临床病理讨论会课件

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1臨床病理討論會小兒科：盧俊維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2A 10 y/o girlChief complaint:临Brief HistoryGrowth & development:Weight: 22 kg (3rd-10th percentile)Height: 130 cm (25-50th percentile)Devel

2、opment milestone: within normal limitPast historyHand-foot-mouth disease in 1998Frequent URI and fever during childhoodNo drug or food allergy临床病理讨论会课件3Brief HistoryGrowth & developmBrief HistoryFamily history:Her sister had fever and URI recently. 临床病理讨论会课件4Brief HistoryFamily history:临床Present Ill

3、nessFever 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 AM临床病理讨论会课件5Present IllnessFever and bilatPresent Illness9/12 morning, visit LMD again, ECG showed arrhythmiaR

4、efer to 亞東 hospital临床病理讨论会课件6Present Illness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 bigeminy临床病理讨论会课件7Present Illness临床病理讨论会课件7Present IllnessLab. findings at 亞東 hospital WBC 9000/mm3, Hb 13.5 g/d

5、l 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 Illness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临床病理讨论会课件9Present Illness临床病理讨论会课件9Present IllnessManag

6、ement 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10Present Illness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

7、临床病理讨论会课件11Physical Examination临床病理讨论会课件1Physical 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临床病理讨论会课件1Physical Examination Abdomen: no hepatomegaly hypoactive bowel sound Ex

8、tremities: freely movable cold and cyanotic poor capillary refilling临床病理讨论会课件13Physical Examination临床病理讨论会课件1Initial 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 DataCBC:临床病理讨论会课件1Initial La

9、b 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 临床病理讨论会课件15Initial Lab DataVBG:临床病理讨论会课件1Initial Lab DataEKG (9/12): 临床病理讨论会课件16Initial Lab DataEKG (9/12):临床病Initial Lab DataEKG (9/12): 临床病理讨论会课件17Initial Lab Dat

10、aEKG (9/12):临床病Initial Lab DataEKG (9/12): 临床病理讨论会课件18Initial Lab DataEKG (9/12):临床病Initial Lab DataEchocardiogram (9/12):LV enlargementLVEF 45%Muscular VSDMild MR, TR, PR 临床病理讨论会课件19Initial Lab DataEchocardiogramEchocardiogram (9/12)临床病理讨论会课件20Echocardiogram (9/12)临床病理讨论会课件Course and TreatmentManag

11、ementFor cardiogenic shock: Dopamine, 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 TreatmentManagementCourse and Treatment9/12 5pm (3 hr after admission)Progr

12、essive hypotensionSudden onset of coma, BP drop (pulseless)EKG: ventricular tachycardiaStart CPR (40 min)Start ECMO, transfer to SICU临床病理讨论会课件22Course and Treatment9/12 5pm (EKG (9/12, 5 PM)临床病理讨论会课件23EKG (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 fe

13、moral vein by cutdownFlow: 2000 ml/minMean BP: 70 mmHgUrine output: 1.72 ml/kg/hr临床病理讨论会课件24Course in SICUECMO setting临床病理Echocardiogram (9/13)临床病理讨论会课件25Echocardiogram (9/13)临床病理讨论会课件Course in SICUVT persistent despite of cardioversion, Lidocaine, Amiodarone, MgSO4 9/12 9/17: ECMO 5 daysPoor LV fun

14、ctionPersistent lung edema (CXR, clinically)TnI slowly decreaseA-line flatten, no pulsatile wave form临床病理讨论会课件26Course in SICUVT persistent deCourse in SICUEndomyocardial biopsy (9/14)Mild to moderate perivascular and interstitial lymphocyte infiltrationFoci of myocyte degeneration Interstitial edem

15、aNo giant cell Compatible with acute myocarditis临床病理讨论会课件27Course in SICUEndomyocardial bCourse 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28Course in SICULA drain (9/17):Echocardiogram (9/17)临床病理讨论会课件29E

16、chocardiogram (9/17)临床病理讨论会课件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 (+)临床病理讨论会课件30Course in SICU9/18, 4am 临床病理讨论Course in SICU9/19, 8am: gross hematuria and ECMO tube thrombo

17、sis reset ECMOProgressive dilated pupils, no light reflex, suspected hypoxic encephalopathyRemove ECMO on 9/23 (10th day)临床病理讨论会课件31Course in SICU9/19, 8am: grossLab data9/129/139/149/159/169/17TnI31.962.41007437.3CK104091242342126759138647026CK-MB196368687403207101Cre0.630.590.560.50.470.51Bil1.240

18、.510.651.361.51.35临床病理讨论会课件32Lab data9/129/139/149/159/169/Lab Data临床病理讨论会课件33Lab Data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34

19、Lab DataSerology study;临床病理讨论会Lab DataCulture:Throat swab (9/12): Staphylococcus aureusNasal swab (9/12): Staphylococcus aureus, Viridans streptococciBlood (9/19): Staphylococcus epidermidis临床病理讨论会课件35Lab DataCulture:临床病理讨论会课件35DiscussionDiagnostic approach: Cause of chest pain in childrenIdiopathic

20、: 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%临床病理讨论会课件36DiscussionDiagnostic approach:Diagnostic approachHx: cough, vomitingPE: hypotension jugular venous distention tachycardia irregular he

21、art beat basal rles poor peripheral perfusion Cardiovascular compromise 临床病理讨论会课件37Diagnostic approachHx: cough, Diagnostic approachFlu-like illness, arrhythmia, cardiovascular compromise Acute myocarditis highly suspectedD/D: Dilated cardiomyopathy Anomalous left coronary artery Chronic tachyarrhyt

22、hmia Pericarditis 临床病理讨论会课件38Diagnostic approachFlu-like ilDiagnostic approachEKG: VPC bigeminy, ventricular tachycardiaST-segment changeElevated cardiac enzymeEchocardiogram: marked LV dyskinesiaEndomyocardial biopsyLymphocyte infiltrationMyocyte degeneration Acute myocarditis confirmed临床病理讨论会课件39D

23、iagnostic approachEKG: Acute Clinical classification of myocarditisFulminantAcuteChronic activeChronic persistentInitial presentationShock, severe LV dysfuntionCHFCHFNormal LV functionEndomyocardial biopsyMultifocal active myocarditisActive or borderline myocarditisActive or borderline myocarditisAc

24、tive or borderline myocarditisNature historyComplete recovery or deathIncomplete recovery or DCMDCMNormal LV function临床病理讨论会课件40Clinical classification of myoMyocarditis: an enigmatic disease!临床病理讨论会课件41Myocarditis: an enigmatic diseDark side of the myocarditisInitial non-specific symptoms Difficult

25、 to establish the diagnosisEtiology hard to findComplexity of pathogenesisOften refractory to conventional treatment临床病理讨论会课件42Dark side of the myocarditisInDark side of the myocarditisInitial non-specific symptoms Similar to patients with sepsis, bronchiolitis, pneumonia, gastroenteritis, hepatitis

26、, and renal failure etc.Aggressive fluid resuscitation may harm unstable patientsRapid progression in fulminant myocarditis临床病理讨论会课件43Dark side of the myocarditisInDark side of the myocarditisDifficult to establish the diagnosisLimited sensitivity and specificity of changes in CXR, ECG, cardiac enzy

27、me (Troponin level: more sensitive)Echocardiogram: LV dysfunction, often regionalEndomyocardial biopsy: as gold standard, but sensitivity 3-63%临床病理讨论会课件44Dark side of the myocarditisDiDallas criteriaBorderline myocarditisActive myocarditisAm J Cadiovasc Pathol 1987;1:3-14临床病理讨论会课件45Dallas criteriaBo

28、rderline myocDark 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 syncytial virus H

29、uman immunodeficiency virusRickettsial Rickettsia ricketsii Rickettsia tsutsugamushiBacterial Meningococcus Klebsiella Leptospira Mycoplasma Salmonella Clostridia Tuberculosis Brucella Legionella pneumophila smallpox Streptococcus Protozoal Trypanosoma cruzi Toxoplasmosis Amebiasis Other parasites T

30、oxocara canis Schistosomiasis Hetereophyiasis Cysticercosis Echinococcus Visceral larva migrans Trichinosis Fungi and yeasts Actinomycosis Coccidiodomycosis Histoplasmosis Candida NONVIRAL CAUSES 临床病理讨论会课件46Dark side of the myocarditisEtDark side of the myocarditisEtiology hard to findToxic Scorpion

31、 Diphtheria Drugs Sulfonamides Phenylbutazone Cyclophosphamide Neomercazole Acetazolamide Amphotericin B Indomethacin Tetracycline Isoniazid Methyldopa Phenytoin PenicillinHypersensitivity/Autoimmune Rheumatoid arthritis Rheumatic fever Ulcerative colitis Systemic lupus erythematosus Mixed connectiv

32、e tissue disease Scleroderma Whipples disease Other Sarcoidosis Kawasaki disease CornstarchNONINFECTIOUS ETIOLOGIES临床病理讨论会课件47Dark side of the myocarditisEtDark side of the myocarditisEtiology hard to findPediatr Cardiol 2001;22:34-9临床病理讨论会课件48Dark side of the myocarditisEtDark side of the myocardit

33、isComplexity of pathogenesisNEJM 2000;343:1388-98临床病理讨论会课件49Dark side of the myocarditisCoDark side of the myocarditisComplexity of pathogenesis Factors contributing to host susceptibilityAutoantibodies: to adenosine nucleotide translocator, myosinExpression of cell adhesion molecules (ICAM-1)Expres

34、sion of coxsackie-adenovirus receptor (CAR)临床病理讨论会课件50Dark side of the myocarditisCoDark side of the myocarditisOften refractory to conventional treatmentStandard therapy: ACE inhibitor, inotropic agents, diuretics often not effective in fulminant myocarditisImmunosuppression: IVIG, steroids, cyclos

35、porin still controversial临床病理讨论会课件51Dark side of the myocarditisOfBright 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 myocarditisBright side of the myocarditisGood long term prognosis of fulmi

36、nant myocarditisNEJM 2000;342:690-5临床病理讨论会课件53Bright side of the myocarditisBright side of the myocarditisGood long term prognosis of fulminant myocarditis临床病理讨论会课件54Bright side of the myocarditisBright side of the myocarditisGood long term prognosis of fulminant myocarditisWhy?Different viral agent?Differen