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Kawasaki syndrome

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

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Incidence, causes, and risk factors

Kawasaki disease occurs mainly in Japan, though its incidence in the United States is 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 etiological theories center on immunological causes for the disease. Much research is being performed to discover a definitive toxin or antigenic substance, possibly a superantigen, that is the specific cause of the disease.

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

Kawasaki disease often begins with a high and persistent fever that is not very responsive to normal doses of acetaminophen or ibuprofen. The fever may persist steadily for up to two weeks. The children develop red eyes, red mucous membranes in the mouth, red cracked lips, a "strawberry tongue" and swollen lymph nodes. Skin rashes may 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 bloodshot or red eyes (conjunctivitis 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 (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 ECG and echocardiography may reveal signs of myocarditis, pericarditis, arthritis, aseptic meningitis, and coronary vasculitis.

Read more at Wikipedia.org


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… And a disease seeking its raison d'etre - Kawasaki syndrome
From Science News, 10/17/87 by Diane D. Edwards

. . . and a disease seeking its raison d'etre

Since it was first reported in a Japanese journal in 1967, a relatively rare childhood disorder called Kawasaki syndrome has stubbornly rebuffed scientists' efforts to understand it. Recently completed studies indicate that the disease's incidence may be increasing in the United States and that it may be caused by a virus from the same group as that causing AIDS. But the lack of consensus among scientists on these and other aspects of the disease shows that Kawasaki syndrome still retains its secrets.

Characterized by fever, rash and occasional damage to coronary arteries, Kawasaki syndrome is thought to be an infectious disease--given its cyclic epidemics that vary with the seasons (SN: 7/6/85, p.10). Researchers have studied a series of possible disease agents, including house dust mites scattered in the air during the shampooing of rugs. But no cause has been unequivocally identified.

At last week's Interscience Conference on Antimicrobial Agents and Chemotherapy in New York City, Jane Burns of Children's Hospital in Boston called Kawasaki syndrome "a disease in search of a virus.'

Burns says her "very preliminary' studies suggest that a virus producing the enzyme reverse transcriptase may be responsible. About 70 percent of 33 Kawasaki patients tested showed an elevated level of enzyme activity typical of reverse transcriptase. Viruses making this enzyme are broadly classified as retroviruses, a group that includes the AIDS virus.

But Marian Melish of the University of Hawaii in Honolulu says that the enzyme activity seen by Burns "probably came from the patients' cells' and that her own studies do not support a human retrovirus as the cause. Melish, who was one of the first to describe Kawasaki syndrome in the United States, also reports that the incidence of the disease in Hawaii has stabilized in the past few years, yet continues to affect primarily those of Asian ancestry.

Some scientists think Kawasaki syndrome is becoming more common in the rest of the United States, where clinicians are trying to measure its possible long-term effects. Although the fever and skin rash of Kawasaki syndrome disappear and most patients recover, there are structural changes in the blood vessels of the heart in about 20 percent of patients--most of whom are less than 5 years old.

"Kawasaki syndrome is now considered the leading cause of acquired heart disease in [U.S.] children,' Stan Shulman of Children's Memorial Hospital in Chicago said at last week's meeting. "We believe the incidence has clearly increased since 1983.' He estimates that 2,000 to 2,500 U.S. cases now occur during an average year. Underreporting of the disease by physicians, however, makes exact numbers impossible to determine, he says.

COPYRIGHT 1987 Science Service, Inc.
COPYRIGHT 2004 Gale Group

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