KAWASAKI SYNDROME

KAWASAKI SYNDROME


Introduction
Kawasaki syndrome (KS) was first described by Tomisaku Kawasaki in the Japanese-language medical literature in 1967. At that time, he reported his experience with 50 children who presented from 1961 to 1967 with symptoms distinct from other known childhood illnesses. He termed the condition “mucocutaneous lymph node syndrome” and originally thought that the syndrome represented a benign childhood illness. [Rowley A H and Shulman S T, 1998]

Kawasaki syndrome (KS) is the most common cause of acquired heart disease in children. This acute, self-limited vasculitis results in permanent coronary artery damage in up to 25% of untreated children. High dose intravenous gamma globulin reduces the risk of coronary artery aneurysm to 3-5% if administered early in the course of disease. However, without a specific diagnostic test, affected children may be difficult to recognize, and delayed diagnosis and treatment continue to result in potentially preventable morbidity and mortality. The etiology of Kawasaki syndrome (KS) remains unknown despite 30 years of intensive search for an agent. [Pediatr Infect Dis J. 2008;27(11):981-985)]

Incidences of Kawasaki Syndrome
In the continental United States, population-based and hospitalization studies have estimated an incidence of KS ranging from 9 to 19 per 100,000 children younger than 5 years of age. Approximately 4248 hospitalizations with KS, of which 3277 (77%) were for children under 5 years of age, were estimated among children younger than 18 years of age in the United States in the year 2000. In 2006, the number of hospitalizations with KS was 5523 (standard error [SE] 289) and the percentage of children under 5 years of age remained the same (unpublished data).

Outside the United States, the disease is most frequently observed in Japan, Taiwan, and Korea. The prevalence of Kawasaki disease increased from 1967 to the mid 1980s and has leveled out at 5000-6000 cases per year. Several epidemics occurred in Japan during the years 1979, 1982, and 1985. The current Japanese incidence is approximately 112 cases per 100,000 population.

In Indonesia, an investigation of KS on January 2005 found 100 cases, generally in children 3 months of age to 4 years of age. In the report, that wasn’t much of an accidence in children under 3 months of age or in children older than 8 years of age. Statistic-based; estimated that in Indonesia will be found 6000 – 7000 cases per year.

Causes

Kawasaki disease is a poorly understood illness. The cause has not been determined. Although, some now believe that many factors (viruses, staphylococci "super antigens") are capable of triggering a final common pathway that results in immune activation. It may be an autoimmune disorder. The disorder affects the mucus membranes, lymph nodes, walls of the blood vessels, and the heart.
Kawasaki disease can cause inflammation of blood vessels in the arteries, especially the coronary arteries. This inflammation can lead to aneurysms. An aneurysm can lead to a heart attack, even in young children, although this is rare.

Symptoms

1. High-grade fever (greater than 39 °C or 102 °F; often as high as 40 °C or 104 °F) that normally lasts for more than 5 days if left untreated.
2. Red eyes (conjunctivitis) without pus or drainage, also known as "conjunctival injection"
3. Bright red, chapped, or cracked lips
4. Red mucous membranes in the mouth
5. Strawberry tongue, white coating on the tongue or prominent red bumps (papillae) on the back of the tongue
6. Red palms of the hands and the soles of the feet
7. Rash which may take many forms, but not vesicular (blister-like), on the trunk
8. Swollen lymph nodes (frequently only one lymph node is swollen), particularly in the neck area
9. Joint pain (arthralgia) and swelling, frequently symmetrical
10. Irritability
11. Tachycardia (rapid heart beat)
12. Peeling (desquamation) palms and soles (later in the illness); peeling may begin around the nails
13. Beau's lines (transverse grooves on nails)
14. Increased peeling on palms of the hand
15. May find breathing difficult.

Additional symptoms may include: diarrhea, vomiting, abdominal pain, cough and runny nose.

Complications

The main complication of Kawasaki disease is development and rupture of coronary artery aneurysms. These aneurysms may also cause heart problems in later life. Other complications include dehydration and limited mobility from joint inflammation.

Prevention

There are no known measures that will prevent this disorder.

Treatment

Children with Kawasaki disease are admitted to the hospital. Treatment must be started as soon as the diagnosis is made to prevent damage to the coronary arteries and heart.
Intravenous gamma globulin is the standard treatment. It is given in high doses. The child's condition usually greatly improves within 24 hours of treatment with IV gamma globulin.
High-dose aspirin is often given along with IV gamma globulin.
Even when they're treated with aspirin and IVIG, up to 25% of children may still develop problems in their coronary arteries. Some research has suggested that adding steroids to the usual treatment routine may improve a child's outcome, but more research is needed.


See more informations : kidshealth, americanheart, emedicine, cdc