Within five days, red, flushed spots appear - usually on the trunk. These spots can resemble hives, measles or scarlet fever. There may be congestion in the throat; the lips become red, dry and cracked and the tongue becomes a strawberry red color. During this time, the palms of the hands and soles of the feet turn purple-red and may swell. The base of the fingernails and toenails may become white spotted or streaked.
Within 10 days, the skin of the palms, the soles of the feet and around the finger and toenails may begin to peel off in sheets that retain the shape of the body after they peel away. Tender, swollen lymph nodes in the neck appear in about half the patients with Kawasaki syndrome. The illness can last two to more than 12 weeks.
More general symptoms may include:
Arthritis or pain in the large joints
Diarrhea
Swelling and tenderness of the urethra, the tube that carries urine from the bladder outside the body
Swelling of the gallbladder from fluid, and
Swelling of the membrane that cover the brain without an infection being present
Kawasaki syndrome can lead to life-threatening complications including:
Swelling and tenderness of the middle layer of the heart's muscle (acute myocarditis)
Arteries that enlarge and develop weak spots. The pressure of the blood moving through the vessels can cause the weak areas to balloon out (aneurysms)
Heart failure
Irregular heart rhythms (arrhythmias)
Swelling and tenderness of the membrane that covers the heart (pericarditis)
Swelling and inflammation of the heart and the arteries of the heart
Causes and Risk Factors of Kawasaki Syndrome
It is not yet known what causes this disease. It may be an infection or an abnormal response by the body to an infection. While it affects many racial and ethnic groups, persons of Japanese descent are most likely to develop this condition. Eight out of 10 children with this syndrome develop it before the age of five. Slightly more boys than girls develop Kawasaki syndrome.
Cases of Kawasaki syndrome can develop at any time of the year, but most often appear in the spring or winter. The syndrome sometimes appears in clusters in a community, although it is not clear how it might spread from one person to another.
Diagnosing Kawasaki Syndrome
A doctor will do a physical examination and tests to rule out other conditions. Because the condition affects different organs, a variety of tests may be needed. The doctor may order:
Blood tests to rule out infections, to uncover anemia (iron poor blood) if it exists. A person with Kawasaki syndrome may have a high white blood cell count, often with many immature white blood cells, especially in the acute stage of the illness
Coronary arteriography
Echocardiograms
Electrocardiograms to check for abnormalities in the electrical system that normally causes the heart to beat in a regular rhythm
Stress tests
Urine tests
A doctor will seek to rule out scarlet fever or measles, blood poisoning, mercury poison or juvenile rheumatoid arthritis, among others.
Treating Kawasaki Syndrome
A team of doctors including an experienced pediatric cardiologist or pediatric infectious disease specialist should be involved in treating a child with Kawasaki syndrome as well as a rheumatologist.
Treatment should be started as soon as possible, ideally within 10 days after the first symptoms appear. Aspirin should be given to reduce the fever and high-dose shots of immune globulin. Most patients respond quickly - within 24 hours of treatment.
Aspirin therapy may be continued for at least eight weeks until repeated echocardiograms echocardiograms show that there are no signs of artery aneurysms or inflammation. If there are abnormalities of the coronary arteries, the child may need to take aspirin indefinitely. If a large aneurysm of the coronary arteries is present, more intense therapy to prevent the blood from clotting may be needed. This might include prescribing coumadin or other anticoagulant.
Special care is needed for children who are taking aspirin on a long-term basis. Should they develop symptoms of flu, a pediatrician should be called immediately to avoid the small risk of developing Reye's syndrome.
It may be necessary to use a different drug until the child no longer has the flu symptoms. Children who are on aspirin therapy for Kawasaki syndrome should receive an influenza vaccination each year.
Because of the high doses of immune globulin given, a child may be less responsive to vaccinations made up of live vaccines. Childhood vaccinations should be delayed until the effects of the immune globulin have worn off.
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