyte degeneration Acute myocarditis confirmed临床病理讨论会课件39Clinical classification of myocarditisFulminantAcuteChronic activeChronic persistentInitial presentationShock,severe LV dysfuntionCHFCHFNormal LV functionEndomyocardial biopsyMultifocal active myocarditisActive or borderline myocar
20、ditisActive or borderline myocarditisActive or borderline myocarditisNature historyComplete recovery or deathIncomplete recovery or DCMDCMNormal LV function临床病理讨论会课件40Myocarditis:an enigmatic disease!临床病理讨论会课件41Dark side of the myocarditisInitial non-specific symptoms Difficult to establish the diag
21、nosisEtiology hard to findComplexity of pathogenesisOften refractory to conventional treatment临床病理讨论会课件42Dark side of the myocarditisInitial non-specific symptoms Similar to patients with sepsis,bronchiolitis,pneumonia,gastroenteritis,hepatitis,and renal failure etc.Aggressive fluid resuscitation ma
22、y harm unstable patientsRapid progression in fulminant myocarditis临床病理讨论会课件43Dark side of the myocarditisDifficult to establish the diagnosisLimited sensitivity and specificity of changes in CXR,ECG,cardiac enzyme(Troponin level:more sensitive)Echocardiogram:LV dysfunction,often regionalEndomyocardi
23、al biopsy:as gold standard,but sensitivity 3-63%临床病理讨论会课件44Dallas criteriaBorderline myocarditisActive myocarditisAm J Cadiovasc Pathol 1987;1:3-14临床病理讨论会课件45Dark side of the myocarditisEtiology hard to findVIRAL CAUSESEnterovirus Coxsackie A Coxsackie B Echovirus PoliovirusAdenovirus Cytomegaloviru
24、s Herpesvirus Influenza A Epstein-Barr virusVaricella Mumps Measles Parvovirus Rabies Hepatitis B,C Rubella Rubeola Respiratory syncytial virus Human immunodeficiency virusRickettsial Rickettsia ricketsii Rickettsia tsutsugamushiBacterial Meningococcus Klebsiella Leptospira Mycoplasma Salmonella Clo
25、stridia Tuberculosis Brucella Legionella pneumophila smallpox Streptococcus Protozoal Trypanosoma cruzi Toxoplasmosis Amebiasis Other parasites Toxocara canis Schistosomiasis Hetereophyiasis Cysticercosis Echinococcus Visceral larva migrans Trichinosis Fungi and yeasts Actinomycosis Coccidiodomycosi
26、s Histoplasmosis Candida NONVIRAL CAUSES 临床病理讨论会课件46Dark side of the myocarditisEtiology hard to findToxic Scorpion Diphtheria Drugs Sulfonamides Phenylbutazone Cyclophosphamide Neomercazole Acetazolamide Amphotericin B Indomethacin Tetracycline Isoniazid Methyldopa Phenytoin PenicillinHypersensitiv
27、ity/Autoimmune Rheumatoid arthritis Rheumatic fever Ulcerative colitis Systemic lupus erythematosus Mixed connective tissue disease Scleroderma Whipples disease Other Sarcoidosis Kawasaki disease CornstarchNONINFECTIOUS ETIOLOGIES临床病理讨论会课件47Dark side of the myocarditisEtiology hard to findPediatr Ca
28、rdiol 2001;22:34-9临床病理讨论会课件48Dark side of the myocarditisComplexity of pathogenesisNEJM 2000;343:1388-98临床病理讨论会课件49Dark side of the myocarditisComplexity of pathogenesis Factors contributing to host susceptibilityAutoantibodies:to adenosine nucleotide translocator,myosinExpression of cell adhesion m
29、olecules(ICAM-1)Expression of coxsackie-adenovirus receptor(CAR)临床病理讨论会课件50Dark side of the myocarditisOften refractory to conventional treatmentStandard therapy:ACE inhibitor,inotropic agents,diuretics often not effective in fulminant myocarditisImmunosuppression:IVIG,steroids,cyclosporin still con
30、troversial临床病理讨论会课件51Bright side of the myocarditisGood long term prognosis of fulminant myocarditisImprovement of mechanical support:LVAD,BVAD,ECMO临床病理讨论会课件52Bright side of the myocarditisGood long term prognosis of fulminant myocarditisNEJM 2000;342:690-5临床病理讨论会课件53Bright side of the myocarditisGo
31、od long term prognosis of fulminant myocarditis临床病理讨论会课件54Bright side of the myocarditisGood long term prognosis of fulminant myocarditisWhy?Different viral agent?Different host response?Autoimmune in nature?临床病理讨论会课件55Bright side of the myocarditisVentricular assistant device(VAD)&Extracorporeal me
32、mbrane oxygenation(ECMO)临床病理讨论会课件56Bright side of the myocarditisVAD and ECMO in fulminant myocarditis:Basically a reversible diseaseIndications:-Failing medical treatment(inotropic requirement with poor perfusion)-Cardiac arrest临床病理讨论会课件57Bright side of the myocarditisOutcome of VAD and ECMO used i
33、n fulminant myocarditis:J Thorac Cardiovasc Surg.2001;112:440-8临床病理讨论会课件58Future strategiesAntiviral agents:interferon,ribavirin,pleconarilVaccine:to specific virus,T-cell receptors,tolerance to myosinEarlier mechanical supportMore specific immunosuppression:OKT3,NO synthetase blocker,临床病理讨论会课件59Clinical diagnosisFulminant myocarditis,possible viral origin,etiology?Cause of death:ECMO dysfunction,Hypoxic-ischemic encephalopathy secondary to circulatory collapseMyocarditis in recovery?临床病理讨论会课件60Thanks for your attention!临床病理讨论会课件61
