Kawasaki Disease

Diagnostic criteria, clinical findings, diagnostic tests & treatments

Kawasaki Disease is the leading cause of heart disease in children with a high incidence in children of Asian ancestry
The majority of cases occur in children < 5 years of age
Cardiac problems are major cause & concern of the disease
CBC- (WBC ↑), ESR ↑, C-reactive protein ↑
Echocardiogram should be ordered in acute phase as well as follow up echocardiograms
Treatment is IV gamma globulin and Aspirin
Kawasaki disease is not contagious
Without treatment, duration of fever is about 12 days
Complications & symptoms of Kawasaki disease should resolve in 6-8 weeks following treatment, but myocardial infarction & sudden death can occur months to years later
Coronary aneurysms occur in 20-25% of untreated patients
Carditis may involve the pericardium, myocardium & endocardium
Mitral & aortic regurgitation may develop
Myocardial infarction is the major cause of death for patients with Kawasaki disease

Diagnostic Criteria

Fever is the prominent symptom, lasting 1-2 weeks or longer despite use of antibiotics & antipyretics
Fever at least for 5 days with 4 of the 5 symptoms:
♦Changes in extremities (erythema & edema in hands & feet, desquamation of fingertips)
♦Polymorphous exanthema (rash)
♦Bilateral painless conjunctival injection without exudate (red eyes)
♦Changes in lips & oral cavity (strawberry tongue, erythema & cracking of lips)
♦Cervical lymphadenopathy (usually unilateral & involving at least 1 node ≥ 1.5 cm in diameter)

PEEL (Menmonic)

Clinical Findings

Perineal rash & desquamation in subacute phase
During convalescence, fingernails may show furrows or Beau’s lines
Respiratory: cough, rhinorrhea, and pulmonary infiltrate
Gastrointestinal: diarrhea, vomiting, and pain, hydrous of gallbladder, paralytic ileum, and mild jaundice
Joints: arthralgia & arthritis
Neurological: irritability & facial palsy (rare)

Diagnosis

Diagnostic Tests

No specific diagnostic tests are available but order:

CBC (leukocytosis with left shift)
Erythrocyte sedimentation rate (ESR) – usually elevated
C-reactive protein- usually elevated
Urinalysis (pyuria & sometimes proteinuria)
Serum transaminases- usually elevated
Albumin (decreased in acute phase)
Lumbar puncture (mononuclear pleocytosis in CSF)
Echocardiogram
Coronary angiography is considered by some as the “gold standard” to assess vascular artery disease but has risks
Consult specialist & arrange for hospitalization for patients with suspected Kawasaki disease
Patient should be managed by a cardiologist

Differential Diagnosis

Treatment

Treatment is supportive care & anti-infammatory therapy

Refer- consult cardiologist & arrange for hospitalization
Treatment should begin immediately: treat with intravenous gamma globulin & aspirin for the first 10 days
Treatment on or before 5 days of fever may prevent coronary inflammation and damage to arteries
Instruct parents to notify health care provider immediately if child is suspected of having influenza or varicella (↑risk of developing Reye’s syndrome from taking aspirin)
Instruct parents on signs & symptoms of possible complications: arthralgia,chest pain, & palpitations

Subsequent Immunizations

Do not administer varicella & measles vaccines for 11 months after gamma globulin therapy
May administer other scheduled immunizations
Administer influenza vaccine yearly to patients 6 months-18 years of age

Follow Up

Patients should be examined frequently during the first 2 months to detect arrhythmias, heart failure, valvular problems, & myocarditis
Echocardiogram should be ordered in acute phase followed by subsequent echocardiograms at 3-8 weeks after onset of illness
Additional echocardiograms will depend on degree of coronary artery involvement
Patients should be managed by cardiologist

Pictures of Kawasaki Disease