Mortality Conference

報告者 :呂淑偉大夫指導者 :麥淑珍主任

Basic Data

• Name:

• Sex: Female

• Age:12Y/O

Chief Complaint

• Conscious change with right limbs weakness since 6 days

Present Illness

• Relative well in the past• 89/11/21-11/24: intermittent fever• Since 11/24: irritable, confusion, crying• Since 11/26: unable to walk

--> other H, CT: negative --> MBD on 11/28• 11/29: conscious disturbance, Rt limbs weakness

--> our OPD --> admitted

Other History

• Birth Hx: G2P2, FT, BBW:3200gm, C/S• Past Hx: nil• Drug Hx: nil• Vaccine Hx: as schedule• Development: as milestones• Family Hx: non-contrubutory

no acute illness

Physical Examination

• GA: acute ill looking, no CP distress• V.S: BP: 134/83 mmHg, PR:96/min,RR:26/min,

BT:37.3C• Skin: no rash• HEENT: no oral ulcer, no throat injection• Neck: supple• Heart: RHB, no murmur• Chest: Clear BS

Physical Examination

• Abdomen: soft, no hepatosplenomegaly• Ext: freely movable, no ROM limitation• NE:

– C: confused with delirum– O: isocoric pupil with prompt LR– M: full MP, normal DTR– A: normal respiratory pattern– Crainal nerve: no special finding

Hospital Course• Day 1: --R/O encephalitis ---> LP: IICP: 370mmH2O,

WBC: 0, RBC:10; EBVCA IgG:1:1280, IgM(-) --> Glycetose,: Tegretol --> EEG: few focal spikes over P4

• Day 2-4: fever, still bizarre behavior, seizure at D3, acute urine retention

• Day 5: Rt lung pneumonia patchy

• Day 6: SOB -->ICU--> ETT intubation; PCN-->unasyn

• Day 8: frequent seizure --> lamictal

• Day 9, 14: extubation failure Paired EBV titer: IgG: 1:2560, IgM: (-)

• Day 15: dilantin ( status epilepticus)

• Day 18-20: fever, Rt lung white out, intractable seizure --> Abx: oxacillin + GM + erythromycin --> AEDs: dilantin, topamas, chloral hydrate

• Day 21--22: extubation, UGI bleeding

• Day 23--28:-- Re-intubation; Sputum: MRSA,GNB; -- Urine candida, sono: fugus ball => cefobid + targocid + fluconazole

• Day 29: tracheostomy

• Day 31: VPC: hold dilantin (normal dilantin level)

• Day 36: skin rash (erythema multiform) --> DC Abx --> solucortef + allermin

• Day 37: hepatomegaly: GOT/GPT:122/85

• Day 41: severe tarry stool --> cimeticine + transamin

• Day 42: urine /C: candia --> fluconazole=> fungizone

• Day 43--50: --Seizure: dilantin, haldol + akineton --Bleeding tendency ( PT, APTT prolonged) --DIC test (+), U/C: E.coli => unasyn + GM -- Jaundice, liver Bx: C/W Ac. Hepatitis

• Day 51: Brain MRI: brain atrophy

• Day 52--62: -- Polyuria, DI, electrolyte imbalance, VPC ( Na:152, S Osm:333, U SpGr:1.007)

• Day 63: -- Electrolyte got normal -- Episodic seizure with VPC, => subsided s/p chloral hydrate -- Sudden onst of Vf, pulseless, cyanosis on at 0:10 => CPR, defibrilation, lidocaine, epinephrine => Expired at 2:18 -- Na/ K/ Ca: 134/5.2/9.7, Glu: 248 ABG: desaturation, no acidosis initially Heart sono: no pericardial effusion

Lab data• WBC N/L Hgb Plt CRP

891201 6190 71/19 12.6 258k <0.01900113 5200 11.1 260k 900201 8220 8.7 261k

• Na K Ca BUN Cre GOT GPT Bil Alb891201 140 4.5 8.5 10900111 149 310 8.1/3.7 3.0 900121 151 3.5 8.8 26 0.9 80 7/3.1 900201 122 1.6 7.6 40 0.2/0 135 2.3 9.8 134 5.2 9.7

Lab Data

• Cultures – CSF virus/C: negative– Sputum (12/6): S. aureus

(12/24): MRSA, GNB ( 1/3): S. aureus (1/31): P. aeruginosa

– Urine (12/26): Candida albicans (1/3): E. coli (2/1): P. aeruginosa

• |-| fever illness |-------Conscious disturbance----------------| |----------------seizure-----------------------| |-------dilantin --| |-----| |-----------| ||-|----VPC----Vf-|| |---DI--| |------Pnuemonia, UTI------------------------|

Final Diagnosis

• Acute encephalitis with intractable seizureHighly suspect EBV encephalitis

• Arrhythmia (VPC, Vf) with cardiogenic shock• Central diabetes insipidus • Pneumonia with respiratory failure• UTI: candida, E.coli, P.aeruginosa• Acute hepatitis with jaundice• UGI bleeding

Cause of her death ? 1. EBV encephalitis

2. VPC and Vf

Epstein-Barr Virus

Epstein-Barr Virus

• a DNA virus

• Causes> 90% infectious mononucleosis• Humans: the only source (Infects >95% population)

Epstein-Barr Virus-- Diagnosis --

• Classic S/S of infectious mononucleosis– fever, pharyngitis, LAP, splenomegaly

• Hetrophil Ab(+)

• EBV-specific serology– Anti -VCA IgM: positive– Paired anti-VCA IgG

• 4X change in acute / convalescent stages

EBV--Complications of IM• Splenic rupture (2nd week, 0.2%)

• Airway impairment

• Neurologic involvement

• Hemolytic anemia, aplastic anemia

• Myocarditis (resolving in 3-4wks)

• Interstitial pneumonia (resolving in 3-4wks)

• Pancreatitis, parotitis, orchitis

• VAHS

EBV-- Neurologic involvement --

• Incidence: 5.5-18%• Manifestations

– Aseptic meningitis: fever, seizure, meningeal signs

– Encephalitis: fever, mental status change, ataxia bizarre movement, seizure, IICP signs

– Meningoencephalitis

– Alice in Wonderland syndrome: perceptual distortions of space/ size

– Transverse myelitis

– Guillian-Barre syndrome

– Cranial neuritis

Epstein-Barr Virus-- Neurologic involvement --

• Neurologic S&S– May precede, occur concomitantly or follow by

several weeks of IM– May be the first /only manifestations

EBV encephalitis and encephalomyelitis< Pediatr Radiol 1996 26:690-693>

• 29 Pt, 1-15 yr• Conscious disturbance (100%)

• Seizures (52%)

• Visual hallucination (41%)

• Acute psychosis (34%)

• Fever (31%)

• Hypersomni-insomnia (6%)

• Urinary retention (10%)

• Abducens palsy (10%)

• Facial palsy (10%)

• Ataxia (7%)

• Acute hemiparesis (7%)

• Transient visual loss (7%)

• Ptosis (7%)

• Auditory hallucination (7%)

• Choreoathetosis (7%)

• Hearing impairment (7%)

• Diplopia (3%)

• Nystagmus (3%)

• Custatory hallucination (3%)

Epstein-Barr Virus-- Treatment --

• Good supportive care

• Avoid contact sports (splenic rupture)

• Aggressive management of IICP

• Anticonvulsant for seizure

• Intravenous acyclovir – Decrease -- viral replication

-- oropharyngeal sheeding– No effect of -- severity of symptoms

-- eventual clinical course

Epstein-Barr Virus-- Prognosis --

• Majority: benign outcomes

• Persistent neurologic abnormalities– Incidence: 40%– Global impairment– Perseverative autistic-like behavior– Persistent extremitis paresis

Acute maninfestations and neurologic sewuelae of EBV encephalitis, Pediatr Infect Dis J, 1996; 15:871-5

Epstein-Barr Virus-- Mortality--

• CNS

• Heart

Epstein-Barr Virus-- Mortality (1)--

• Early report– Guillain-Barre syndrome / brain edema, herniation

• Recent report– Brainstem encephalitis

• 8M/O, drowsiness, fever, vomiting x 3 days-->8hr: gasping, comatous, LP:high pressure(400) lymphocyte pleocytosis--> Abx + acyclovir-->expired on day 16 from central faliure

• VCA IgG 4X change, Brain sono,, Brain CT

Fatal brainstem encephalitis caused by EBV, C.S.Chi, Pediatr Radiol 1994 24:596-597

Epstein-Barr Virus-- Mortality (2)--

• Acute encephalitis in primary EBV infection– 6 y/o girl, acute onset of IM-like syndrome

• D19: progressed to encephalitis, coma

• D27: death

• Acyclovir: did not improve

• Serology: primary EBV infection

Fatal acute encephalitis in primary EBV infectionArch-Fr-Pediatr. 1990 Aug-Sep’ 47(7):513-4

Epstein-Barr Virus-- Mortality (3)--

• Polyradiculomeningoencephalitis by EBV– 21y/o boy, encephalitis– Sudden drop of BP– Necropsy: moderate inflammatory infiltrations

in leptomeninges, atypical lymphoid cells– Heart arrest from acute autonomic neuropathy

Fortschr-Neurol-Psychiatr. 1984 Mar 52(3):73-82

Epstein-Barr Virus-- Mortality (4)--

• Fatal EBV myocarditis in repetitive myocarditis– 9y/o boy

– Previous 2 myocarditis: chicken pox; undetermined origin

– The fatal episode: seroloy --acute EBV infection• Autopsy: PCR for EBV (+) in heart & liver

• HLA-DR: DR4, DR13(+)< DR4: associated with autoimmune dz-- dilated cardiomyopathy>

– Postulate: aberrant immune response

Pediatr-Patho-Lab-Med. 1995 Sep-Oct;; 15(5):805-12

Epstein-Barr Virus-- Mortality (5)--

• 14 y/o girl (1977)– Atypical lymphocytosis, fever, sorethroat– Day 8: cardiopulmonary attest --> death

• Initial monitor: Vf

– Autopsy: myocardial mononuclear infiltrates

• 20 y/o – Hetrophil titer 1:448– Chest pain, EGK: RBBB, inverted T– Cardiac arrest

Epstein-Barr Virus-- Myocarditis--

• 38 y/o female– Viral syndrome, myagia, fever, malaise– Chest pain, SOB, near-syncope– EKG: AMI– Endomyocrdal biopsy: viral cause– EBVCA IgG: 1:640 --> 1:320

EBNA: 1:20---> 1:40

Southern-medical-journal 1989 Sep; 82(9): 1184-7

Ventricular arrhythmias

1. Premature QRS 4. Abnormal ST, T

2. Different QRS 5. No preceded p

3. Prolonged QRS duration

Bigeminy, Trigeminy, Couplets,Triplets

Premature ventricular contractions (PVCs)

PVCs -- Causes• Healthy children (50-70% on 24hr EKG)• LV false tendon (Purkinge fibers)• Myocarditis, myocardial injury, infarction• Cardiomyopathy (dilated, hypertrophic)• Cardiac tumor; MVP• RV dysplasia• Long QT syndrome (QTc>0.44sec)• CHD, acquired heart dz (pre-OP, post-OP)• Digitalis, drugs (catecholamines, theophylline,

caffeine, amphetamines, anesthetic agents)• Electrolyte imbalance (k, Ca, Mg)

PVCs -- Significance

• Associated underlying heart dz

• History of syncope, sudden death of family

• Precipitated by/ more frequent -- activity

• PVC with symptoms

• Multiform (esp. couplets)

• R on T phenomenon

• > 6 PVCs / one min

Multifocal PVCs

R on T

PVCs -- Treatment

• Symptomatic– Frequent PVCs

• IV bolus lidocaine 1mg/kg

• Maintain: 20-50ug/kg/min

– Cardiomyopathy, RV dysplasia-blockers (atenolol 1-2mg/kg, po, qd)

– Dilantin, mexiletine( avoid class IA, III: amiodarone)

Ventricular fibrillation : Vf

• Terminal arrhythmia --> ineffective circulation

• Bizarre QRS, varying sized /onfiguration, rate: rapid / irregular

Vf -- Causes

• Postoperative state

• Severe hypoxia

• Hyperkalemia

• Digitalis or quinidine toxicity

• Myocarditis

• Myocardial infarction

• Drugs (catecholamines, anesthetics)

• |----------------seizure-----------------------|

|-------dilantin --| |-----| |-----------| |-----| midazolam |--| |-----| (BPCO,arrest)

|---------| Tegretol (AV block,arrhythmia,hypotension)

(1st AV block) |------| Lamictal hadol |------------------| (palpitation) Topiramate |------------| |----Chloral hydrate---------------------| ||-|----VPC----Vf-||

Hypotension, arrhythmiea, Vf

Guidelines 2000 for Cardiopulmonary Resuscitation and Emergency Cardiovascular

Care

• Pediatric Advanced Life Support

VF/pulseless VT

• Defibrillation– Up to 3 times if needed– 2-->4-->4J/kg

• Epinephrine– IV/IV: 0.01mg/kg

(1:10000; 0.1mg/kg)– ETT: 0.1mg/kg

(1:1000;0.1mg/kg)

*Attempt - ETT, vascular access*Give - Epi, q3-5mins*Consider - Vasopressors - Antiarrhythmics - Buffers*Identify & Tx - Hypoxemia - Hypovolemia - Hypothermia - Hypo/ Hyper-K - Tamponade - Tension pneumothorax - Toxins/poisons/drugs - Thromboembolism

• Attempt defibrillation 4J/kg(within 30-60 secs after each medication)– CPR-drug-shock (repeat)– CPR-drug-shock-shock-shock (repeat)

• Antiarrhythmic– Amiodarone: 5mg/kg bolus IV/IO– Lidocaine: 1mg/kg bolus– Magnesium: 25-50 mg (max:2g)

( torsades de pointes or hypomagnesemia)

• Attempt defibrillation with 4J/kg(within 30-60 seconds after each medication)– CPR-drug-shock (repeat)– CPR-drug-shock-shock-shock (repeat)

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