Transcript Kawasaki Disease

Morning Report 7/13/09
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Acute febrile vasculitic syndrome of early
childhood
Affecting all blood vessels in the body but
mostly medium and small vessels with a
preferential involvement of the coronary
arteries.
Exact etiology unknown but thought to be
infectious in nature
Immune response thought to be oligoclonal or
antigen driven
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Race: Japanese > Blacks, Polynesians, Filipinos
> Whites
Gender: Male:Female~ 3:2
Age:
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90-95% <10years old
Peak incident 18-24months
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Presence of 5 or more days of fever + 4 or more
of the 5 principle clinical features
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Arthritis/arthralgia
Irritability
Diarrhea, Vomiting, Abdominal Pain
Hepatomegally, Jaundice
Pleural Effusions, infiltrates
Stiff Neck secondary to aseptic meningitis
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Children with unexplained fever for more than
5 days associated with 2-3 of the principle
clinical features
More common in young infants
May be supported by laboratory evidence of
systemic inflammation
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Viral Infections (Measles, adenovirus,
enterovirus, EBV)
Scarlet Fever
Staphylococcal scalded skin syndrome
Bacterial cervical lymphadenitis
Rocky Mountain Spotted Fever
Leptospirosis
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Moderate to high WBC count with left shift
Anemia
Elevated ESR, CRP
Thrombocytosis
Mild-Moderate elevation in transaminases
Sterile Pyuria
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Toxic Shock Syndrome
Drug Hypersensitivity
Steven-Johnson syndrome
Juvenile idiopathic arthritis
Juvenile Polyarteritis Nodosa
Mercury hypersensitivity reaction
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Echocardiogram is critical for the evaluation of all
patients suspected of having KD.
Baseline echo during acute stage to r/o coronary artery
aneurysms and evidence of myocarditis, valvulitis, or
pericardial effusion
 Echo should be repeated in 2nd-3rd week of illness and
again 1 month after (or once all lab values normalize)
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Prior to treatment 20-25% of patients had Cardiac
involvement with mortality rate 0.1-2%
With IVIG risk reduced to 5%
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Standard therapy is IVIG with Asprin
During the acute phase of illness
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IVIG (2gm/kg) and
Asprin 80-100mg/kg /day
Continue high dose asprin until day 14 of
illness if still afebrile
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Continue asprin 3-5mg/kg/day until no evidence of
coronary changes by 6-8 weeks
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~10% fail to respond to initial IVIG therapy
(persistence of fever after 36hrs)
Retreatment with IVIG at same dose
recommended
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3rd dose IVIG
Pulse Steroids (Methylprednisolone mg/kg for
2-3 hours qday x3days)
Infliximab (monoclonal ab against tumor
necrosis factor)
Cyclophosphamide
Methotrexate
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MI caused by thrombotis occlusion of abnormal
coronary artery Is principle cause of death
Usually occurs within first year
Children at high risk need frequent ECHO evaluations
Small solitary aneurysms-long term asprin therapy
Giant aneurysms or multiple complex aneurysms-long
term antiplatelet therapy and anticoagulation
Primary surgical management is coronary artery
bypass graft