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Kawasaki Disease

Kawasaki disease, also called mucocutaneous lymph node syndrome, is a disease that affects children. Most children who have it are under the age of five. The average age of onset is two-years-old. Boys are almost twice as likely as girls are to develop the disease. It is the leading cause of heart disease in children, but if it's found early, it's usually entirely treatable. Most children who receive treatment will recover fully. 

Kawasaki disease causes inflammation of the blood vessels, which can damage the coronary arteries. It also causes problems with the lymph nodes, skin, mouth, nose, and throat. 

About one-quarter of children who have Kawasaki disease may experience heart effects. It causes inflammation of and damage to the coronary arteries and the heart itself. When coronary arteries are weakened, an aneurysm may occur, which can be deadly. Infants are at greatest risk for heart complications, and they tend to become more seriously ill than older children who develop the disease. 

Causes and risks of Kawasaki disease

Experts aren’t sure what causes Kawasaki disease, but the theory is that it’s a combination of genetics, exposure to viruses or bacteria, and other factors in the environment. While doctors don’t think Kawasaki disease is contagious, it does tend to occur in outbreaks within a community, which leads them to believe there is a viral or bacterial component to it.

Signs and symptoms of Kawasaki disease

Kawasaki disease tends to have a very quick onset, but its symptoms occur in three phases – acute, subacute, and convalescent. Children may experience heart trouble in as soon as 10 days after symptoms start, so it’s important to seek treatment right away.

Symptoms include:

  • Fever above 101F that doesn’t respond well to fever reducing medications and lasts more than five days
  • A rash and/or peeling skin that tends to show up between the chest and legs, in the groin area, and (later) on the fingers and toes
  • Swelling and redness of the hands and the soles of the feet that is followed by skin peeling
  • Eye redness
  • Enlarged lymph nodes in the neck and elsewhere
  • Heart rhythm irregularities
  • Irritation in the mouth, lips, and throat
  • Swollen, bright red tongue
  • Diarrhea, stomach pain, and vomiting
  • Joint pain
  • Irritability
  • Weakness in the arms, legs, or face muscles

Phases of Kawasaki disease

  • Acute phase – The acute phase is the part of the illness when symptoms appear and are at their worst. It typically lasts between 10 and 14 days, but it can last longer in some children. 
  • Subacute phase – During the second phase, the subacute phase, the fever, rash, and lymph node swelling go away, but other symptoms may remain. Some of the symptoms associated with this phase are irritability, poor appetite, some eye redness, and peeling skin on the hands and feet. It usually ends about three or four weeks after the onset of fever. 
  • Convalescent stage – This is the third stage. During the convalescent stage, all symptoms are gone, but a blood test shows there is still abnormal inflammation in the body. This stage usually lasts for six to eight weeks after the fever began. 

Diagnosis and treatment of Kawasaki disease

If you suspect your child has Kawasaki disease – or he or she has had a fever for more than four days along with other symptoms of the disease – call the doctor right away. 

Kawasaki disease is rare in the United States, so your child’s doctor will likely try to rule out other conditions, such as scarlet fever, measles, or Rocky Mountain spotted fever, before making a diagnosis of Kawasaki disease. 

Unfortunately, there is not yet a reliable diagnostic test for Kawasaki disease, so your child’s doctor will evaluate symptoms and rule out more common conditions with similar symptoms before making a diagnosis. 

If your child’s doctor believes Kawasaki disease is a possibility, he or she will likely schedule an echocardiogram to look at the structure of your child’s heart. This test lets the doctor check for blood vessel abnormalities like dilation or aneurysm in the coronary arteries. The doctor may also recommend a coronary angiography to get a better picture of the blood vessels in the heart.

Treatment for Kawasaki disease

Treatment for Kawasaki disease focuses on helping your child feel better and preventing any serious or long-term complications, such as blood clots or heart disease. Your child may need to see a specialist, such as a pediatric cardiologist, for treatment, and he or she will have to be treated in a children’s hospital for at least the first few days.

The first line of treatment is a single dose of gamma globulin, which is given directly into a vein, along with a dose of aspirin. While aspirin is normally not used in children due to the risk of Reye’s syndrome, it is an important part of treatment for Kawasaki disease, so the benefits of giving aspirin in this case outweigh the risks. 

Gamma globulin is a combination of proteins and antibodies collected from donated blood products. It can be given every day for several days if the fever continues. Doctors aren’t sure why gamma globulin works, but they do know that the sooner it is started, the more effective it is at treating Kawasaki disease. Complications, such as aneurysms in the coronary arteries, can often be prevented with early treatment, but if treatment doesn’t start until 10 days into the illness, it may not prevent complications.

Aspirin therapy should continue for six to eight weeks and should not be stopped until after symptoms of the acute phase have disappeared. 
Doctors may also use corticosteroids or immunosuppressive medication if gamma globulin is not effective. For cases of Kawasaki disease that don’t respond to any of the standard treatment, doctors may recommend plasmapheresis, which involves removing blood from the body, filtering it to remove proteins and antibodies, and returning it to the body. 

If your child has a severe case of Kawasaki disease, he or she may need to have surgery to correct complications, like heart problems.
After your child recovers, you will need to have follow up visits with his or her pediatrician and a pediatric cardiologist to ensure his or her heart is working well. Your child may need to have x-rays, echocardiograms, electrocardiograms, and other tests to make sure he or she has fully recovered and doesn’t need further treatment.

For a referral to a Beaumont pediatric cardiologist, call 855-480-KIDS (855-480-5437) or find one online.