Kawasaki disease

Kawasaki disease or Kawasaki syndrome is a fairly rare condition of young children of unknown cause that involves a vasculitis, or inflammation of the blood vessels. It is characterised by high fever, and characteristic redness of the eyes, lips and mucous membranes most commonly. Sometimes there is marked swelling of the lymph nodes of the head and neck region. This condition can lead to damage to the vessels of the heart (microaneurysms) and to an increased risk of heart attack. Thankfully, there is now available a dramatically effective treatment, high dose intravenous gamma globulin, which when coupled with early diagnosis seems to give this condition a much brighter outlook. This disease is one of the few conditions for which (high dose) aspirin is prescribed in childhood.

The strict diagnostic criteria for Kawasaki disease are fever that persists for at least five days and at least four of the following five findings:

  • Both eyes exhibit inflammation without discharge
  • Lymph node swelling in the neck, usually a single red, hard node at least 3/4 inch in diameter
  • Rash, variable type
  • redness of the mucous membranes in the mouth, usually within 1-3 days of the beginning of fever
  • Purple-red discolorations of the palms and soles, often painful enough to interfere with walking

The disease is believed to be caused by an infectious agent - probably viral - that only affects susceptible children. It occurs in outbreaks periodically and sweeps in waves through the country. It is 1-1/2 times more frequent in boys compared to girls. Eighty percent of cases occur in children less than five years old, but it spares very young infants (probably because of immunity acquired from their mothers before birth). Peak incidence of the disease in the US is in winter and spring.

Careful followup by pediatric cardiology is warranted for affected children, because of the risk of coronary artery aneurysm formation (ballooning out of a weakened area) during the recovery phase of the illness.

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