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Kawasaki Syndrome

Reviewed 06/2019
 


BASICS

DESCRIPTION

  • Kawasaki syndrome (KS) is a self-limited acute, febrile, systemic vasculitis of small- and medium-sized arteries that predominantly affects patients age 6 months to 5 years and is th...

DIAGNOSIS

≥5 days of fever and ≥4 of the following 5 principal clinical features; or <4 features and presence of coronary artery disease on 2D echocardiography: 
  • Bilateral conjunctival injection wit...

TREATMENT

GENERAL MEASURES

Use antibiotics until bacterial etiologies are excluded (e.g., sepsis or meningitis). 

MEDICATION

  • Optimal therapy is IVIG 2 g/kg IV over 10 to 12 hours with high-dose aspirin p...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

With aneurysms, contact and high-risk sports should be avoided. 

Patient Monitoring

  • Repeat ECG and echocardiogram at 6 to 8 weeks. If abnormal, repeat at 6 to 12 mo...

REFERENCES

1
McCrindle BW, Rowley AH, Newburger JW, et al; for American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee of the Council on Cardiovascular Disease in the Y...

ADDITIONAL READING

  • Huang SK, Lin MT, Chen HC, et al. Epidemiology of Kawasaki disease: prevalence from national database and future trends projection by system dynamics modeling. J Pediatr.  2013...

CODES

ICD10

M30.3 Mucocutaneous lymph node syndrome [Kawasaki] 

ICD9

446.1 Acute febrile mucocutaneous lymph node syndrome [MCLS] 

SNOMED

75053002 Acute febrile mucocutaneous lymph node syndrome (disorder)...

CLINICAL PEARLS

  • The diagnosis of KS rests on a constellation of clinical features.

  • Once KS is suspected, all patients need an inpatient cardiac evaluation, including ECG and echocardiogram.

  • Expert recom...

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