Kawasaki Disease

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Kawasaki Disease
(Kawasaki Syndrome, Mucocutaneous Lymph Node Syndrome)
Pronounced: Kah-wah-sock-ee
Definition
Kawasaki disease is a type of inflammatory disease that usually only affects children. Typical symptoms include high fevers, swollen lips and throat, swollen lymph nodes, and peeling hands and feet.
Usually, it's a self-limited condition that has a mild and uncomplicated course, and children often recover without treatment. However, more serious cases can lead to complications that affect the coronary arteries. The coronary arteries are blood vessels that supply the heart with blood.
If these coronary arteries become inflamed, the wall of the arteries may weaken. This weakening can cause a focal dilatation of a blood vessel, which is called an aneurysm . An aneurysm can lead to blockage of the artery. If these blockages occur, the heart, which is a muscle, will suffer from insufficient oxygen. This can cause chest pain ( angina ). It can even cause a heart attack and permanently damage the heart. Early treatment can help prevent these aneurysms from developing.
This is a potentially serious condition that requires care from your doctor. The sooner Kawasaki disease is treated, the more favorable the outcome. If you suspect your child has this condition, contact your doctor immediately.
Coronary Artery

Causes
The cause of Kawasaki disease is unknown. It does not seem to be contagious or hereditary.
Risk Factors
A risk factor is something that increases your chance of getting a disease or condition. The following factors increase your chance of developing Kawasaki disease:
• Age: children under age five
o Children over the age of eight rarely get this disease and it is very rare in adults.
o The average age of patients is two years old.
• Sex: more common in boys than girls
• Ethnicity: Asian ethnicity
• Season: more common during the winter and early spring months
Symptoms
If your child experiences any of these symptoms, do not assume it is due to Kawasaki disease. These symptoms may be caused by other, less serious health conditions.
Symptoms include:
• High fever – lasting for at least five days
• Rash
• Swollen hands and feet that may look red and the skin may peel
• Conjunctivitis (also known as “pink eye”) – red or “bloodshot” whites of the eye
• Swollen lymph nodes (organs of the immune system) in the neck
• Soreness and swelling of the mouth, lips, and throat
• Strawberry tongue – tongue has some white/yellow coating and bright red bumps
• Irritability
Diagnosis
There is no specific test to diagnose Kawasaki disease. Your doctor will ask about your child’s symptoms and medical history, and perform a physical exam.
Your doctor will be able to diagnose Kawasaki disease using the following diagnostic criteria:
• Fever lasting more than five days
• Four of the following:
o Swelling of the conjunctiva
o Swelling of the lips, tongue, and pharynx
o Skin changes over limbs
o Rash over trunk
o Swollen lymph nodes in neck
Blood and urine tests may be performed to rule out other conditions and to document the presence of anemia and inflammation.
Additional tests will also be ordered be ordered to assess for potential involvement of the heart and coronary arteries:
• Electrocardiogram (ECG) – This test that measures the heartbeat's electrical activity. If Kawaski disease is suspected, an ECG is done to exclude a heart attack, a rare complication of this condition in children.
• Echocardiogram – When Kawasaki diseases is suspected or diagnosed, your doctor will take an echocardiogram to find out if coronary arteries are involved. This test should be repeated every year to screen for heart problems. If damage to the heart is found, your child should get an echocardiogram done each year.
Treatment
The goal of treatment is to prevent any damage to the coronary arteries and the heart. It is also important to make your child as comfortable as possible as the illness runs its course. The earlier treatment is started the better. Early treatment can prevent long-term heart and joint problems. Treatment is usually given in the hospital and a pediatric cardiologist may come to examine your child.
Talk with your doctor about the best treatment plan for your child. Treatment options include the following:
Aspirin
High doses of aspirin are given to:
• Prevent blood clots from forming
• Reduce your child’s fever
• Ease joint inflammation
• Treat rashes
It is important to note that if your child is given aspirin therapy and develops signs/symptoms of a viral infection, especially chickenpox , the physician should be consulted about discontinuing aspirin therapy. Aspirin has been associated with the development of Reye’s syndrome , a potentially fatal condition.
Intravenous Gamma Globulin
If treatment is given early in the illness, your doctor may administer this protein found in the blood that helps fight infection and lessens the risk of developing problems with the coronary arteries.
Plasmapheresis (Therapeutic Plasma Exchange)
This is a process in which the fluid part of the blood, called plasma, is removed from blood cells by a device known as a cell separator. With Kawasaki disease, it is only used in rare and selected cases.
If Kawasaki disease leads to complications (eg, heart failure ), they will need to be treated accordingly.
Prevention
There is no known way to prevent Kawasaki disease but it is important to treat it as early as possible to prevent damage to the coronary arteries and the heart. Twenty percent to 25% of children who do not receive treatment get damage.