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Kawasaki syndrome (KS) is an acute, febrile, self-limited, infectious, multisystem vasculitis, which occurs in young children. Fever is often prolonged and coronary aneurysms may lead to myocardial infarction and death.
Kawasaki syndrome has a peak incidence between 1 and 2 years of age. The disease is rare in children older than 8 years old, and it is uncommon before 3 months of age.
Boys are affected more often than girls by a ratio of 1.5 to 1. Japanese and Korean children are at greatest risk (145 per 100,000).
The rate for European children is 9 per 100,000, and the rate for African children is 20 per 100,000.
Pathophysiology
Kawasaki syndrome is a multisystem vasculitis with a predilection for the coronary arteries. KS is probably caused by an infectious agent, based on the acute febrile exanthematous character of the disorder and the occurrence of epidemics.
During the first 10 days of illness, an inflammatory infiltrate appears in the coronary arteries, with pancarditis and pericarditis. Death during this phase is usually caused by an arrhythmia, although fatal heart failure may sometimes occur.
Ten to 40 days from the onset of fever, the most common cause of death in untreated children is myocardial infarction caused by coronary aneurysms.
After 40 days, healing and stenosis of the post-aneurysmal coronary kowasaki sindrome
Clinical Manifestations
Abrupt onset of high but remittent fever between 38 and 41 degrees C is characteristic of KS.
Within 2 to 5 days, the child develops other diagnostic signs of KS: Conjunctival injection, mouth changes, an erythematous rash, changes in the hands and feet, and unilateral cervical lymphadenopathy.
Eye involvement consists of
The lips are initially bright red, progressing over 3 days to swelling, cracking, and bleeding. Prominent papillae on the tongue create a strawberry appear ance, and the oral cavity and pharynx is diffusely erythematous.
The skin rash is deeply erythematous with slightly raised margins, varying in size from 2 to 3 mm papules to large plaques covering several centimeters. The rash often is urticarial and may be intensely pruritic. The rash
Treatment of Kawasaki syndrome consists of