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Monday, January 05, 2009

Kawasaki Disease

According to the American Heart Association, more than 4,000 cases of the disease are diagnosed annually in the United States. It occurs more often in boys of Japanese and Korean descent, but has been identified in children of all ethnicities and races, Alenick said. The danger of Kawasaki syndrome is that it can cause large aneurysms in the blood vessels that feed blood to the heart, said Alenick.

“Kawasaki doesn’t come in degrees of severity, but it varies in that it may cause no aneurysms, small aneurysms, moderate aneurysms or giant aneurysms,” he said. “Giant aneurysms are more common in babies. But it’s the aneurysms that form in the vessels and the arteries that feed the heart that have the potential to cause a fatal heart attack.”

The disease was first identified in Japan in 1967 by Dr. Tomisaku Kawasaki, and the cause of the illness is still unknown, Alenick said. There also is no blood test to identify the illness. Instead, patients are given a clinical diagnosis based on whether they display at least five of these six symptoms: a high fever that lasts for more than five days, red lips and tongue, swelling of the hands and feet, bloodshot eyes, rash and swollen glands.

In a 2003 interview on the Montel Williams show, Preston talked about her son’s struggle with Kawasaki disease. “It causes swelling in the organs, so your heart can swell, different important organs can swell,” she said. “We thought at one point we were going to lose him.” (Jett Travolta)

Actress Kelly Preston has said her 16-year old son with husband John Travolta became very sick when he was 2 and was diagnosed with Kawasaki disease, an illness that affects the blood vessels in young children. Jett Travolta, died Friday after suffering a seizure at the family’s vacation home in the Bahamas, though it wasn’t immediately clear whether the death was related to his illness. The boy was found in a bathtub, where he reportedly fell and hit his head. (Source)

Kawasaki disease can cause vasculitic changes (inflammation of blood vessels) in the coronary arteries and subsequent coronary artery aneurysms. These aneurysms can lead to myocardial infarction (heart attack) even in young children. Overall, about 10–18% of children with Kawasaki disease develop coronary artery aneurysms with much higher prevalence among patients who are not treated early in the course of illness. Kawasaki disease and rheumatic fever are most common causes of acquired heart disease among children in the United States.

What are the consequences of vasculitis?

In an extreme situation, when a segment of a blood vessel becomes weakened, it may then stretch and bulge (called an “aneurysm”). The wall of the blood vessel can become so weak that it ruptures and bleeds. Fortunately, this is a very rare event.

If a blood vessel becomes inflamed and narrowed, blood supply to that area may be partially or completely eliminated. If collateral blood vessels (thought of as alternate routes of blood supply) are not available in sufficient quantity to carry the blood to such sites, the tissue supplied by the affected blood vessels will die. This is called infarction.

Kawasaki disease often begins with a high and persistent fever that is not very responsive to normal doses of paracetamol (acetaminophen) or ibuprofen. The fever may persist steadily for up to two weeks and is normally accompanied by irritability. Affected children develop red eyes, red mucous membranes in the mouth, red cracked lips, a “strawberry tongue“,[11] iritis, keratic precipitates (detectable by an ophthalmologist but usually too small to be seen by the unaided eye), and swollen lymph nodesSkin rashes occur early in the disease, and peeling of the skin in the genital area, hands, and feet (especially around the nails and on the palms and soles) may occur in later phases. Some of these symptoms may come and go during the course of the illness. If left untreated, the symptoms will eventually relent, but coronary artery aneurysms will not improve, resulting in a significant risk of death or disability due to myocardial infarction (heart attack). If treated in a timely fashion, this risk can be mostly avoided and the course of illness cut short

Childrens Hospital Boston


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