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Kawasaki Disease |
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Kawasaki disease
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Lymphatic system
Kawasaki's disease, peeling of the fingertips
Kawasaki's disease - edema of the hand
Alternative names Return to top
Mucocutaneous lymph node syndrome; Infantile polyarteritis
Definition Return to top
Kawasaki disease is a rare condition in children that involves
inflammation of the blood vessels.
Causes, incidence, and risk factors Return to top
Kawasaki disease occurs most frequently in Japan, where the disease
was first discovered. In the United States, Kawasaki disease is the
leading cause of acquired heart disease in children. It affects more
than 4,000 children each year. Eighty percent of patients are
younger than age 5.
Kawasaki disease is a poorly understood illness. The cause has not
been determined. It may be an autoimmune disorder. The disorder
affects the mucus membranes, lymph nodes, walls of the blood
vessels, and the heart.
Kawasaki disease can cause inflammation of blood vessels in the
coronary arteries, which can lead to aneurysms. An aneurysm can lead
to a heart attack, even in young children, although this is rare.
Symptoms Return to top
Kawasaki disease often begins with a high and persistent fever
greater than 102°F, often as high as 104°F. A persistent fever
lasting at least 5 days is considered a classic sign. The fever may
last for up to 2weeks and does not usually go away with normal doses
of acetaminophen (Tylenol) or ibuprofen.
Other symptoms often include:
Extremely bloodshot or red eyes (without pus or drainage)
Bright red, chapped, or cracked lips
Red mucous membranes in the mouth
Strawberry tongue, white coating on the tongue, or prominent red
bumps on the back of the tongue
Red palms of the hands and the soles of the feet
Swollen hands and feet
Skin rashes on the middle of the body, NOT blister-like
Peeling skin in the genital area, hands, and feet (especially around
the nails, palms, and soles)
Swollen lymph nodes (frequently only one lymph node is swollen),
particularly in the neck area
Joint pain and swelling, frequently one both sides of the body
Signs and tests Return to top
There are no tests that specifically diagnose Kawasaki disease. The
diagnosis is usually made based on the patient having most of the
classic symptoms.
However, some children may have a fever lasting more than 5 days,
but few of the other classic symptoms of the disease. Therefore, all
children with fever lasting more than 5 days should be evaluated,
with Kawasaki disease considered as a possibility. Early treatment
is essential for those who do have the disease.
The following tests may be performed:
Chest x-ray
Complete blood count
C-reactive protein (CRP)
Echocardiogram
Electrocardiogram
ESR
Serum albumin
Serum transaminase
Urinalysis - may show pus in the urine or protein in the urine
Procedures such as ECG and echocardiography may reveal signs of
myocarditis, pericarditis, arthritis, aseptic meningitis, and
inflammation of the coronary arteries.
Treatment Return to top
Children with Kawasaki disease are admitted to the hospital.
Treatment must be started as soon as the diagnosis is made to
prevent damage to the coronary arteries and heart.
Intravenous gamma globulin is the standard treatment. It is given in
high doses. The child's condition usually greatly improves within 24
hours of treatment with IV gamma globulin.
High-dose aspirin is often given along with IV gamma globulin.
Despite aspirin and IVIG treatment, 10-15% of children still develop
problems in their coronary arteries. Some research has suggested
that adding steroids to the usual treatment routine may improve a
child's outcome, but more research is needed.
www.cidpusa.org www.cidpusa.org/P/ivig.htm http://www.cidpusa.org/disease.html http://www.cidpusa.org/Lahore.html
Info from the NIH in USA http://www.cidpusa.org/FIBROMYALGIA1.html