课件：临床病理讨论会.ppt

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 girl,Chief complaint: Chest discomfort, vomiting and dry cough for one day,Brief History,Growth & development: Weight: 22 kg (3rd-10th percentile) Height: 130 cm (25-50th percentile) Development milestone: within normal limit Past history Hand-foot-mouth disease in 1998 Frequent URI and fever during childhood No drug or food allergy,Brief History,Family history: Her sister had fever and URI recently.,Present Illness,Fever and bilateral hand arthralgia attack once 1 month ago Chest discomfort and cough since 9/11 afternoon, 2001 Visit LMD and URI was told Vomiting and chest tightness on 9/12 0 AM and 5 AM,Present Illness,9/12 morning, visit LMD again, ECG showed arrhythmia Refer to 亞東 hospital,Present Illness,Findings at 亞東 hospital Clear consciousness, ill-looking, pallor appearance, no cyanosis Irregular heart beat EKG: VPC bigeminy,Present Illness,Lab. 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,Present Illness,Echocardiogram at 亞東 hospital Multiple small VSDs, muscular trabecular type, at apex LV dyskinesia, LVEF 60-70% Mild TR, mild MR,Present Illness,Management at 亞東 hospital Lidocaine iv drip Dopamine 10 mg/kg/min Refer to NTUH (2pm),Physical Examination,Physical 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,Physical Examination,Neck: jugular venous engorgement Chest: bilateral basal rles Heart: irregularly irregular beats, distant heart sound no murmur,Physical Examination,Abdomen: no hepatomegaly hypoactive bowel sound Extremities: freely movable cold and cyanotic poor capillary refilling,Initial Lab Data,CBC: 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,Initial Lab Data,VBG: pH pCO2 pO2 HCO3 BE 7.36 47.4 27.3 26.9 +1.4 Cardiac enzyme: CPK(U/L) CK-MB Troponin I (ng/ml) 1040 196.5 31.9 CRP: 0.53 mg/dl,Initial Lab Data,EKG (9/12):,Initial Lab Data,EKG (9/12):,Initial Lab Data,EKG (9/12):,Initial Lab Data,Echocardiogram (9/12): LV enlargement LVEF 45% Muscular VSD Mild MR, TR, PR,Echocardiogram (9/12),Course and Treatment,Management For cardiogenic shock: Dopamine, Dobutamin, Primacor, Lasix For ventricular arrhythmia: Amiodarone, Lidocaine, MgSO4 For myocarditis: IVIG, Consider extracorporeal membranous oxygenator (ECMO) support,Course and Treatment,9/12 5pm (3 hr after admission) Progressive hypotension Sudden onset of coma, BP drop (pulseless) EKG: ventricular tachycardia Start CPR (40 min) Start ECMO, transfer to SICU,EKG (9/12, 5 PM),Course in SICU,ECMO setting V-A ECMO: 15 Fr Rt femoral artery, 19 Fr Rt femoral vein by cutdown Flow: 2000 ml/min Mean BP: 70 mmHg Urine output: 1.72 ml/kg/hr,Echocardiogram (9/13),Course in SICU,VT persistent despite of cardioversion, Lidocaine, Amiodarone, MgSO4 9/12 9/17: ECMO 5 days Poor LV function Persistent lung edema (CXR, clinically) TnI slowly decrease A-line flatten, no pulsatile wave form,Course in SICU,Endomyocardial biopsy (9/14) Mild to moderate perivascular and interstitial lymphocyte infiltration Foci of myocyte degeneration Interstitial edema No giant cell,Compatible with acute myocarditis,Course in SICU,LA drain (9/17): To decompress LV, avoid thrombosis LA dome cannulation connecting to FV cannula ECMO FA LAP: 22 mmHg 10 mmHg,Echocardiogram (9/17),Course in SICU,9/18, 4am Acute thrombosis at LA cannula and ECMO circuit poor flow CPR for 30 min. and emergent re-set ECMO tubing Cons. After CPR: E1M1VT Light reflex (+),Course in SICU,9/19, 8am: gross hematuria and ECMO tube thrombosis reset ECMO Progressive dilated pupils, no light reflex, suspected hypoxic encephalopathy Remove ECMO on 9/23 (10th day),Lab data,Lab Data,Lab Data,Serology study; Mycoplasma pneumonia IgM: (9/12) positive, (9/21) negative Other virology study: all negative Coxsackie A, Coxsackie B1-B6, CMV IgG & IgM, Enterovirus 70, Influenza A & B,Lab Data,Culture: Throat swab (9/12): Staphylococcus aureus Nasal swab (9/12): Staphylococcus aureus, Viridans streptococci Blood (9/19): Staphylococcus epidermidis,Discussion,Diagnostic approach: Cause of chest pain in children Idiopathic: 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 approach,Hx: cough, vomiting PE: hypotension jugular venous distention tachycardia irregular heart beat basal rles poor peripheral perfusion Cardiovascular compromise,Diagnostic approach,Flu-like illness, arrhythmia, cardiovascular compromise Acute myocarditis highly suspected D/D: Dilated cardiomyopathy Anomalous left coronary artery Chronic tachyarrhythmia Pericarditis,Diagnostic approach,EKG: VPC bigeminy, ventricular tachycardia ST-segment change Elevated cardiac enzyme Echocardiogram: marked LV dyskinesia Endomyocardial biopsy Lymphocyte infiltration Myocyte degeneration,Acute myocarditis confirmed,Clinical classification of myocarditis,Myocarditis: an enigmatic disease!,Dark side of the myocarditis,Initial non-specific symptoms Difficult to establish the diagnosis Etiology hard to find Complexity of pathogenesis Often refractory to conventional treatment,Dark side of the myocarditis,Initial non-specific symptoms Similar to patients with sepsis, bronchiolitis, pneumonia, gastroenteritis, hepatitis, and renal failure etc. Aggressive fluid resuscitation may harm unstable patients Rapid progression in fulminant myocarditis,Dark side of the myocarditis,Difficult to establish the diagnosis Limited sensitivity and specificity of changes in CXR, ECG, cardiac enzyme (Troponin level: more sensitive) Echocardiogram: LV dysfunction, often regional Endomyocardial biopsy: as gold standard, but sensitivity 3-63%,Dallas criteria,Borderline myocarditis,Active myocarditis,Am J Cadiovasc Pathol 1987;1:3-14,Dark side of the myocarditis,Etiology hard to find,VIRAL CAUSES Enterovirus Coxsackie A Coxsackie B Echovirus Poliovirus Adenovirus Cytomegalovirus Herpesvirus Influenza A Epstein-Barr virus Varicella Mumps Measles Parvovirus Rabies Hepatitis B,C Rubella Rubeola Respiratory syncytial virus Human immunodeficiency virus,Rickettsial Rickettsia ricketsii Rickettsia tsutsugamushi Bacterial Meningococcus Klebsiella Leptospira Mycoplasma Salmonella Clostridia 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 Coccidiodomycosis Histoplasmosis Candida,NONVIRAL CAUSES,Dark side of the myocarditis,Etiology hard to find,Toxic Scorpion Diphtheria Drugs Sulfonamides Phenylbutazone Cyclophosphamide Neomercazole Acetazolamide Amphotericin B Indomethacin Tetracycline Isoniazid Methyldopa Phenytoin Penicillin,Hypersensitivity/Autoimmune Rheumatoid arthritis Rheumatic fever Ulcerative colitis Systemic lupus erythematosus Mixed connective tissue disease Scleroderma Whipples disease Other Sarcoidosis Kawasaki disease Cornstarch,NONINFECTIOUS ETIOLOGIES,Dark side of the myocarditis,Etiology hard to find,Pediatr Cardiol 2001;22:34-9,Dark side of the myocarditis,Complexity of pathogenesis,NEJM 2000;343:1388-98,Dark side of the myocarditis,Complexity of pathogenesis Factors contributing to host susceptibility Autoantibodies: to adenosine nucleotide translocator, myosin Expression of cell adhesion molecules (ICAM-1) Expression of coxsackie-adenovirus receptor (CAR),Dark side of the myocarditis,Often refractory to conventional treatment Standard therapy: ACE inhibitor, inotropic agents, diuretics often not effective in fulminant myocarditis Immunosuppression: IVIG, steroids, cyclosporin still controversial,Bright side of the myocarditis,Good long term prognosis of fulminant myocarditis Improvement of mechanical support: LVAD, BVAD, ECMO,Bright side of the myocarditis,Good long term prognosis of fulminant myocarditis,NEJM 2000;342:690-5,Bright side of the myocarditis,Good long term prognosis of fulminant myocarditis,Bright side of the myocarditis,Good long term prognosis of fulminant myocarditis Why? Different viral agent? Different host response? Autoimmune in nature?,Bright side of the myocarditis,Ventricular assistant device (VAD) & Extracorporeal membrane oxygenation (ECMO),Bright side of the myocarditis,VAD and ECMO in fulminant myocarditis: Basically a reversible disease Indicati