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Kawasaki Disease

What is Kawasaki disease?

Kawasaki disease is the most common form of vasculitis that primarily affects children. The disease produces irritation and inflammation of many tissues of the body, including the hands, feet, whites of the eyes, mouth, lips, and throat. High fever and swelling of the lymph nodes in the neck also are characteristic of this illness. The inflammation is uncomfortable, but resolves with time. However, the main threat from Kawasaki disease comes from its effect on the heart and blood vessels. Heart-related complications can be temporary or may affect the child long-term.

Another name for Kawasaki disease is mucocutaneous lymph node syndrome.

How often does it occur?

Kawasaki disease is fairly common in the US. According to the American Heart Association, the illness is a major cause of heart disease in children. According to the most recent statistics, about 4,300 new cases were diagnosed in the US in 2000, and the incidence is on the rise. Kawasaki disease, together with acute rheumatic fever, is the leading cause of acquired heart disease in children in the US and Japan.

Who is affected by Kawasaki disease?

Kawasaki disease occurs more often in Japan than in any other country. In the US, children of Asian or Asian American heritage are affected more often than other races, although Kawasaki disease can occur in any racial or ethnic group.

The vast majority of children who develop Kawasaki disease are under age five. The average age child seen with the illness is two years old. It occurs in boys more often than in girls.

What causes Kawasaki disease?

It is not clear what causes Kawasaki disease. Scientists believe a virus may be responsible, but current research is still underway. Kawasaki disease does not appear to be contagious, nor does it appear to be hereditary. It was once thought that Kawasaki disease was linked to recent rug or carpet cleaning; however, no studies have shown this to be a cause of the disease.

It is rare for more than one child in a family to develop the disease. As a result, less than 2 percent of persons with Kawasaki disease develop the disease more than once.

Why is Kawasaki disease a concern?

Kawasaki disease can be a very uncomfortable illness, since it causes fever, as well as irritation and inflammation in many tissues of the body. However, these symptoms usually run their course and resolve within a few weeks. The primary concern with Kawasaki disease is heart and blood vessel involvement.

The coronary arteries are the vessels that provide the heart muscle with an oxygen-rich blood supply. Kawasaki disease can weaken the wall of one or more of the coronary arteries, causing it to bulge or balloon out. This weakened, ballooned area is called an aneurysm.

Blood clots can form in the ballooned area and possibly block the blood flow through the coronary artery. When this happens, the heart muscle will no longer receive an adequate supply of oxygen-rich (red) blood, and the heart muscle can be damaged. On rare occasions, the aneurysm can also burst.

The illness may also cause the heart muscle (myocardium) to be irritated and inflamed, as well as the membrane covering the heart (pericardium). Irregular heart rhythms and heart valve problems may also occur with Kawasaki disease.

In most cases, the effects on the heart caused by Kawasaki disease are temporary, and resolve within five or six weeks. However, coronary artery problems may sometimes persist for longer periods of time.

What are the symptoms of Kawasaki disease?

The following are the most common symptoms of Kawasaki disease. However, each child may experience symptoms differently. Symptoms may include:

The symptoms of Kawasaki disease may resemble other conditions or medical problems. Always consult your child's physician for a diagnosis.

How is Kawasaki disease diagnosed?

Your child's physician will obtain a medical history, and perform a physical examination. Several of the above mentioned symptoms need to be present for your child's physician to consider Kawasaki's disease as the cause for the illness.

Diagnostic tests may also include:

Treatment for Kawasaki disease:

Aspirin and the Risk of Reye Syndrome in Children

Do not give aspirin to a child without first contacting the child's physician. Aspirin, when given as treatment for children, has been associated with Reye syndrome, a potentially serious or deadly disorder in children. Therefore, pediatricians and other healthcare providers recommend that aspirin (or any medication that contains aspirin) not be used to treat any viral illnesses in children.

Specific treatment for Kawasaki disease will be determined by your child's physician based on:

Prior to the diagnosis of Kawasaki disease by your child's physician, the goal of treatment is to provide comfort for your child. Fever reduction and increasing fluids are very helpful. Acetaminophen or ibuprofen is often used for fever.

Once the diagnosis of Kawasaki disease is made, your child's physician may prescribe aspirin to help decrease the inflammation that the illness produces, as well as to prevent clots from forming. However, always check with your child's physician before giving your child aspirin. Some children benefit from an intravenous (IV) medication called gamma globulin that may decrease the risk of the heart being affected. Your child may need to stay in the hospital for a few days if IV gamma globulin is prescribed.

Can Kawasaki disease be prevented?

Currently, there is no known way to prevent Kawasaki disease.

Long-term outlook after having Kawasaki disease:

About 80 percent of all children have a full recovery after Kawasaki disease resolves. If an aneurysm is detected, echocardiograms will be repeated periodically, sometimes for several years after the illness. Some heart problems may not be evident right away, so it is important to keep follow-up appointments with your child's physician, even if your child is feeling well.

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