Kawasaki disease

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}} Kawasaki disease, also known as mucocutaneous lymph node syndrome, mucocutaneous lymph node disease, infantile polyarteritis and Kawasaki syndrome, is a poorly-understood non-contagious illnessthat affects the mucous membranes, lymph nodes, blood vesselwalls, and the heart. It was first described in 1967by Dr. Tomisaku Kawasaki.

Incidence, causes, and risk factors

Kawasaki disease occurs mainly in Japan, though its incidence in the United Statesis increasing. Kawasaki disease is predominantly a disease of young children, with 80% of patients younger than 5 years of age, but no other contributing factors are known.

The causative agent of Kawasaki disease is still unknown. But current etiologicaltheoriescenter on immunologicalcauses for the disease. Much research is being performed to discover a definitive toxinor antigenicsubstance, possibly a superantigen, that is the specific cause of the disease.

The cardiac involvementand complications are, by far, the most important aspect of the disease. Kawasaki disease can cause vasculitis(inflammation of blood vessels) in the coronary arteriesand subsequent coronary artery aneurysms. These aneurysmscan lead to myocardial infarction(heart attack) even in young children (rarely). About 20–40% of children with Kawasaki disease will have evidence of vasculitiswith cardiac involvement.

Kawasaki disease often begins with a high and persistent feverthat is not very responsive to normal doses of acetaminophenor ibuprofen. The fever may persist steadily for up to two weeks. The children develop red eyes, red mucous membranesin the mouth, red cracked lips, a "strawberry tongue" and swollen lymph nodes. Skin rashesmay occur early in the disease and peeling of the skin in the genital area, hands, and feet (especially around the nails and on the palms and soles) may occur.

Symptoms

• High-grade fever (greater than 39 °C or 102 °F; often as high as 40 °C or 104 °F) that is unresponsive to medication and lasts more than three days (persistent fever lasting at least five days is considered a hallmark sign)
• Extremely bloodshotor red eyes (conjunctivitiswithout pus or drainage)
• Bright red, chapped, or cracked lips
• Red mucous membranesin the mouth
• Strawberry tongue, white coating on the tongue or prominent red bumps (papillae) on the back of the tongue
• Red palms of the hands and the soles of the feet
• Swollen hands and feet
• Peeling palms and soles (later in the illness); peeling may begin around the nails
• Rashes similar to erythema multiforme(rash, NOT blister-like, on the trunk)
• Swollen lymph nodes(frequently only one lymph node is swollen), particularly in the neck area
• Joint pain (arthralgia) and swelling, frequently symmetrical

Signs and tests

A physical examination will demonstrate many of the symptoms listed above. Procedures such as ECGand echocardiographymay reveal signs of myocarditis, pericarditis, arthritis, aseptic meningitis, and coronary vasculitis.

Tests include:

• Angiography
• Complete blood count(CBC)
• Erythrocyte sedimentation rate(ESR)
• Electrocardiogram
• Echocardiogram
• Chest x-ray
• Urinalysis
  • May show pus in the urine (pyuria)
  • May show protein in the urine (proteinuria)

Treatment

Children with Kawasaki disease are hospitalized and care is normally shared between pediatric cardiologyand infectious diseasespecialists, although no infectious agenthas been demonstrated. It is imperative that treatment be started as soon as the diagnosis is made to prevent damage to the coronary arteries, heart, and eyes.

Intravenousgamma globulinis the standard treatment for Kawasaki disease and is administered in high doses. Marked improvement is usually noted within 24 hours of treatment with IVgamma globulin.

Salicylate therapy, particularly aspirin, remains an important part of the treatment but salicylatesalone are not as effective as IVgamma globulin.

Prognosis

With early diagnosisand treatment, full recovery can be expected. However, about 2% of patients die from complications of coronary vasculitis. Some doctors recommend that patients who have had Kawasaki disease should have an echocardiogramevery 1–2 years to screen for progression of cardiac involvement. Treatment is by giving intravenous immunoglobulin.

Complications

Complications involving the heart, including coronary vasculitisand coronary aneurysm, can cause acutemyocardial infarctionlater in life or at a young age.

Reference

• Kawasaki T. Acute febrile mucocutaneous syndrome with lymphoid involvement with specific desquamation of the fingers and toes in children (in Japanese). Arerugi 1967;16:178-222. PMID 6062087.

This complication is extremely rare if treated in time with intravenous immunoglo9bulin

External links

• Kawasaki Disease Research Program
• Kawasaki Disease Foundation
• Kawasaki Disease informationfrom Seattle Children's Hospital Heart Center
• GPnotebook1751842816de:Kawasaki-Syndrom

es:Síndrome de Kawasaki fr:Maladie de Kawasaki nl:Ziekte van Kawasaki ja:???

This article is licensed under the GNU Free Documentation License.
It uses material from the http://en.wikipedia.org/wiki/Kawasaki+disease Wikipedia article Kawasaki disease.