What is Kawasaki disease?

Kawasaki disease is a form of vasculitis, or inflammation of the blood vessels. It typically develops in young children with over 80% of patients under the age of 5, and affects boys more than girls.

Kawasaki disease was first identified by Tomasaku Kawasaki in Japan in 1967. It was also known as mucocutaneous lymph node syndrome, Kawasaki’s disease, Kawasaki’s syndrome and infantile polyarteritis.

Kawasaki disease is considered in the U.S. to be the leading cause of acquired heart disease in children.

What is the cause of Kawasaki disease?

The cause of Kawasaki disease has not been discovered. There are theories that it is the result of an infection, but this has not yet been proven. There are also those that believe it is an autoimmune condition. In addition, there appears to be a genetic link to development of the disease.

What are the symptoms of Kawasaki disease?

Kawasaki disease develops quickly and is very aggressive for several weeks. After the disease clears, there can still be cardiac complications that appear years later.

The symptoms include:

• High-grade fever, 102 °F to 104 °F, that lasts for more than 5 days if left untreated
• Conjunctivitis, also know as “Pink Eye”
• Chapped, bright red lips
• Red mucous membranes in the mouth
• Strawberry looking tongue
• Joint pain and swelling on both sides of the body
• Rapid heart beat
• Peeling skin on the palms and soles of the feet
• Palms of the hands and soles of the feet are red
• Swollen lymph nodes, usually in the neck area
• Irritability

Other symptoms can include abdominal pain, diarrhea, jaundice and meningitis.

What is the treatment for Kawasaki disease?

Children should be hospitalized and treated by a physician experienced in treating Kawasaki disease. The usual course of treatment includes intravenous immunoglobulin (IVIG) given in high doses. In addition, Kawasaki disease is one of the few cases where aspirin is used to treat children. Aspirin is given in high doses until the fever subsides, and then in low doses for two to three months to prevent blood clotting.

What is the prognosis?

If the disease is promptly diagnosed and treatment started quickly the recovery is generally rapid. If left untreated the patient will usually recover, although in a longer time frame than if treated. However, if untreated the risk of coronary artery disease is much higher.

Overall, about 2% of Kawasaki disease patients die from complications of coronary inflammation. Patients who have had Kawasaki disease should have an echocardiogram initially every few weeks, and then every 1–2 years to screen for progression of cardiac involvement.

It is also possible that the patient may experience a relapse of the symptoms shortly after treatment with IVIG which would require hospital treatment again. Also, treatment with aspirin can increase the risk of bleeding and has been associated with the risk of Reye’s syndrome in children.

Originally posted 2009-01-26 14:15:01. Republished by Blog Post Promoter

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According to the American Heart Association, Kawasaki disease is the most common cause of pediatric coronary artery disease with more than 4,000 new diagnoses each year. As many as a quarter of those will develop cardiac problems.

A recently completed 25-year follow up study has shown that coronary artery bypass grafting (CABG), or bypass surgery, provides long-term benefits for adolescents and children who have Kawasaki disease with cardiac involvement.

To evaluate the long-term success in patients who had undergone CABG surgery, Dr. Soichiro Kitamura, of the National Cardiovascular Center in Osaka, Japan, and his colleagues followed 114 patients for up to 25 years. All of the patients had bypass surgery between the ages of 1 and 19 years old.

Of these 114 patients, 95% were still alive 25 years later, and most of them were free from any limitations in daily activities, according to Dr. Kitamura. Even though the number of cardiac events increased through the 25 year follow-up, the majority of the events were managed successfully.

There were five deaths among the patients that were tracked, all cardiac in nature. All of these patients had a history of heart attack and frequent irregular heart beat.

At the conclusion of follow-up, three quarters of the surviving patients were taking one to six medications. They were all taking aspirin. Other medications included warfarin, ACE inhibitors, angiotensin receptor blockers, calcium antagonists, diuretics, nitrates, statins, and beta-blockers.

All of the survivors were symptom free at the last checkup and 80% did not have any limitations in performing daily activities. Most could play sports.

The researchers stated that Kawasaki disease has been increasingly treated with early gamma-globulin administration and PCI instead of surgery, although the long-term benefits of these therapies remain unknown.

According to the researchers, “prolonged observation is mandatory for these patients. By employing early additional therapeutic procedures such as percutaneous coronary intervention (PCI) and redo operations, the patients’ survival and quality of life were favorably influenced during 25 postoperative years.”

Dr. Kitamura performed the first bypass grafts in patients with Kawasaki disease in the late 1970s and early 1980s.

The research was published online in Circulation: Journal of the American Heart Association.

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