Kawasaki Disease
Basics
Basics
Basics
Description
Description
- Acute inflammatory process involving multiple organs
- Leading cause of childhood-acquired heart disease in developed countries
- Vasculitis is most severe in medium-sized arteries
- Acute cardiac sequelae:
- Coronary artery aneurysm:
- Often leads to stenosis after healing
- Giant aneurysm:
- Myocarditis
- Pericarditis
- Stages:
- Acute (lasts 1–2 wk):
- Fever
- Oral mucosal erythema
- Conjunctival injection
- Erythema and edema of hands and feet
- Cervical adenopathy
- Aseptic meningitis
- Hepatic dysfunction
- Diarrhea
- Myocarditis
- Pericardial effusion (20–40%)
- No aneurysms by ECHO
- Subacute (when fever, rash, and lymphadenopathy resolve until about 4 wk):
- Anorexia
- Irritability
- Desquamation of hands and feet
- Thrombocytosis
- Coronary artery aneurysms (20% if untreated)
- Risk for sudden death is highest
- Convalescent phase (about 6–8 wk):
- Clinical signs are absent
- ESR normalizes
- Epidemiology:
- 80% of cases occur in children <4 yr old; peak at 1–2 yr; rare in infants <3 mo old
- Adult cases have been reported
- Asians are at highest risk
- Males > females 1.5:1
- Genetics:
- Possible genetic predisposition
- Risks for nonresponse to standard therapy (10–15%):
- Elevated band count
- Low albumin level
- Abnormal initial ECHO
- Risks for development of coronary artery aneurysms:
- Extremes of age
- Male gender
- Prolonged fever
- Persistent fever after treatment
- Delay in diagnosis
- Increased WBC and/or band count
- Low hematocrit
- Significant increase in CRP and/or ESR
- High prevalence of Kawasaki disease (KD) related adverse cardiac events in young adults
- Up to 5% of young adults with MI have a history of known or suspected KD
Etiology
Etiology
- Unknown – believed to be infectious based on manifestations of disease, epidemics, and increased numbers of cases in winter and early spring
- Current theory:
- Activation of immune system in response to an antigen
- Genetically susceptible host
- May explain why certain ethnicities have higher incidence of disease:
- More prominent in Asian countries
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