臨床病理討論會課件

1、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 1998Frequent URI and fever during

2、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 chest tightness on 9/12 0 AM and 5

3、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 hospital WBC 9000/mm3, Hb 13.5 g/d

4、l 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 drip Dopamine 10 mg/kg/min Refer to

5、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 basal rles Heart: irregularly irregu

6、lar 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 0.1%BCS: BUN Cre Na K Cl Ca 12.8 0.6

7、3 141 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 DataEchocardiogram (9/12):LV enlargemen

8、tLVEF 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 oxygenator (ECMO) supportCourse and Tre

9、atment9/12 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 femoral vein by cutdownFlow: 2000 ml/mi

10、nMean 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 pulsatile wave formCourse in SICUEndo

11、myocardial 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 cannulation connecting to FV cannula EC

12、MO 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 tube thrombosis reset ECMOProgressiv

13、e 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 DataSerology study;Mycoplasma pneum

14、onia 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 streptococciBlood (9/19): Staphylococcu

15、s 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: cough, vomitingPE: hypotension jugula

16、r 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 left coronary artery Chronic tachyarr

17、hythmia Pericarditis Diagnostic approachEKG: VPC bigeminy, ventricular tachycardiaST-segment changeElevated cardiac enzymeEchocardiogram: marked LV dyskinesiaEndomyocardial biopsyLymphocyte infiltrationMyocyte degeneration Acute myocarditis confirmedClinical classification of myocarditisFulminantAcu

18、teChronic activeChronic persistentInitial presentationShock, severe LV dysfuntionCHFCHFNormal LV functionEndomyocardial biopsyMultifocal active myocarditisActive or borderline myocarditisActive or borderline myocarditisActive or borderline myocarditisNature historyComplete recovery or deathIncomplet

19、e 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 of the myocarditisInitial non-speci

20、fic 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 establish the diagnosisLimited sensiti

21、vity 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 Cadiovasc Pathol 1987;1:3-14Dark side of

22、 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 syncytial virus Human immunodeficiency vir

23、usRickettsial Rickettsia ricketsii Rickettsia tsutsugamushiBacterial Meningococcus Klebsiella Leptospira Mycoplasma Salmonella Clostridia Tuberculosis Brucella Legionella pneumophila smallpox Streptococcus Protozoal Trypanosoma cruzi Toxoplasmosis Amebiasis Other parasites Toxocara canis Schistosomi

24、asis 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 Phenylbutazone Cyclophosphamide Neo

25、mercazole Acetazolamide Amphotericin B Indomethacin Tetracycline Isoniazid Methyldopa Phenytoin PenicillinHypersensitivity/Autoimmune Rheumatoid arthritis Rheumatic fever Ulcerative colitis Systemic lupus erythematosus Mixed connective tissue disease Scleroderma Whipples disease Other Sarcoidosis Ka

26、wasaki 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 contributing to host susceptibilityA

27、utoantibodies: 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 agents, diuretics often not effec

28、tive 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 term 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 myocard