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1994-01-17
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$Unique_ID{BRK03894}
$Pretitle{}
$Title{Kawasaki Disease}
$Subject{Kawasaki Disease Mucocutaneous Lymph Node Syndrome MLNS MLN Measles
Streptococcal Infections}
$Volume{}
$Log{}
Copyright (C) 1986, 1990, 1991 National Organization for Rare Disorders,
Inc.
186:
Kawasaki Disease
** IMPORTANT **
It is possible the main title of the article (Kawasaki Disease) is not
the name you expected. Please check the SYNONYMS listing to find the
alternate names and disorders subdivisions covered by this article.
Synonyms
Mucocutaneous Lymph Node Syndrome, also known as MLNS and MLN
Information on the following Diseases can be found in the Related
Disorders Section of this report.
Measles
Streptococcal Infections
General Discussion
** REMINDER **
The information contained in the Rare Disease Database is provided for
educational purposes only. It should not be used for diagnostic or treatment
purposes. If you wish to obtain more information about this disorder, please
contact your personal physician and/or the agencies listed in the "Resources"
section of this report.
Kawasaki disease is characterized by diseased lymph nodes in the neck
with a high spiking fever and a rash, primarily over the trunk.
Polyarteritis (multiple sites of inflammatory and destructive lesions in the
arterial system) also occurs, especially in large and medium sized arteries.
The coronary vessels are particularly vulnerable.
Symptoms
Kawasaki Disease is a childhood illness characterized by diseased lymph nodes
in the neck, high fever, sore throat, irritability or fatigue, a bright red
tongue, dry, cracked lips, and a rash primarily over the trunk. This
syndrome predominantly affects people of Japanese ancestry. Multiple sites
of inflammatory and destructive lesions in the arterial system
(polyarteritis) occur, and may involve the coronary vessels in twenty percent
of those affected.
Causes
Recent research suggests that Kawasaki disease may be caused by a retrovirus.
Although they have not yet identified the virus, antibodies to retroviruses
have been found in the white blood cells of Kawasaki patients.
Affected Population
Kawasaki Disease occurs mostly in children of Japanese descent under nine
years of age. An estimated 4,000 Americans will acquire this disorder each
year. There have been rare reports of this disorder in individuals up to
twenty-seven years of age. The male to female ratio is 1.5 to 1, with
serious complications found more often in males. Although the disease was
first recognized as a clinical entity in Japan during 1961, cases have been
reported throughout the world. It is presumed to occur most commonly in the
Japanese, with fifteen cases per 100,000 population reported per year in
children ages four years and under.
It is estimated that twenty percent of affected children develop heart
complications from Kawasaki disease. The death rate in the United States
from this disorder is only one percent of those affected.
Related Disorders
Measles, also known as rubeola, is a very contagious viral disease causing
fever and rash. Measles is usually considered a disease of childhood; it
can, however, affect persons of all ages. Symptoms include fever, white
spots in the eyelids and mouth (Koplik spots), sore throat, cough, red eyes
and runny nose, as well as a red rash. The illness usually lasts two weeks
or more. There is no treatment for measles other than treating the symptoms
of the illness. A vaccine is available for the protection of anyone fifteen
months or older.
Streptococcal infections result from infection with the streptococcus
bacteria. This infection usually results in illnesses such as sore throat,
tonsillitis, impetigo, scarlet fever, chorea, and nephritis. Antibiotics are
used for treatment of these types of infection.
Therapies: Standard
Early in the course of Kawasaki Disease, administration of high dose
intravenous gamma-globulin plus aspirin often reduces the occurrence of
coronary-artery abnormalities. This treatment significantly reduces fever,
and may reduce inflammation of the respiratory tract mucous membranes,
central nervous system, joints and skin. Guarding against infection is
recommended. Further treatment should be symptomatic and supportive.
Gammaglobulin appears to be an effective treatment in many cases.
ECG's should be checked frequently. Some authorities recommend close
follow-up with two-dimensional electrocardiograph and possible coronary
angiography whenever a coronary aneurysm is suspected. Long-term evaluation
of patients with suspected coronary-artery abnormalities is recommended to
hopefully prevent later development of coronary disease.
Aspirin therapy may prevent formation of coronary aneurysms. The
duration of aspirin therapy is guided by the course of the disorder and is
usually continued for several months. If coronary aneurysms develop, aspirin
is often prescribed for a longer period of time to prevent clotting. In some
cases, coronary artery bypass surgery may be beneficial. Corticosteroids are
not recommended as routine therapy in this syndrome.
In 1990, the National Institutes of Health recommended that standard
therapy for Kawasaki Disease should include intravenous immunoglobulin
(gammaglobulin) and aspirin.
Therapies: Investigational
Investigators are trying to determine if a single high dose of intravenous
gamma globulin with aspirin is a more effective initial treatment for
Kawasaki Disease than four lower doses.
Kawasaki Disease is currently being studied by Jane W. Newburger, M.D.,
a senior associate in the Department of Cardiology at Children's Hospital in
Boston, MA.
Fred S. Rosen, M.D. and James L. Gamble, who is a professor of pediatrics
at Harvard Medical School, have been studying this disorder.
This disease entry is based upon medical information available through
October 1991. Since NORD's resources are limited, it is not possible to keep
every entry in the Rare Disease Database completely current and accurate.
Please check with the agencies listed in the Resources section for the most
current information about this disorder.
Resources
For more information on Kawasaki Disease, please contact:
National Organization for Rare Disorders (NORD)
P.O. Box 8923
New Fairfield, CT 06812-1783
(203) 746-6518
NIH/National Institute of Allergy and Infectious Diseases (NIAID)
9000 Rockville Pike
Bethesda, MD 20892
(301) 496-5717
Centers for Disease Control (CDC)
1600 Clifton Road, NE
Atlanta, GA 30333
(404) 639-3900
References
THE MERCK MANUAL, 15th ed., Robert Berkowe, M.D., ed in chief, published by
Merck, Sharp & Dohme Research Labs, Rahway, NJ, 1987. Pp. 2050.
THE CECIL TEXTBOOK OF MEDICINE, 18th Ed.: James B. Wyngaarden and Lloyd H.
Smith, Jr., Eds; W.B. Saunders Co., 1988. 2323-2324, 1523.