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Clinical Reference Systems: Pediatric Advisor 10.0

Kawasaki Disease

What is Kawasaki disease?

Kawasaki disease (also called mucocutaneous lymph node syndrome) is a disease that affects small and medium-sized arteries throughout the body. Arteries are vessels that carry blood from the heart to the tissues of the body. Children who get Kawasaki disease develop inflammation (swelling) of the walls of arteries. This inflammation causes your child to have:

  • a fever for 5 or more days
  • skin rash
  • swollen, dry, cracked lips or red, swollen tongue
  • red ("bloodshot") eyes
  • swollen lymph nodes in the neck
  • swelling and redness of the hands and feet, sometimes with peeling skin of the hands and feet, especially around the tips of the fingers and toes.

Kawasaki disease is rare. It usually affects children between the ages of 6 months and 5 years.

What is the cause?

There are many theories about why children get Kawasaki disease, but no one really knows the cause. Medical researchers have considered bacteria, viruses, and environmental chemicals or pollutants as possible causes, but none have proven to be a common link.

How is it diagnosed?

To diagnose Kawasaki disease, the doctor first needs to check if your child has other diseases that might cause similar symptoms. If your child has had a fever for many days and also has four or five of the other symptoms listed above, then the doctor will probably diagnose your child with Kawasaki disease. A diagnosis of "atypical Kawasaki syndrome" may be made when fever and fewer than four of the symptoms are present.

What are the complications of Kawasaki disease?

When untreated, Kawasaki disease can cause many serious problems including:

  • Inflammation of the heart muscle. The inflammation of the heart can make the heart muscle pump blood poorly.
  • Abnormalities of the coronary arteries. Coronary arteries, which are blood vessels that carry blood to the heart muscle, can form aneurysms (weak, bulging areas in the artery). Blood can clot in an aneurysm and eventually block blood flow. When blood flow to the heart is blocked, the heart muscle can be damaged.
  • Other problems include joint swelling (arthritis), poor digestion, diarrhea, and inflammation of the gallbladder. For unknown reasons, children with Kawasaki disease can be very irritable.

What is the treatment?

All children with Kawasaki disease need to be admitted to the hospital. If the disease is diagnosed while the child still has a fever, the complications of Kawasaki disease can usually be prevented. First, your child will get gamma globulins through a vein (IV). This treatment greatly reduces the risk of heart problems, especially coronary artery aneurysms.

If your child develops a coronary artery aneurysm, he will need to start some long-term treatments including taking aspirin to prevent clotting.

Your child will need to be seen regularly by a pediatric heart specialist (cardiologist).

Call Your Child's Physician Immediately If:

  • You think your child has Kawasaki disease and he is acting very sick.
  • Your child was recently diagnosed with Kawasaki disease (1 to 2 months) and is having chest pain.

Call Your Child's Physician during Office Hours If:

  • You would like more information about Kawasaki disease.

Written by Robert Brayden, MD, The Children's Hospital, Denver.
Copyright 1999 Clinical Reference Systems