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$Unique_ID{BRK04186}
$Pretitle{}
$Title{Reye Syndrome}
$Subject{Reye Syndrome Hepatic Encephalopathy Liver
Degeneration-Encephalopathy Fatty Liver with Encephalopathy Medium Chain CoA
Dehydrogenase Deficiency}
$Volume{}
$Log{}
Copyright (C) 1986, 1987, 1988, 1991, 1992 National Organization for Rare
Disorders, Inc.
108:
Reye Syndrome
** IMPORTANT **
It is possible the main title of the article (Reye Syndrome) is not the
name you expected. Please check the SYNONYM listing to find the alternate
names and disorder subdivisions covered by this article.
Synonyms
Hepatic Encephalopathy
Liver Degeneration-Encephalopathy
Fatty Liver with Encephalopathy
Disorders section of this report.
Medium Chain CoA Dehydrogenase Deficiency
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.
Reye Syndrome is a combination of acute brain disease (encephalopathy)
and fatty degeneration of the abdominal organs (viscera), which tends to
follow some acute virus infections (such as flu or chicken pox), combined
with certain toxins (usually aspirin). Besides these viruses and toxins,
deficiencies of the enzymes needed in the urea cycle appear to be a
contributing factor to Reye Syndrome.
Symptoms
Vomiting after the onset of a viral illness is usually the first sign of Reye
Syndrome. However, children under the age of 2 years may exhibit diarrhea
and/or hyperventilation instead of vomiting. Next, behaviorial changes such
as listlessness, sleepiness, irritability, aggressive and irrational
behavior, and disorientation may occur. The child may then progress to a
comatose state often within three to five days after the onset of symptoms.
There may also be seizures. A complete recovery is possible. However, brain
damage, ranging from a slight decrease in I.Q. to total paralysis, may occur.
Causes
Reye Syndrome appears to be caused by certain toxins given to individuals
with a deficiency of enzymes needed in the digestive cycle. These enzymes
break down the ammonium from proteins into urea which is excreted in the
urine (urea cycle). This disorder is often associated with the use of
aspirin to treat viral infections. The Centers for Disease Control, the AMA,
and the American Academy of Pediatrics have issued statements warning against
the use of aspirin in children with chickenpox and gastrointestinal flu-like
illness. The FDA has also stated that medications such as tigan, compazine,
thorazine and phenergan as well as other phenothiazines (a broad category of
drugs) used to stop vomiting might possibly contribute to the severity of
Reye Syndrome or mask its early symptoms. Except for aspirin, these drugs
are only available by prescription.
A recent scientific study indicated that ninety percent of children
diagnosed with Reye Syndrome had taken salicylate drugs (such as aspirin)
during the illness preceding onset of Reye's. The FDA considers this to be
firm scientific evidence of the link between Reye Syndrome and aspirin.
In 1990 researchers discovered that a few children who had died of Reye
Syndrome lacked a certain enzyme that is needed to break down short-chain
fatty acids. The beta oxidation defects cause fatty change in the liver
muscle, and swelling of the brain. They suspect that this defect may only
cause symptoms after a long period of fasting (not eating) which triggers low
blood sugar and high concentrations of lactic acid in these children. The
enzyme deficiency is inherited. However, more research is needed to confirm
this theory.
Affected Population
Reye Syndrome occurs most frequently in white suburban children under the age
of 16 years who have recently had viral illnesses such as chickenpox or
influenza. However, it can also occur in newborns, adolescents, and even the
middle-aged. The incidence of Reye Syndrome in teenagers has been rising in
recent years, indicating that they may self-medicate with aspirin.
The incidence of Reye Syndrome seems to be affected by the intensity
and/or type of influenza activity year by year, according to the National
Reye Syndrome Surveillance System. However, in 1984, influenza activity rose
while reported cases of Reye Syndrome in children under ten years of age
decreased. The number of cases in adolescents increased slightly. The
decreased incidence for children under ten was apparent in cases with both
chicken pox (varicella) and respiratory illness.
Related Disorders
Medium Chain CoA Dehydrogenase Deficiency is a very rare metabolic disorder
characterized by a deficiency of the enzyme medium chain CoA dehydrogenase.
This enzyme is needed in the breakdown (metabolism) of fats. Low blood sugar
(hypoglycemia), lack of energy (lethargy) and possibly coma, associated with
fatty changes in the liver, usually occur. During hypoglycemic periods,
tests usually show massive amounts of dicarboxylic acid in the urine. (For
more information on this disorder, choose "Medium Chain CoA" as your search
term in the Rare Disease Database.)
Therapies: Standard
Reye Syndrome is diagnosed by liver function testing and the presenting
symptoms. Immediate supportive treatment and care in an intensive care unit
is vital for recovery since there is no known specific therapy for the
disorder. Permanent neurological damage, such as mental impairment, can be
present after recovery from the acute episode of the disease.
Therapies: Investigational
This disease entry is based upon medical information available through March
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 Reye Syndrome, please contact:
National Organization for Rare Disorders (NORD)
P.O. Box 8923
New Fairfield, CT 06812-1783
(203) 746-6518
National Reye Syndrome Foundation
P.O. Box 829
Bryan, Ohio 43506
(419) 636-2679
NIH/National Institute of Neurological Disorders & Stroke (NINDS)
9000 Rockville Pike
Bethesda, MD 20892
(301) 496-5751
(800) 352-9424
Centers for Disease Control (CDC)
1600 Clifton Road, N.E.
Atlanta, GA 30333
(404) 639-3534
Office of Consumer Affairs (HFE-88)
Food and Drug Administration
5600 Fishers Lane
Rockville, MD 20857
References
Budd, R., "Spotting Reye Syndrome while there's still time", RN,
December, 1983. p. 39-42.