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KEY FEATURES

ESSENTIALS OF DIAGNOSIS

  • Fever lasting 5 or more days with at least 4 of the following:

    • – Bilateral nonexudative conjunctival injection

    • – Injected lips or pharynx, or “strawberry tongue”

    • – Acute nonsuppurative cervical lymphadenopathy

    • – Erythema of the palms and soles, or edema of the hands and feet

    • – Polymorphous exanthem

  • Exclusion of common bacterial and viral infections

  • Coronary artery aneurysms, myocarditis, and valve regurgitation

GENERAL CONSIDERATIONS

  • May have a genetic and ethnic predisposition

  • Also called mucocutaneous lymph node syndrome

  • Acute vasculitis of unknown etiology, which occurs mostly in infants and children

  • One of the most common vasculitides of childhood

  • Heart failure may rarely complicate the acute phase of illness due to myocarditis

  • Routine treatment with intravenous immune globulin (IVIG) and aspirin usually results in rapid clinical improvement

  • Myocardial dysfunction during or after the second week of illness may suggest coronary artery aneurysm and resultant ischemia or infarction

  • In severe cases, coronary or peripheral artery occlusions may occur and children may experience myocardial infarction, arrhythmias, or sudden death

  • Kawasaki disease accounts for 5% of acute coronary syndromes in adults < 40 years of age

CLINICAL PRESENTATION

SYMPTOMS AND SIGNS

  • Unexplained fever in an infant or child

  • Skin rash

  • Heart failure symptoms in patients with myocarditis in the first week of illness

  • Chest pain, arrhythmias, or sudden death in patients

PHYSICAL EXAM FINDINGS

  • Bilateral conjunctival injection

  • Oral mucous membrane changes, including injected or fissured lips, injected pharynx, or “strawberry tongue”

  • Palmar or solar erythema or edema

  • Periungual desquamation

  • Polymorphous rash

  • Cervical lymphadenopathy

  • Tachycardia out of proportion to the degree of fever

  • Cardiac gallop sounds and muffled heart tones

  • In severe cases, palpable brachial artery aneurysms; cold, pale, cyanotic, or rarely gangrenous digits

DIFFERENTIAL DIAGNOSIS

  • Streptococcal and staphylococcal infections

  • Measles, enterovirus, and adenovirus infections

  • Systemic allergic reactions to medications

DIAGNOSTIC EVALUATION

LABORATORY TESTS

  • Acute-phase reactants (C-reactive protein, erythrocyte sedimentation rate, and alpha1-antitrypsin) elevation

  • White blood count: leukocytosis and left shift in white blood count

  • Reactive thrombocytosis

  • Normocytic, normochromic anemia

  • Pyuria of urethral origin (may be missed by bladder tap or catheterization)

  • Elevated liver transaminase levels or hyperbilirubinemia due to intrahepatic congestion

  • Mononuclear pleocytosis without low glucose or elevated protein in cerebrospinal fluid

ELECTROCARDIOGRAPHY

  • Low R-wave voltages

  • T-wave flattening

  • Findings of acute myocardial infarction in rare cases

IMAGING STUDIES

  • Echocardiography: small pericardial effusion, mild left ventricular dilatation, reduced left ventricular systolic function, mitral regurgitation, aortic root dilation, mild aortic regurgitation, diffuse dilation of coronary artery lumen, or coronary artery aneurysms

    • – Limited usefulness for measurement of coronary aneurysms after the acute phase of the illness

DIAGNOSTIC PROCEDURES

  • Stress testing: ...

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