What Is Kawasaki Disease?
Kawasaki (KAH-wah-SAH-ke) disease is a rare
childhood disease. It's a form of a disease called
vasculitis
(vas-kyu-LI-tis).
In Kawasaki disease, the walls of the blood vessels
throughout the body become inflamed. The disease can affect any type of blood
vessel in the body, including the arteries, veins, and capillaries.
In some cases, Kawasaki disease affects the coronary
arteries, which carry oxygen-rich blood to the heart. As a result, a small
number of children who have Kawasaki disease may develop serious heart
problems.
Overview
The cause of Kawasaki disease isn't known. The
body's response to a virus or infection combined with genetic factors may cause
the disease. However, no specific virus or infection has been found, and the
role of genetics isn't known.
The disease can't be passed from one child to
another. Your child won't get it from close contact with a child who has the
disease. Also, if your child has the disease, he or she can't pass it on to
another child.
Kawasaki disease affects children of all races and
both genders. It occurs most often in children of Asian and Pacific Island
descent. It's more likely to affect males, and most cases occur in children
younger than 5 years.
One of the main symptoms of this disease is a fever
that lasts longer than 5 days. The fever remains high even after treatment with
standard childhood fever medicines. Children with the disease also may have red
eyes, red lips, and redness on the palms of their hands and soles of their
feet. These are all signs of inflamed blood vessels.
Early treatment is important. It helps reduce the
risk that Kawasaki disease will affect the coronary arteries and cause more
serious problems.
Outlook
Kawasaki disease can't be prevented. However, most
children who develop Kawasaki disease fully recoverusually within weeks
of getting symptoms. Further problems are rare.
In some children, the disease affects the coronary
arteries. This can cause serious problems. These children need long-term care
and treatment.
Researchers continue to look for the cause of
Kawasaki disease and better ways to diagnose and treat it. They also hope to
learn more about long-term health risks, if any, for people who have had the
disease.
Other Names for Kawasaki Disease
- Kawasaki syndrome
- Mucocutaneous lymph node syndrome
What Causes Kawasaki Disease?
The cause of Kawasaki disease isn't known. The
body's response to a virus or infection combined with genetic factors may cause
the disease. However, no specific virus or infection has been found, and the
role of genetics isn't known.
Kawasaki disease can't be passed from one child to
another. Your child won't get it from close contact with a child who has the
disease. Also, if your child has the disease, he or she can't pass it on to
another child.
Who Is At Risk for Kawasaki Disease?
Kawasaki disease affects children of all races and
ages and both genders. It occurs most often in children of Asian and Pacific
Island descent.
The disease is about 1.5 times more likely to affect
boys than girls. Most cases occur in children younger than 5 years, and it's
most common in children younger than 3 years. It's very rare in children older
than 8 years.
What Are the Signs and Symptoms of Kawasaki
Disease?
Major Signs and Symptoms
One of the main symptoms during the early part of
Kawasaki disease, called the acute phase, is fever. The fever lasts longer than
5 days. It remains high even after treatment with standard childhood fever
medicines.
Other classic signs of the disease are:
- Swollen lymph nodes in the neck
- A rash on the mid-section of the body and in the
genital area
- Red, dry, cracked lips and a red, swollen
tongue
- Red, swollen palms of the hands and soles of the
feet
- Redness of the eyes
Other Signs and Symptoms
During the acute phase, your child also may be
irritable and have a sore throat, joint pain, diarrhea, vomiting, and stomach
pain.
Within 2 to 3 weeks of the start of the first
symptoms, the skin of your child's fingers and toes may peel, sometimes in
large sheets.
How Is Kawasaki Disease Diagnosed?
Kawasaki disease is diagnosed based on your child's
signs and symptoms and the results from diagnostic tests and procedures.
Specialists Involved
Pediatricians often are the first to suspect a child
has Kawasaki disease. Pediatricians are doctors who treat children.
If the disease has affected your child's coronary
arteries, a pediatric cardiologist will confirm the diagnosis and give ongoing
treatment. Pediatric cardiologists treat children who have heart problems.
Other specialists also may be involved in treating
children who have Kawasaki disease.
Signs and Symptoms
The doctor will check your child for the classic
signs and symptoms of Kawasaki disease.
The doctor will rule out other diseases that cause
similar symptoms. These diseases include Rocky Mountain spotted fever, scarlet
fever, and juvenile rheumatoid arthritis.
Generally, your child will be diagnosed with
Kawasaki disease if he or she has a fever that lasts longer than 5 days plus
four other classic signs or symptoms of the disease.
However, not all children have classic signs and
symptoms of Kawasaki disease. Diagnostic tests and procedures can help confirm
whether a child has the disease.
Diagnostic Tests and Procedures
Echocardiography
All children suspected of having Kawasaki disease
have a test called
echocardiography
(EK-o-kar-de-OG-ra-fee). This painless test uses sound waves to create images
of the heart. These pictures show the coronary arteries.
Doctors use the test when they suspect a child has
the disease, but the child has fewer than four of the five classic signs.
Echocardiography also can help show the disease's
effects over time, if any, on your child's coronary arteries. Often, the
disease's effects on the coronary arteries don't show up until the second or
third week after the first symptoms appear. Therefore, this test is done
regularly after the diagnosis.
Some children who don't have the classic signs and
symptoms of the disease's acute phase may not be diagnosed until 2 to 3 weeks
after the onset of the disease. This is when another common sign of Kawasaki
disease occurspeeling of the skin on the fingers and toes.
If your child is diagnosed at this point, he or she
will likely need echocardiography right away to see whether the disease has
affected the coronary arteries.
Other Diagnostic Tests
Doctors also use other tests to help diagnose
Kawasaki disease. These include:
- Blood tests. These tests can show whether the
body's blood vessels are inflamed.
- Chest x ray. This test can help show whether
Kawasaki disease has affected the heart.
- EKG
(electrocardiogram). This simple test detects and records the heart's
electrical activity. EKG can show whether Kawasaki disease has affected the
heart.
How Is Kawasaki Disease Treated?
Kawasaki disease is mainly treated with medicines.
Rarely, medical procedures and surgery may be used for children whose coronary
arteries are affected.
The goals of treatment are to:
- Lower fever and reduce inflammation to improve
symptoms
- Prevent the disease from affecting the coronary
arteries
Initial Treatment
Kawasaki disease can cause serious complications.
Therefore, your child will likely be treated in a hospital, at least for the
early part of the treatment.
The standard treatment during the disease's acute
phase is high-dose aspirin and immune globulin. Immune globulin is a medicine
given intravenously (injected into a vein).
Most children who receive these treatments improve
greatly within 24 hours. For a small number of children, fever remains. In
these cases, a second round of immune globulin may be given.
At the start of treatment, high doses of aspirin are
given. As soon as your child's fever goes away, a low dose of aspirin is given.
The low dose helps prevent blood clots, which can form in the inflamed small
arteries.
Most children treated for Kawasaki disease fully
recover from the acute phase and don't need any further treatment. They should,
however, follow a healthy diet and adopt healthy lifestyle habits to lower
their risk for future heart disease (this is advised for all children, not just
those who have the disease).
Children who have had immune globulin should wait 11
months before having the measles and chicken pox vaccines. Immune globulin can
prevent those vaccines from working properly.
Long-Term Care and Treatment
If Kawasaki disease has affected your child's
coronary arteries, he or she will need ongoing care and treatment. It's best if
a pediatric cardiologist provides this care to reduce the risk of severe heart
problems. A pediatric cardiologist is a doctor who treats children who have
heart problems.
Medicines and Tests
When Kawasaki disease affects the coronary arteries,
they may expand and twist. If this happens, your child's doctor may prescribe
anticoagulants (like warfarin) to prevent blood clots from forming in the
affected coronary arteries.
Anticoagulants usually are stopped after the
coronary arteries heal. Healing may occur about 18 months after the acute phase
of the disease.
For the small number of children whose coronary
arteries don't heal, routine tests are needed. These include:
- Echocardiography.
This test uses sound waves to create images of the heart.
- EKG
(electrocardiogram). This test detects and records the heart's electrical
activity.
- Stress
test. This test gives the doctor information about how the heart works
during physical activity or stress.
Medical Procedures and Surgery
Rarely, a child may need
cardiac
catheterization (KATH-e-ter-i-ZA-shun). This procedure is used to diagnose
and treat some heart conditions. A long, thin, flexible tube called a catheter
is put into a blood vessel in the arm, groin (upper thigh), or neck and
threaded to the heart. Through the catheter, doctors can perform diagnostic
tests and treatments on the heart.
Very rarely, a child may need to have other
procedures, or even surgery, if inflammation narrows his or her coronary
arteries and blocks blood flow to the heart.
Coronary
angioplasty (AN-jee-oh-plas-tee),
stent
placement, or
coronary
artery bypass grafting (CABG) may be used.
Coronary angioplasty restores blood flow through a
narrowed or blocked coronary artery. A thin tube with a balloon on the end is
threaded through a blood vessel in the arm or groin (upper thigh) to the
narrowed or blocked coronary artery. The balloon is then inflated to widen the
artery and restore blood flow.
A stent may be placed in the coronary artery during
angioplasty. Stents are small mesh tubes. They're used to keep narrowed or
weakened arteries open. Stents can help improve blood flow and prevent the
artery from bursting.
Rarely, a child may need to have CABG. This surgery
is used when a coronary artery is severely blocked. During CABG, a healthy
artery or vein from another part of the body is connected, or grafted, to the
blocked coronary artery. The grafted artery or vein bypasses (that is, it goes
around) the blocked part of the coronary artery. This improves blood flow to
the heart.
How Can Kawasaki Disease Be Prevented?
Kawasaki disease can't be prevented. However, most
children who develop the disease fully recoverusually within weeks of
getting signs and symptoms. Further problems are rare.
Living With Kawasaki Disease
Most children who develop Kawasaki disease fully
recoverusually within weeks of getting symptoms. Further problems are
rare.
However, early treatment is important to reduce the
risk of serious problems.
Researchers continue to look for the cause of
Kawasaki disease and better ways to diagnose and treat it. They also hope to
learn more about long-term health risks, if any, for people who have had the
disease.
What To Expect After Treatment
Most children treated for Kawasaki disease fully
recover from the acute phase. They don't need further treatment. They should,
however, follow a healthy diet and adopt healthy lifestyle habits to reduce the
risk for future heart disease (this is advised for all children, not just those
who have the disease).
Children treated with immune globulin should wait 11
months before having measles and chicken pox vaccines. Immune globulin can
prevent these vaccines from working properly.
Ongoing Health Care Needs
If Kawasaki disease has affected your child's
coronary arteries, he or she will need ongoing care and treatment. It's best if
a pediatric cardiologist provides this care to reduce the risk of severe heart
problems. A pediatric cardiologist is a doctor who treats children who have
heart problems.
Support Group
For more information on Kawasaki disease, go to the
Kawasaki Disease Foundation Web site. This site provides
information about Kawasaki disease, access to volunteers who help families cope
with Kawasaki disease, and a link to an online support group for parents and
others caring for children with the disease.
Key Points
- Kawasaki disease is a rare childhood disease. In
Kawasaki disease, the walls of the blood vessels throughout the body become
inflamed. This disease can affect any type of blood vessel in the body,
including the arteries, veins, and capillaries.
- The cause of Kawasaki disease isn't known. The
body's response to a virus or infection combined with genetic factors may cause
the disease. However, no specific virus or infection has been found, and the
role of genetics isn't known.
- Kawasaki disease can't be passed from one child
to another.
- Kawasaki disease affects children of all races
and ages and both genders. It occurs most often in children of Asian and
Pacific Island descent. It's more likely to affect males, and most cases occur
in children younger than 5 years.
- One of the main symptoms during the early part
(acute phase) of Kawasaki disease is fever. The fever lasts longer than 5 days.
It remains high even after treatment with standard childhood fever medicines.
- Other classic signs and symptoms are swollen
lymph nodes in the neck; a rash on the mid-section and genital area; red, dry,
cracked lips and a red, swollen tongue; red, swollen palms of the hands and
soles of the feet; and redness of the eyes.
- During the acute phase of Kawasaki disease, your
child also may be irritable and have a sore throat, joint pain, diarrhea,
vomiting, and stomach pain. Within 2 to 3 weeks of the start of symptoms, the
skin on your child's fingers and toes may peel, sometimes in large sheets.
- Kawasaki disease is diagnosed based on your
child's signs and symptoms and the results from diagnostic tests and
procedures. Generally, your child will be diagnosed with Kawasaki disease if he
or she has a fever lasting longer than 5 days and four other classic signs of
the disease. Because not all children have classic signs of the disease,
diagnostic tests and procedures can help confirm a diagnosis.
- Kawasaki disease is mainly treated with
medicines. Rarely, medical procedures and surgery also may be used for children
whose coronary arteries are affected.
- Kawasaki disease can't be prevented. However,
most children who develop the disease fully recoverusually within weeks
of getting signs and symptoms. Further problems are rare.
- If Kawasaki disease has affected your child's
coronary arteries, he or she will need ongoing treatment.
- Researchers continue to look for the cause of
Kawasaki disease and better ways to diagnose and treat it. They also hope to
learn more about long-term health risks, if any, for people who have the
disease.
Links to Other Information About Kawasaki Disease
NHLBI Resources
Non-NHLBI Resources
Clinical Trials
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