This is a 2 1/2 month old male who presented to the 
ED with a 2 week history of intermittent low grade 
fevers.  He was seen by his primary physician 2 weeks 
prior to this ED visit for chapped/bleeding lips and 
mucocutaneous lesions.  There was no significant rash 
or fever and it was thought that he may have developed 
a reaction to amoxicillin-clavulinate.  Five days prior to 
the ED visit, he received his first DPT shot and 
developed fever and fussiness which subsequently 
resolved.  The day of the ED visit, he was seen by his 
pediatrician for increased fussiness, decreased oral 
intake and fever.  His exam in the office was significant 
for a temperature of 38.3, mild desquamation of his 
finger tips, and mild meatitis.  Laboratory evaluation 
revealed:  WBC 25,000 with 52% segs and 10% bands, 
Hgb 8.2, Hct 24.3, platelet count 576,000, and ESR 54.  
The patient was subsequently sent to the hospital for a 
sepsis workup.
     The patient had an essentially unremarkable birth 
history and past medical history.  His family history is 
significant for an older brother who died of 
Stevens-Johnson syndrome 3 years ago.
     The patient arrived in the ED late that evening and 
was noted to be pale with cool extremities.  His initial 
oxygen saturation was 80%.  Approximately 20 minutes 
after arrival, the patient went into respiratory arrest, with 
a HR of 35 bpm, and CPR was initiated.  He was 
immediately intubated and chest compressions were 
started.  Intravenous access initially failed; therefore, an 
intraosseous needle was immediately placed.  
Epinephrine was given via the ET tube one minute into 
the code with no increase in HR.  A second epinephrine 
dose was given one minute later without response.  
This was followed by atropine via the ET tube and an 
intraosseous NS bolus.  Five minutes into the 
resuscitation, the HR was up to 120 and chest 
compressions were discontinued.  His initial arterial pH 
was 6.68 with a PaO2 134, PaCO2 51, base deficit 31 
on 100% O2.  However, his HR again dropped into the 
70's five minutes later and chest compressions were 
reinitiated.  A chest radiograph is obtained.

View CXR.

     This CXR reveals slight accentuation of the central 
pulmonary markings, with a normal heart size.  The ET 
tube is in good position.  There is a nasogastric tube 
and a vascular catheter in satisfactory position .
     During the course of the resuscitation, the patient 
eventually received 9 doses of epinephrine, 11 doses of 
atropine, 400 cc of normal saline, 50 cc of albumin, 30 
cc of D25W, 50 meq of NaHCO3, 200 meq of CaCl2, 
and 50 cc of PRBC's.  After initial stabilization in the 
ED, the patient was admitted to the PICU approximately 
two hrs after arrival.
     Upon arrival to the PICU his systolic BP was 70, HR 
151, with extremely diminished pulses and poor 
perfusion.  Shortly thereafter, he became bradycardic, 
then asystolic.
     After 20 minutes of chest compressions and other 
resuscitation measures which were unsuccessful, the 
patient was pronounced dead.
     The working diagnosis at the time of death was 
cardiopulmonary failure secondary to overwhelming 
sepsis.  The postmortem examination revealed findings 
consistent with Kawasaki disease.  Gross evaluation of 
the heart showed mild chamber dilatation, mitral and 
aortic valvulitis, focal myocarditis, and right coronary 
arteritis.  Microscopic examination of the heart showed 
inflammatory infiltrates of the right coronary artery 
consistent with Kawasaki disease.  There was no 
evidence of coronary artery thrombosis.  Examination of 
the brain showed diffuse cerebral edema, and 
cerebellar arachnoid hemorrhages most likely due to 
the prolonged cerebral ischemia.
     Kawasaki disease is an acute febrile multisystem 
vasculitis affecting children.  It is diagnosed by the 
presence of fever (classically greater than 5 days 
duration) and 4 of the 5 following features:

1.  Conjunctival injection
2.  Oral changes
     erythema, fissuring, and crusting of the lips
     diffuse oropharyngeal erythema
     strawberry tongue
3.  Peripheral extremity changes
     hand and feet induration
     erythema of palms and soles
     desquamation of fingertips and toes, about 2 wks
          after onset
     transverse grooves across fingernails, 2-3 months
          after onset
4.  Erythematous rash
5.  Lymphadenopathy of greater than 1.5 cm in
     diameter (classically cervical, non-suppurative).

     Also consistent with the diagnosis are increases in 
the ESR, WBC and platelet count.  The disease is 
usually self-limited and benign; however, complication 
rates can be reduced if treated early with IVIG 
(intravenous gamma globulin) and aspirin.  The most 
important clinical association of Kawasaki disease is 
cardiac disease (incidence roughly 30%), and early 
studies report a 2% mortality rate secondary to sudden 
cardiac death.  Autopsy studies report acute thrombosis 
of coronary arteries in 80% of fatal cases.  Risk factors 
most often associated with coronary involvement are: 
age under 1 yr, anemia, WBC > 30,000, elevated ESR 
and/or C-reactive protein, and fever for 14 days or 
longer.  The patient described above had 4 of these 5 
risk factors.
     The diagnosis of Kawasaki disease in the ED is 
often difficult because of atypical presentations (i.e., 
less than 4 of the 5 criteria).  Young infants in particular 
often have milder or more subtle signs and symptoms.  
The term "atypical Kawasaki disease" describes 
patients who do not have 4 of the 5 criteria but who 
have evidence of coronary artery involvement.  This 
patient fits the diagnosis of atypical Kawasaki disease, 
since he did not have either a rash or 
lymphadenopathy at time of presentation.  Kawasaki 
syndrome is often difficult to distinguish from serum 
sickness, Stevens-Johnson syndrome or erythema 
multiforme.  Because there is a risk of sudden cardiac 
death in Kawasaki disease, the ED physician must be 
able to recognize the clinical features of the disease so 
that pharmacological therapy (IVIG and ASA) can be 
initiated.
     Since Kawasaki syndrome is often misdiagnosed as 
serum sickness, Stevens-Johnson syndrome, erythema 
multiforme, or viral exanthems, it is important to 
consider the diagnosis of Kawasaki syndrome 
whenever any of the above diagnoses are entertained.  
Of interest in this case is the family history of a sibling 
who died from Stevens-Johnson syndrome.
     Primary cardiac causes of bradycardia are rare in 
the pediatric population, and such cases represent a 
formidable challenge for ED personnel.  The steps 
taken in the attempted resuscitation outlined above 
were in accordance with that outlined in the Pediatric 
Advanced Life Support (PALS) manual (at that time).  
After repeated doses of epinephrine and atropine, 
another therapeutic modality that may be considered is 
transcutaneous or esophageal pacing.  The extent of 
this patient's cardiac lesions suggests that he probably 
would not have benefited from electrical pacing.  
However, for bradycardic patients with Kawasaki 
disease and less severe (and reversible) cardiac 
lesions, external pacing might be beneficial while 
waiting for IVIG and ASA therapy to take effect.

References
     Yamamoto LG, Martin JG.  Kawasaki syndrome in 
the ED.  Am J Emerg. Med 1994;12:178-182. 
     Melish ME. Hicks RV.  Kawasaki Syndrome: Clinical 
features, pathophysiology, etiology, and therapy.  J 
Rheumatoloty (suppl 24) `1990;17:2-10.
     Gersony WM.  Diagnosis and management of 
Kawasaki disease.  JAMA 1991;256(20):2699-2703.
     Levy M, Koren G. Atypical Kawasaki disease: 
analysis of cinical presentation and diagnostic clues.  
Pediatr Infect Dis J 1990;9:122-126.