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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A 10 y/o girlChief complaint:Chest discomfort, vomiting and dry cough for one dayBrief HistoryGrowth & development:Weight: 22 kg (3rd-10th percentile)Height: 130 cm (25-50th percentile)Development milestone: within normal limitPast historyHand-foot-mouth disease in
2、1998Frequent URI and fever during childhoodNo drug or food allergyBrief HistoryFamily history:Her sister had fever and URI recently. Present IllnessFever and bilateral hand arthralgia attack once 1 month agoChest discomfort and cough since 9/11 afternoon, 2001Visit LMD and URI was toldVomiting and c
3、hest tightness on 9/12 0 AM and 5 AMPresent Illness9/12 morning, visit LMD again, ECG showed arrhythmiaRefer to 亞東 hospitalPresent IllnessFindings at 亞東 hospital Clear consciousness, ill-looking, pallor appearance, no cyanosis Irregular heart beat EKG: VPC bigeminyPresent IllnessLab. findings at 亞東
4、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/LPresent IllnessEchocardiogram at 亞東 hospital Multiple small VSDs, muscular trabecular type, at apex LV dyskinesia, LVEF 60-70% Mild TR, mild MRPresent IllnessManagement at 亞東 hospital Lidocaine iv dr
5、ip Dopamine 10 mg/kg/min Refer to NTUH (2pm)Physical 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 lipPhysical Examination Neck: jugular venous engorgement Chest: bilateral ba
6、sal rles Heart: irregularly irregular beats, distant heart sound no murmurPhysical Examination Abdomen: no hepatomegaly hypoactive bowel sound Extremities: freely movable cold and cyanotic poor capillary refillingInitial Lab DataCBC: WBC Hb Hct Plt 8840 12.7 37.2 % 160 K Seg 82.4%, Lym 13.8%, Eos 0.
7、1%BCS: BUN Cre Na K Cl Ca 12.8 0.63 141 4.5 104 2.41 Initial 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 Initial Lab DataEKG (9/12): Initial Lab DataEKG (9/12): Initial Lab DataEKG (9/12): Initial Lab DataE
8、chocardiogram (9/12):LV enlargementLVEF 45%Muscular VSDMild MR, TR, PR Echocardiogram (9/12)Course and TreatmentManagementFor cardiogenic shock: Dopamine, Dobutamin, Primacor, LasixFor ventricular arrhythmia: Amiodarone, Lidocaine, MgSO4For myocarditis: IVIG, Consider extracorporeal membranous oxyge
9、nator (ECMO) supportCourse and Treatment9/12 5pm (3 hr after admission)Progressive hypotensionSudden onset of coma, BP drop (pulseless)EKG: ventricular tachycardiaStart CPR (40 min)Start ECMO, transfer to SICUEKG (9/12, 5 PM)Course in SICUECMO settingV-A ECMO: 15 Fr Rt femoral artery, 19 Fr Rt femor
10、al vein by cutdownFlow: 2000 ml/minMean BP: 70 mmHgUrine output: 1.72 ml/kg/hrEchocardiogram (9/13)Course in SICUVT persistent 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 pul
11、satile wave formCourse in SICUEndomyocardial biopsy (9/14)Mild to moderate perivascular and interstitial lymphocyte infiltrationFoci of myocyte degeneration Interstitial edemaNo giant cell Compatible with acute myocarditisCourse in SICULA drain (9/17): To decompress LV, avoid thrombosisLA dome cannu
12、lation connecting to FV cannula ECMO FALAP: 22 mmHg 10 mmHgEchocardiogram (9/17)Course in 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 (+)Course in SICU9/19, 8am: gross hematuria and ECMO tu
13、be thrombosis reset ECMOProgressive dilated pupils, no light reflex, suspected hypoxic encephalopathyRemove ECMO on 9/23 (10th day)Lab data9/129/139/149/159/169/17TnI31.962.41007437.3CK104091242342126759138647026CK-MB196368687403207101Cre0.630.590.560.50.470.51Bil1.240.510.651.361.51.35Lab DataLab D
14、ataSerology 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 & BLab DataCulture:Throat swab (9/12): Staphylococcus aureusNasal swab (9/12): Staphylococcus aureus, Viridans strep
15、tococciBlood (9/19): Staphylococcus epidermidisDiscussionDiagnostic 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: 4-7%Cardiac disorders: 0-4%Diagnostic approachHx: cou
16、gh, vomitingPE: hypotension jugular venous distention tachycardia irregular heart beat basal rles poor peripheral perfusion Cardiovascular compromise Diagnostic approachFlu-like illness, arrhythmia, cardiovascular compromise Acute myocarditis highly suspectedD/D: Dilated cardiomyopathy Anomalous lef
17、t coronary artery Chronic tachyarrhythmia Pericarditis Diagnostic approachEKG: VPC bigeminy, ventricular tachycardiaST-segment changeElevated cardiac enzymeEchocardiogram: marked LV dyskinesiaEndomyocardial biopsyLymphocyte infiltrationMyocyte degeneration Acute myocarditis confirmedClinical classif
18、ication of myocarditisFulminantAcuteChronic activeChronic persistentInitial presentationShock, severe LV dysfuntionCHFCHFNormal LV functionEndomyocardial biopsyMultifocal active myocarditisActive or borderline myocarditisActive or borderline myocarditisActive or borderline myocarditisNature historyC
19、omplete recovery or deathIncomplete recovery or DCMDCMNormal LV functionMyocarditis: an enigmatic disease!Dark side of the myocarditisInitial non-specific symptoms Difficult to establish the diagnosisEtiology hard to findComplexity of pathogenesisOften refractory to conventional treatmentDark side o
20、f the myocarditisInitial non-specific symptoms Similar to patients with sepsis, bronchiolitis, pneumonia, gastroenteritis, hepatitis, and renal failure etc.Aggressive fluid resuscitation may harm unstable patientsRapid progression in fulminant myocarditisDark side of the myocarditisDifficult to esta
21、blish the diagnosisLimited sensitivity and specificity of changes in CXR, ECG, cardiac enzyme (Troponin level: more sensitive)Echocardiogram: LV dysfunction, often regionalEndomyocardial biopsy: as gold standard, but sensitivity 3-63%Dallas criteriaBorderline myocarditisActive myocarditisAm J Cadiov
22、asc Pathol 1987;1:3-14Dark side of the myocarditisEtiology hard to findVIRAL CAUSESEnterovirus Coxsackie A Coxsackie B Echovirus PoliovirusAdenovirus Cytomegalovirus Herpesvirus Influenza A Epstein-Barr virusVaricella Mumps Measles Parvovirus Rabies Hepatitis B,C Rubella Rubeola Respiratory syncytia
23、l virus Human immunodeficiency virusRickettsial Rickettsia ricketsii Rickettsia tsutsugamushiBacterial Meningococcus Klebsiella Leptospira Mycoplasma Salmonella Clostridia Tuberculosis Brucella Legionella pneumophila smallpox Streptococcus Protozoal Trypanosoma cruzi Toxoplasmosis Amebiasis Other pa
24、rasites Toxocara canis Schistosomiasis Hetereophyiasis Cysticercosis Echinococcus Visceral larva migrans Trichinosis Fungi and yeasts Actinomycosis Coccidiodomycosis Histoplasmosis Candida NONVIRAL CAUSES Dark side of the myocarditisEtiology hard to findToxic Scorpion Diphtheria Drugs Sulfonamides P
25、henylbutazone Cyclophosphamide Neomercazole Acetazolamide Amphotericin B Indomethacin Tetracycline Isoniazid Methyldopa Phenytoin PenicillinHypersensitivity/Autoimmune Rheumatoid arthritis Rheumatic fever Ulcerative colitis Systemic lupus erythematosus Mixed connective tissue disease Scleroderma Whi
26、pples disease Other Sarcoidosis Kawasaki disease CornstarchNONINFECTIOUS ETIOLOGIESDark side of the myocarditisEtiology hard to findPediatr Cardiol 2001;22:34-9Dark side of the myocarditisComplexity of pathogenesisNEJM 2000;343:1388-98Dark side of the myocarditisComplexity of pathogenesis Factors co
27、ntributing to host susceptibilityAutoantibodies: to adenosine nucleotide translocator, myosinExpression of cell adhesion molecules (ICAM-1)Expression of coxsackie-adenovirus receptor (CAR)Dark side of the myocarditisOften refractory to conventional treatmentStandard therapy: ACE inhibitor, inotropic
28、 agents, diuretics often not effective in fulminant myocarditisImmunosuppression: IVIG, steroids, cyclosporin still controversialBright side of the myocarditisGood long term prognosis of fulminant myocarditisImprovement of mechanical support: LVAD, BVAD, ECMOBright side of the myocarditisGood long t
29、erm prognosis of fulminant myocarditisNEJM 2000;342:690-5Bright side of the myocarditisGood long term prognosis of fulminant myocarditisBright side of the myocarditisGood long term prognosis of fulminant myocarditisWhy?Different viral agent?Different host response? Autoimmune in nature?Bright side o
30、f the myocarditisVentricular assistant device (VAD) & Extracorporeal membrane oxygenation (ECMO) Bright side of the myocarditisVAD and ECMO in fulminant myocarditis:Basically a reversible diseaseIndications: - Failing medical treatment ( inotropic requirement with poor perfusion) - Cardiac arrestBri
31、ght side of the myocarditisOutcome of VAD and ECMO used in fulminant myocarditis:J Thorac Cardiovasc Surg. 2001;112:440-8Future strategiesAntiviral agents: interferon, ribavirin, pleconarilVaccine: to specific virus, T-cell receptors,tolerance to myosinEarlier mechanical supportMore specific immunosuppression: OKT3, NO synthetase blocker, Clinical diagnosisFulminant myocarditis, possible viral origin, etiology?Cause of death: ECMO dysfunction, Hypoxic-ischemic encephalopathy secondary to circulatory collapseMyocarditis in recovery?Thanks for your attention!
