Kawasaki Syndrome

Kawasaki syndrome (KS) is an acute febrile illness of unknown aetiology that primarily affects children younger than 5 years of age. Kawasaki syndrome was first described in Japan by Tomisaku Kawasaki in 1967, and the first cases outside of Japan were reported in Hawaii in 1976.

Kawasaki syndrome is characterised by fever, rash, swelling of the hands and feet, irritation and redness of the whites of the eyes, swollen lymph glands in the neck, and irritation and inflammation of the mouth, lips, and throat. Serious complications of Kawasaki syndrome include coronary artery dilatations and aneurysms, and Kawasaki syndrome is a leading cause of acquired heart disease in the United States. The standard treatment with intravenous immunoglobulin and aspirin substantially decreases the development of these coronary artery abnormalities.

Kawasaki syndrome occurs worldwide, with the highest incidence in Japan, and it most often affects boys and younger children. Kawasaki syndrome may have a winter-spring seasonality, and community-wide outbreaks have been reported occasionally. In the continental United States, population-based and hospitalization studies have estimated an incidence of Kawasaki syndrome ranging from 9 to 19 per 100,000 children younger than 5 years of age. Approximately 4248 hospitalizations for Kawasaki syndrome, of which 3277 (77%) were for children under 5 years of age, were estimated among children younger than 18 years of age in the United States in the year 2000.

CDC uses hospital discharge data, a passive KS surveillance system, and special studies to describe the incidence and epidemiology of Kawasaki syndrome in the United States. The Kawasaki syndrome surveillance system has been maintained by CDC since 1976 and is based on voluntary reporting of Kawasaki syndrome cases by health care providers and local and state health authorities. A standardized case report form is used to collect information on patients.

Kawasaki Syndrome Case Definition

For epidemiologic surveillance, CDC defines a case of Kawasaki syndrome as illness in a patient with fever of 5 or more days duration (or fever until the date of administration of intravenous immunoglobulin if it is given before the fifth day of fever), and the presence of at least 4 of the following 5 clinical signs:

  • Rash
  • Cervical lymphadenopathy (at least 1.5 cm in diameter)
  • Bilateral conjuctival injection
  • Oral mucosal changes
  • Peripheral extremity changes.

Patients whose illness does not meet the above Kawasaki syndrome case definition but who have fever and coronary artery abnormalities are classified as having atypical or incomplete Kawasaki syndrome.

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