MONDAY, Oct. 25 (HealthDayNews) -- The American Heart Association has issued revised guidelines to help doctors and nurses diagnose, treat, and manage care for children with Kawasaki disease.

The new guidelines are contained in a scientific statement published in the current issue of Circulation.

Kawasaki disease, an acute inflammation of blood vessels, is the leading cause of acquired heart disease among children in the United States. It affects more than 4,000 children in the United States each year, and about 80 percent of cases involve kids younger than 5 years old.

There are no laboratory tests that can accurately diagnose Kawasaki disease. Classic criteria for the disease include fever for at least five days, along with four of the five symptoms detected by physical examination:

• Both eyes are red, usually without drainage or crusting.
• Lips and mouth are often bright red, and the top layer of the tongue peels off, leaving the tongue red and glossy.
• Hands and feet may be red and swollen. In the second week, skin under the fingernails and toenails begins to peel.
• A rash is usually present and often worse in the groin area.
• Lymph nodes in the neck may be swollen.

"In the past, physicians have relied on the classic criteria to decide which children should be treated for Kawasaki disease. We now understand that some children with Kawasaki disease never fulfill these classic criteria, yet are at risk for developing coronary artery aneurysms," lead author Dr. Jane W. Newburger, a professor of pediatrics at Harvard University, said in a prepared statement.

An aneurysm is an abnormal ballooning of a blood vessel.

"Children with Kawasaki disease who are not treated with high-dose intravenous gamma globulin within the first week to 10 days of fever onset have five times the risk of developing coronary artery aneurysms. Sometimes the diagnosis of Kawasaki disease is not made until a child or adult (who had Kawasaki disease as a child) has a heart attack," Newburger said.

The new guidelines propose an algorithm to help doctors decide which children with fever for at least five days and with fewer than four of the classic criteria for Kawasaki disease should be studied with an echocardiogram and/or be treated for Kawasaki disease.

The revised guidelines still recommend that children in the acute phase of Kawasaki disease be treated with intravenous gamma globulin and aspirin.

"The impact of Kawasaki disease in childhood on outcomes later in life depends to a large extent upon the amount of damage to the coronary arteries during the acute illness. The new recommendations tailor the long-term management of the disease according to the threat to coronary health," Newburger said.

More information

The Nemours Foundation has more about Kawasaki disease.