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Kawasaki diseaseURL of this page: http://www.nlm.nih.gov/medlineplus/ency/article/000989.htm

Kawasaki disease is a rare condition in children that involves inflammation of the blood vessels.

Causes

Kawasaki disease occurs most frequently in Japan, where the disease was first discovered. In the United States, aftercongenital heart defects, Kawasaki disease is the leading cause of heart disease in children. Most of these patients are

younger than age 5. The disease occurs more often in boys than in girls.

Kawasaki disease is a poorly understood illness. The cause has not been determined. It may be an autoimmunedisorder. 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 arteries, especially the coronary arteries. This

inflammation can lead to aneurysms. An aneurysm can lead to a heart attack, even in young children, although this israre.

Symptoms

Kawasaki disease often begins with a high and persistent fever greater than 102F, often as high as 104F. A persistent

fever lasting at least 5 days is considered a classic sign. The fever may last for up to 2 weeks and does not usually goaway 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 lipsRed mucous membranes in the mouthStrawberry 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 feetSwollen hands and feet

Skin rashes on the middle of the body, NOT blister-likePeeling 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 areaJoint pain and swelling, frequently one both sides of the body

Additional symptoms may include:

IrritabilityDiarrhea, vomiting, and abdominal painCough and runny nose

Exams and Tests

No tests 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 not all of the classic symptoms of the disease.These children may be diagnosed with atypical Kawasaki 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 whodo have the disease.

asaki disease: MedlinePlus Medical Encyclopedia (Print Version) http://www.nlm.nih.gov/medlineplus/print/ency/article/00

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The following tests may be performed:

Chest x-rayComplete blood count

C-reactive protein (CRP)Echocardiogram

ElectrocardiogramESRSerum albumin

Serum transaminaseUrinalysis - may show pus in the urine or protein in the urine

Procedures such as ECG and echocardiography may reveal signs of myocarditis, pericarditis, arthritis, asepticmeningitis, and inflammation of the coronary arteries.

Treatment

Children with Kawasaki disease are admitted to the hospital. Treatment must be started as soon as the diagnosis ismade 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.

Even when they're treated with aspirin and IVIG, up to 25% of children may still develop problems in their coronaryarteries. Some research has suggested that adding steroids to the usual treatment routine may improve a child's

outcome, but more research is needed.

Outlook (Prognosis)

With early recognition and treatment, full recovery can be expected. However, about 1% of patients die fromcomplications of coronary blood vessel inflammation. Patients who have had Kawasaki disease should have an

echocardiogram every 1 - 2 years to screen for heart problems.

Possible Complications

Complications involving the heart, including vessel inflammation and aneurysm, can cause a heart attack at a young ageor later in life.

When to Contact a Medical Professional

Call your health care provider if symptoms of Kawasaki disease develop. A persistent high fever that does not comedown with acetaminophen or ibuprofen and lasts more than 24 hours should be evaluated by a health care provider.

Prevention

There are no known measures that will prevent this disorder.

Alternative Names

Mucocutaneous lymph node syndrome; Infantile polyarteritis

References

Sakata K, Hamaoka K, Ozawa S, et al. A randomized prospective study on the use of 2 g-IVIG or 1 g-IVIG as therapyfor Kawasaki disease. Eur J Pediatr. 2007 Jun;166(6):565-71.

Newburger JW, Sleeper LA, McCrindle BW, et al. Randomized trial of pulsed corticosteroid therapy for primary

treatment of Kawasaki disease. N Engl J Med. 2007 Feb 15;356(7):663-75.

Zipes DP, Libby P, Bonow RO, Braunwald E, eds. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine,

8th ed. St. Louis, Mo; WB Saunders; 2007.


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Update Date: 12/10/2007

Updated by: David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.; and Mark A Fogel, MD, FACC, FAAP, Associate

Professor of Pediatrics and Radiology, Director of Cardiac MR, The Children's Hospital of Philadelphia.

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policy, editorial process and privacy policy. A.D.A.M. is also a founding member of Hi-Ethics and subscribes to the principles of theHealth on the Net Foundation (www.hon.ch).

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensedphysician should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites areprovided for information only -- they do not constitute endorsements of those other sites. Copyright 1997-2009, A.D.A.M., Inc. Any duplication ordistribution of the information contained herein is strictly prohibited.


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kawasaki disease_ medlinepl

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