Kawasaki disease in children

Herma Heart Institute physicians work closely with other pediatric physician experts at Children's Wisconsin to diagnose and treat all aspects of Kawasaki disease. Several diagnostic tests may be conducted to confirm Kawasaki disease. Kawasaki disease treatment starts with strategies to help your child feel more comfortable by reducing fever and inflammation. Efforts to prevent clots and other heart effects may also be recommended, including regular monitoring for signs of aneurysm.

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 also common Kawasaki disease symptoms. 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. The coronary arteries, small vessels that take blood to the heart muscle, are affected in as many as 20 percent of children with Kawasaki disease if not treated with IVIG. Another name for Kawasaki disease is mucocutaneous lymph node syndrome.

How often does Kawasaki disease in children 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. About 1,800 new cases are diagnosed in the US each year, 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 cases of Kawasaki disease in children are under age 5. The average age child seen with the illness is 2 years old. It occurs in boys twice as often as 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  in children 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. Less than 2 percent of persons with Kawasaki disease develop the disease more than once.

Why is Kawasaki disease a concern?

Kawasaki disease symptoms can be very uncomfortable causing 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.

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 most common Kawasaki disease symptoms?

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

• moderate to high fever (101° F to 104° F) that rises and falls for at least 5 days and up to three weeks
• irritability
• swollen lymph glands in the neck
• rash on the back, chest, abdomen, and/or groin
• bloodshot eyes
• sensitivity to light
• swollen, coated tongue
• dry, red, cracked lips
• dark red interior surfaces of the mouth
• red, swollen palms of hands and soles of feet
• peeling skin around the nailbeds, hands, or feet
• swollen, painful joints

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. At least 5 days of fever and 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:

• electrocardiogram (ECG or EKG) - a test that records the electrical activity of the heart, shows abnormal rhythms (arrhythmias or dysrhythmias), and detects heart muscle damage.
• echocardiogram (echo) - a procedure that evaluates the structure and function of the heart by using sound waves recorded on an electronic sensor that produce a moving picture of the heart and heart valves.
• x-ray - a diagnostic test which uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film.
• complete blood count (CBC) - a measurement of size, number, and maturity of different blood cells in a specific volume of blood. The physician will look for an elevation in the numbers of white blood cells which normally multiply in the presence of infection and may notice elevated platelet levels with Kawasaki disease as well.
• erythrocyte sedimentation rate (ESR or sed rate) - a  blood test that measures the amount of inflammation going on in the body. This is not a specific test to determine why or where the inflammation is occurring.
• urinalysis - testing of a urine sample for protein, red blood cells, white blood cells, or casts to indicate kidney disease associated with several rheumatic diseases.

Kawasaki disease treatment

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

• your child's age, overall health, and medical history
• extent of the disease
• your child's tolerance for specific medications, procedures, or therapies
• expectations for the course of the disease
• your opinion or preference

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 Kawasaki disease diagnosis 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. Intravenous medication called gamma globulin (IVIG) decreases the risk of the heart being affected and can significantly reduce the risk for long term complications for Kawasaki disease. IVIG is given in the hospital.

Can Kawasaki disease be prevented?

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

Long-term outlook after having Kawasaki disease

With appropriate Kawasaki disease treatment, about 95 percent of all children have a full recovery after the illness 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.