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Excerpt from Kawasaki Disease


Synonyms, Key Words, and Related Terms: Kawasaki disease, Kawasaki syndrome, KD, bilateral conjunctival injection, bilateral nonpurulent conjunctivitis, bilateral conjunctival erythema, mucocutaneous lymph node syndrome

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Background

Kawasaki disease (KD), also known as Kawasaki syndrome, was first described in Japan in 1967 by the pediatrician Tomisaku Kawasaki.1

Since its original description, Kawasaki disease has been reported worldwide in children of all ethnic origins. Originally, Kawasaki disease was believed to be an acute, self-limiting febrile disease with an excellent prognosis; however, subsequent reports indicated that nearly 2% of patients with Kawasaki disease died from the illness, usually as a result of secondary cardiac sequelae.

See related CME at Highlights of the Pediatric Academic Societies and Asian Society for Pediatric Research Joint Meeting.

Pathophysiology

Kawasaki disease is a systemic vasculitis of unknown etiology that affects the small- and medium-sized blood vessels of the body, in particular, the coronary arteries.

Frequency

United States

The yearly incidence of endemic Kawasaki disease in the United States is 67 cases per 100,000 children who are younger than 5 years. Approximately 3,000 hospitalizations occur annually in the United States as a result of Kawasaki disease. Seasonal peaks occur in the spring and winter with a relative paucity of cases in the summer months.

International

The incidence in Europe is comparable to that of the United States. In Japan, where Kawasaki disease has the highest incidence, an estimated 5,000-6,000 cases occur annually.

Race

  • The incidence of Kawasaki disease remains the highest in children of Japanese descent, although the disease has now been reported in children of all ethnic origins.
  • In the United States, Kawasaki disease occurs most often in children of Asian ancestry, followed by African Americans. The lowest incidence is in whites.

Sex

The male-to-female ratio is approximately 1.5:1.

Age

  • Of all Kawasaki disease cases, 80% occur in children younger than 4 years, with most cases in the United States presenting in children aged 1-2 years.
  • In Japan, the highest incidence occurs in persons aged 6-12 months. Cases in children older than 14 years are rare.

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