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Kawasaki Syndrome (KS)
Monday, 23 June 2003
Thursday, 10 November 2005
Kawasaki Disease

What

Kawasaki Syndrome (KS) is a relatively rare and acute disease seen in young children that is characterized by an unexplained fever lasting 5 days or more, rash, cracked red lips and mouth, red eyes, swollen lymph nodes in the neck, and is sometimes accompanied by problems in the blood vessels around the heart.

Who

Kawasaki Syndrome occurs worldwide and affects all races; however, in the United States it is most prevalent in the Asian American and Pacific Islanders populations, with an overall annual incidence of 18 per 100,000 children less than 5 years old in the United States. Most cases occur in infants and children under the age of 5. It is rarely seen in children over 8 years old. For reasons unknown, boys are affected 50 percent more commonly than girls.

Signs and Symptoms

Children afflicted with KS have a high fever (103-105°F) that does not respond to antibiotics. It usually lasts more than 5 days and has been observed to last as long as 2-3 weeks. The conjunctiva around both eyes is congested and red (i.e. they look blood shot). Redness and cracking is observed around the lips, mouth, and throat; and the tongue becomes red and swollen. A rash often covers the entire body and is sometimes followed by peeling of the skin on the hands and fingers. The extremities may also exhibit a hard swelling and redness. The child is often extremely irritable.

Possible Causes

The cause of Kawasaki Syndrome is unknown. Kawasaki Syndrome is a systemic vasculitis (an abnormal inflammation of the body’s blood and lymph vessels) that affects children. It does not appear to be obviously contagious, but since community-wide outbreaks of the disease have occurred, and because the disease will ultimately get better on its own, this suggests that it may be caused by an infectious agent. Suggestions that certain viruses or bacterial toxins are the cause of the majority of cases have not been proven. However, recently one study showed a possible link between a new strain of the Coronavirus (a virus that can cause the common cold) and Kawasaki Syndrome. Most likely, there are many triggers that cause the inflammatory reaction seen in KS. There is no vaccine or way to prevent a child from getting the disease, and it does not seem to be contagious in the usual ways.

Diagnosis

There is no specific laboratory test to diagnose Kawasaki Syndrome. Cases are confirmed when the history of the illness and the signs & symptoms meet most of the characteristics by which the disease is defined.

Treatment

Most patients are admitted to the hospital where they can be closely monitored. An older child with a mild form of the disease may be cared for on an outpatient basis. Children with Kawaski Syndrome should get high dose aspirin and IVIG (a preparation of antibodies from blood donors that seems to decrease the inflammation the disease causes). These treatments are very different from those used to treat infections or other problems that look similar, which is why it is important to make a diagnosis. However, antibiotics are administered early on until other causes of fever have been excluded. IV fluids may be needed if fever and irritability make feeding difficult or impossible. Alternative drugs may be prescribed particularly for treating some of the potential complications of Kawasaki Syndrome. Prompt and adequate treatment can often prevent the complications of the disease, like the heart problems.

Prognosis

Kawasaki Syndrome is a rapid onset, short-course disease with a low risk of coming back. The major potential complication involves the cardiovascular system. Roughly 20% of untreated patients develop an abnormal dilation (aneurysm) of the coronary vessel, especially male infants with a fever longer than 2 weeks. These can cause heart attacks, which are the main risk from Kawasaki syndrome. Occasionally other places in the body can get these aneurysms as well. Treated patients usually do not get aneurysms. If there are no aneurysms, the outcome of Kawasaki syndrome is very good, with no long term problems. If there are aneurysms there is a small chance of dying from a heart attack, and these children need to be checked frequently by a pediatric cardiologist.

Connect with other parents

In the spirit of community and support, Madisons Foundation offers the unique service of connecting parents of children with rare diseases. If you would like to be connected to other parents of children with this disease, please fill out this brief form.

Weblinks

Kawasaki Families' Network
This is a great, well organized site which offers information about the syndrome and seeks to share information and offer support to patients and families affected by Kawasaki syndrome. A link is also available to a parent's guide in Spanish.

Health Topics A-Z
This site offers concise information about the disease that is not too technical.

The Mayo Clinic
Trusted site provides comprehensive information about the disease.

Google Search for Kawasaki Syndrome (KS)

References and Sources

1. www.intelihealth.com/IH/ihtIH/WSIHW000/9339/23825.html 2. ourworld.compuserve.com/homepages/kawasaki/ 3. www.principalhealthnews.com/topic/topic100587047 4. rarediseases.info.nih.gov/news-reports/workshops/KAWASAKI19970513_et.html 5. Esper F, et al. (2005) Association between a novel human coronavirus and Kawasaki disease. J Infect Dis Feb 15;191(4):499-502. 6. Robert Sundel, MD (2005) Etiology and clinical manifestations of Kawasaki disease. UpToDate.