Kawasaki disease (KD) is a rare condition that causes inflammation of blood vessels (vasculitis) and several other manifestations noted below. KD usually occurs in children younger than 5, more often in boys than girls. In the United States, about 4000 new cases are diagnosed each year. It’s the leading cause of acquired heart disease in children.
The cause is unknown, but an infectious agent may trigger the inflammation in people with a genetic predisposition to it. KD isn’t contagious, however. It is not a hereditary disorder.
Symptoms include fever that lasts for more than 5 days, and is associated with red swollen eyes (conjunctivitis); cracking lips; strawberry-colored tongue; red, swollen, sore throat; swollen lymph glands in the neck; and rash on the trunk and limbs. Complications that may mean heart involvement include shortness of breath; chest pain; and symptoms of congestive heart failure, such as leg swelling, being unable to lie flat in bed, suddenly waking up short of breath, and shortness of breath with exertion.
The health care provider makes a diagnosis from physical examination and symptoms: fever lasting more than 5 days plus four of five other features. These features include inflammation of the whites of the eyes (conjunctivitis) and swollen eyes; mucous membrane changes (red tongue and dry, cracked lips); swollen hands and feet; swollen neck lymph glands; and rash on the trunk of the body.
The health care provider may order blood tests and x-rays, but no specific blood test can diagnose KD.
For suspected heart involvement, echocardiography (a sonogram of the heart) and cardiac catheterization can be done. In catheterization, the doctor puts a tube into the leg artery and moves it up into heart arteries. Dye is then injected to show blockages in coronary (heart) arteries.
Aspirin is used for fever, rash, and pain and to prevent blood clots in coronary arteries. Intravenous immunoglobulin can significantly reduce coronary artery disease and improve the heart’s pumping if given early. For coronary artery and heart involvement, the health care provider may suggest percutaneous transluminal coronary angioplasty. In this procedure, a balloon-tipped tube is put into the coronary artery. The balloon is inflated to open the diseased artery. A child with many diseased vessels may have coronary artery bypass surgery. Heart transplant may sometimes be considered.
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