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Reye's Syndrome

Reye's syndrome

Definition

Reye's syndrome is a disorder principally affecting the liver and brain, marked by rapid development of life-threatening neurological symptoms.

Description

Reye's syndrome is an emergency illness chiefly affecting children and teenagers. It almost always follows a viral illness such as a cold, the flu, or chickenpox . Reye's syndrome may affect all the organs of the body, but it most seriously affects the brain and liver. Brain swelling and massive accumulations of fat in the liver and other organs lead to the rapid development of severe neurological symptoms, including lethargy, confusion, seizures, and coma. Reye's syndrome is a life-threatening emergency, with a fatality rate of about 30 to 50 percent.

Demographics

Reye's syndrome is a rare illness, even rarer in the early 2000s than when it was first described in the early 1970s. The incidence of the disorder peaked in 1980, with 555 cases reported. The number of cases declined rapidly thereafter once researchers made the association between aspirin use and the development of Reye's syndrome. Cases dropped precipitously when parents and healthcare providers were clearly warned against using any aspirin-containing products in children. As of 2004, fewer than 20 cases of Reye's syndrome are reported annually. Because of the rarity of Reye's syndrome, it is often misdiagnosed as encephalitis , meningitis , diabetes, or poisoning , and the true incidence may be higher than the number of reported cases indicates.

Causes and symptoms

Reye's syndrome causes fatty accumulation in the organs of the body, especially the liver. In the brain, it causes fluid accumulation (edema), which leads to a rise in pressure in the brain (intracranial pressure). This pressure compresses blood vessels, preventing blood from entering the brain. Untreated, this pressure increase leads to brain damage and death.

Although as of 2004 the cause remains unknown, Reye's syndrome appears to be linked to an abnormality in the energy-converting structures (mitochondria) within the body's cells.

Reye's syndrome usually occurs after a viral illness with fever , most often an upper respiratory tract infection. It is most often associated with use of aspirin during the fever, and for this reason aspirin and aspirin-containing products are not recommended for people under the age of 19 during fever. Although rare, Reye's syndrome may occur without aspirin use and in adults.

After the beginning of recovery from the viral illness, the affected person suddenly becomes more ill again, with the development of persistent vomiting . This may be followed rapidly by quietness, lethargy, agitation or combativeness, seizures, and coma. In infants, diarrhea may be more common than vomiting. Fever is usually absent at this point.

Diagnosis

Reye's syndrome may be suspected in a child who begins vomiting three to six days after a viral illness, followed by an alteration in consciousness. Diagnosis involves blood tests to determine the levels of certain liver enzymes, which are highly elevated in Reye's syndrome. Other blood changes may occur as well, including an increase in the level of ammonia and amino acids, a drop in blood sugar, and an increase in clotting time. A liver biopsy may also be done after clotting abnormalities are corrected with vitamin K or blood products. A lumbar puncture (spinal tap) may be needed to rule out other possible causes, including meningitis or encephalitis.

Treatment

Reye's syndrome is a life-threatening emergency that requires intensive management. The likelihood of recovery is greatest if it is recognized early and treated promptly. Children with Reye's syndrome should be managed in an intensive-care unit.

Treatment in the early stages includes intravenous sugar to return blood sugar levels to normal and plasma transfusion to restore normal clotting time in the blood. Intracranial pressure is monitored and, if elevated, is treated with drugs such as mannitol and barbiturates placing the patient on a ventilator so that hyperventilation can be used.

Prognosis

The mortality rate for Reye's syndrome is between 30 and 50 percent. The likelihood of recovery is increased to 90 percent by early diagnosis and treatment. Almost all children who survive Reye's syndrome recover fully, although recovery may be slow. In some patients, permanent neurologic damage may remain, requiring physical or educational special services and equipment.

Prevention

Because Reye's syndrome is so highly correlated with use of aspirin for fever in young people, avoidance of aspirin use by children is strongly recommended. Aspirin is in many over-the-counter and prescription drugs, including drugs for headache , fever, menstrual cramps, muscle pain , nausea , upset stomach, and arthritis. It may be used in drugs taken orally or by suppository.

Any of the following ingredients indicates that aspirin is present:

  • aspirin
  • acetylsalicylate
  • acetylsalicylic acid
  • salicylic acid
  • salicylate

Teenagers who take their own medications without parental consultation should be warned not to take aspirin-containing drugs.

KEY TERMS

Acetylsalicylic acid Aspirin; an analgesic, antipyretic, and antirheumatic drug prescribed to reduce fever and to relieve pain and inflammation.

Edema The presence of abnormally large amounts of fluid in the intercellular tissue spaces of the body.

Mitochondria Spherical or rod-shaped structures of the cell. Mitochondria contain genetic material (DNA and RNA) and are responsible for converting food to energy.

Resources

BOOKS

Michaels, Marian G. "Reye Syndrome." In Principles and Practice of Pediatric Infectious Diseases, 2nd ed. Edited by Sarah S. Long et al. St. Louis, MO: Elsevier, 2003.

Rudolph, Jeffrey A., and William F. Balistreri. "Reye's Syndrome and the Mitochondrial Hepatophathies." In Nelson Textbook of Pediatrics. Edited by Richard E. Behrman et al. Philadelphia: Saunders, 2004.

ORGANIZATIONS

National Reye's Syndrome Foundation. PO Box 829, Bryan, OH 43506-0829. Web site: <www.reyessyndrome.org>.

Richard Robinson Rosalyn Carson-DeWitt, MD

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"Reye's Syndrome." Gale Encyclopedia of Children's Health: Infancy through Adolescence. . Encyclopedia.com. 22 May. 2017 <http://www.encyclopedia.com>.

"Reye's Syndrome." Gale Encyclopedia of Children's Health: Infancy through Adolescence. . Encyclopedia.com. (May 22, 2017). http://www.encyclopedia.com/medicine/encyclopedias-almanacs-transcripts-and-maps/reyes-syndrome-1

"Reye's Syndrome." Gale Encyclopedia of Children's Health: Infancy through Adolescence. . Retrieved May 22, 2017 from Encyclopedia.com: http://www.encyclopedia.com/medicine/encyclopedias-almanacs-transcripts-and-maps/reyes-syndrome-1

Reye's Syndrome

Reye's Syndrome

Definition

Reye's syndrome is a disorder principally affecting the liver and brain, marked by rapid development of life-threatening neurological symptoms.

Description

Reye's syndrome is an emergency illness chiefly affecting children and teenagers. It almost always follows a viral illness such as a cold, the flu, or chicken pox. Reye's syndrome may affect all the organs of the body, but most seriously affects the brain and liver. Rapid development of severe neurological symptoms, including lethargy, confusion, seizures, and coma, make Reye's syndrome a life-threatening emergency.

Reye's syndrome is a rare illness, even rarer now than when first described in the early 1970s. The incidence of the disorder peaked in 1980, with 555 cases reported. The number of cases declined rapidly thereafter due to decreased use of aspirin compounds for childhood fever, an important risk factor for Reye's syndrome development. Because of its rarity, it is often misdiagnosed as encephalitis, meningitis, diabetes, or poisoning, and the true incidence may be higher than the number of reported cases indicates.

Causes and symptoms

Reye's syndrome causes fatty accumulation in the organs of the body, especially the liver. In the brain, it causes fluid accumulation (edema ), which leads to a rise in intracranial pressure. This pressure squeezes blood vessels, preventing blood from entering the brain. Untreated, this pressure increase leads to brain damage and death.

Although the cause remains unknown, Reye's syndrome appears to be linked to an abnormality in the energy-converting structures (mitochondria) within the body's cells.

Reye's syndrome usually occurs after a viral, fever-causing illness, most often an upper respiratory tract infection. Its cause is unknown. It is most often associated with use of aspirin during the fever, and for this reason aspirin and aspirin-containing products are not recommended for people under the age of 19 during fever. Reye's syndrome may occur without aspirin use, and in adults, although very rarely.

After the beginning of recovery from the viral illness, the affected person suddenly becomes worse, with the development of persistent vomiting. This may be followed rapidly by quietness, lethargy, agitation or combativeness, seizures, and coma. In infants, diarrhea may be more common than vomiting. Fever is usually absent at this point.

Diagnosis

Reye's syndrome may be suspected in a child who begins vomiting three to six days after a viral illness, followed by an alteration in consciousness. Diagnosis involves blood tests to determine the levels of certain liver enzymes, which are highly elevated in Reye's syndrome. Other blood changes may occur as well, including an increase in the level of ammonia and amino acids, a drop in blood sugar, and an increase in clotting time. A liver biopsy may also be done after clotting abnormalities are corrected with vitamin K or blood products. A lumbar puncture (spinal tap) may be needed to rule out other possible causes, including meningitis or encephalitis.

Treatment

Reye's syndrome is a life-threatening emergency that requires intensive management. The likelihood of recovery is greatest if it is recognized early and treated promptly. Children with Reye's syndrome should be managed in an intensive-care unit.

Treatment in the early stages includes intravenous sugar to return levels to normal and plasma transfusion to restore normal clotting time. Intracranial pressure is monitored, and if elevated, is treated with intravenous mannitol and hyperventilation to constrict the blood vessels in the brain. If the pressure remains high, barbiturates may be used.

Prognosis

The mortality rate for Reye's syndrome is between 30-50%. The likelihood of recovery is increased to 90% by early diagnosis and treatment. Almost all children who survive Reye's syndrome recover fully, although recovery may be slow. In some patients, permanent neurologic damage may remain, requiring physical or educational special services and equipment.

Prevention

Because Reye's syndrome is so highly correlated with use of aspirin for fever in young people, avoidance of aspirin use by children is strongly recommended. Aspirin is in many over-the-counter and prescription drugs, including drugs for headache, fever, menstrual cramps, muscle pain, nausea, upset stomach, and arthritis. It may be used in drugs taken orally or by suppository.

Any of the following ingredients indicates that aspirin is present:

  • aspirin
  • acetylsalicylate
  • acetylsalicylic acid
  • salicylic acid
  • salicylate

KEY TERMS

Acetylsalicylic acid Aspirin; an analgesic, antipyretic, and antirheumatic drug prescribed to reduce fever and for relief of pain and inflammation.

Edema The abnormal accumulation of fluid in interstitial spaces of tissue.

Mitochondria Small rodlike, threadlike, or granular organelle witin the cytoplasm that function in metabolism and respiration.

Teenagers who take their own medications without parental consultation should be warned not to take aspirin-containing drugs.

Resources

ORGANIZATIONS

National Reye's Syndrome Foundation. P.O. Box 829, Bryan, OH 43506-0829. (800) 233-7393. http://www.bright.net/reyessyn.

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"Reye's Syndrome." Gale Encyclopedia of Medicine, 3rd ed.. . Retrieved May 22, 2017 from Encyclopedia.com: http://www.encyclopedia.com/medicine/encyclopedias-almanacs-transcripts-and-maps/reyes-syndrome-0

Reye's syndrome

Reye's syndrome (rīz), rare but life-threatening disease characterized by acute encephalopathy and fatty infiltration of internal organs, especially the liver. It occurs almost entirely in children under age 15. The cause is unknown, but the disease usually follows an acute viral infection (particularly influenza or chicken pox), especially when aspirin or other salicylates have been given. The symptoms, which occur about a week after the virus infection, are vomiting and disorientation; these may be followed by seizures, coma, and respiratory arrest. Treatment is directed toward reducing brain swelling, correcting blood chemistry changes due to liver damage, and providing respiratory support if needed. Doctors recommend that children be given acetaminophen rather than aspirin for viral infections or fever.

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"Reye's syndrome." The Columbia Encyclopedia, 6th ed.. . Encyclopedia.com. 22 May. 2017 <http://www.encyclopedia.com>.

"Reye's syndrome." The Columbia Encyclopedia, 6th ed.. . Encyclopedia.com. (May 22, 2017). http://www.encyclopedia.com/reference/encyclopedias-almanacs-transcripts-and-maps/reyes-syndrome

"Reye's syndrome." The Columbia Encyclopedia, 6th ed.. . Retrieved May 22, 2017 from Encyclopedia.com: http://www.encyclopedia.com/reference/encyclopedias-almanacs-transcripts-and-maps/reyes-syndrome

Reye's Syndrome

REYE'S SYNDROME

DEFINITION


Reye's syndrome is a disorder that primarily affects the liver and brain. It attacks the nervous system very quickly and can cause death.

DESCRIPTION


Reye's syndrome primarily affects children and teenagers. In almost all cases, it follows a viral illness, such as a cold (see common cold entry), the flu (see influenza entry), or chickenpox (see chickenpox entry). The disorder can affect any organ in the body, but its most serious effects occur in the brain and the liver. As the disorder develops, it attacks the body's nervous system. It produces symptoms such as listlessness, confusion, seizures, and coma. In extreme cases, it can lead to death.

Reye's syndrome is a rare disorder. It was first discovered in the early 1970s. The number of cases of Reye's syndrome rose slowly until 1980. In that year, 555 cases of the disorder were diagnosed. Researchers had learned at that point that children who are given aspirin are at risk for Reye's syndrome. Doctors began to warn parents against the use of aspirin with sick children. As a result of those warnings, the number of cases of Reye's syndrome began to fall. By the late 1990s the condition was very rare in the United States.

Reye's syndrome is sometimes confused with other nervous-system disorders. The reason for this confusion is that many doctors have never seen a case of Reye's syndrome. They may diagnose a patient with encephalitis, diabetes, poisoning, or some other condition by mistake. The actual number of cases of Reye's syndrome may therefore be somewhat higher than the official total.

CAUSES


The cause of Reye's syndrome is not known. What researchers do know is that the disorder usually appears after a viral infection in the upper respiratory (breathing) system.

Reye'S Syndrome: Words to Know

Acetylsalicylic acid:
The chemical name for the primary compound from which aspirin is made. Shorthand terms for acetylsalicylic acid include acetylsalicylate, salicylic acid, and salicylate.
Encephalitis:
An infection of the brain, also known as "brain fever."
Enzymes:
Chemicals present in all cells that make possible the biological reactions needed to keep a cell alive.
Hyperventilation:
Deep, heavy breathing.

One important fact researchers have discovered is the role of aspirin in Reye's syndrome. They have learned that people who take aspirin to treat the viral infection are at greater risk for Reye's syndrome. For that reason, aspirin is no longer recommended for children under the age of nineteen who have a fever.

SYMPTOMS


Whatever the cause, Reye's syndrome is accompanied by two kinds of effects on the body. First, there is a buildup of fats in body organs, especially the liver. These fats interfere with the normal operation of the organ. They may cause it to shut down.

Second, fluids begin to accumulate in the brain. These fluids push on blood vessels in the brain. The pressure may become so great that blood can no longer flow into the brain. If the brain can no longer function death will result.

The symptoms of Reye's syndrome appear after a viral infectionafter the original viral infection has gotten better. The patient may think that the original disease is over. But new symptoms then appear quite suddenly. The first of these symptoms is violent vomiting. This may be followed by a state of quietness, lethargy, agitation, seizures, and coma. In infants, diarrhea may be more common than vomiting. Fever is usually absent at this point.

DIAGNOSIS


Diagnosis of Reye's syndrome is often based on the patient's pattern of illness. The patient may have been ill with a viral disease and then gotten better. If the symptoms described above then suddenly appear, Reye's syndrome may be suspected.

Confirmation of this diagnosis can be made with a blood test. The test is designed to look for certain liver enzymes. Enzymes are chemicals in the body that change the rate at which reactions take place in cells. When an organ is diseased, the number of enzymes present may increase dramatically. The blood test for Reye's syndrome involves measuring changes in liver enzymes.

TREATMENT


Reye's syndrome is a life-threatening emergency. It requires immediate medical attention. The likelihood of recovery is best if the condition is recognized and treated promptly.

The first two steps in treatment are usually an intravenous (into the bloodstream) feeding of sugar and a blood transfusion. These steps help the body recover some of its normal functions. Pressure in the brain is carefully monitored. If it becomes too high, hyperventilation may be needed. Hyperventilation is any process by which the patient is made to breathe more rapidly. The process helps tighten blood vessels in the brain, releasing pressure on the brain.

PROGNOSIS


The death rate for Reye's syndrome is between 30 percent and 50 percent. The chance of recovery can be as high as 90 percent if the disorder is recognized and treated early. Almost all children who survive Reye's syndrome recover fully. However, recovery may take a long time. Some patients who recover experience permanent damage to the nervous system. These patients may require special physical and/or educational services and equipment.

PREVENTION


Since the cause of Reye's syndrome is not known, there is no certain way to prevent the condition. The best advice seems to be to avoid giving aspirin to children. Aspirin appears to be associated with many cases of Reye's syndrome. Many products contain aspirin even if that name is not used. Other names that indicate the presence of aspirin in a product include:

  • Acetylsalicylate
  • Acetylsalicylic acid
  • Salicylic acid
  • Salicylate

Children who require medication for fever or pain should take other, non-aspirin products, such as acetaminophen (pronounced uh-SEE-tuh-MIN-uh-fin, trade name Tylenol) or ibuprofen (pronounced I-byoo-pro-fen, trade names Motrin, Advil).

FOR MORE INFORMATION


Organizations

National Reye's Syndrome Foundation. PO Box 829, Bryan, OH 435060829. (800) 2337393. reyessyn@mail.bright.net.

Web sites

"Childhood InfectionsReye's Syndrome." Kidshealth.org. [Online] http://kidshealth.org/parent/common/reye.html.

"Reye's Syndrome." [Online] http://www.ama-assn.org/insight/h_focus/nemours/infectio/childhd/reyes.htm.

"Reye's Syndrome." [Online] http://gi.ucsf.edu/alf/info/inforeyes.html.

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Reye's syndrome

Reye's syndrome (rayz) n. a rare disorder occurring in childhood. It is characterized by the symptoms of encephalitis combined with evidence of liver failure. Treatment is aimed at controlling cerebral oedema and correcting metabolic abnormalities, but there is a significant mortality and there may be residual brain damage. The cause is not known, but aspirin has been implicated and this drug should not be used in children below the age of 16 unless specifically indicated. [ R. D. K. Reye (1912–77), Australian histopathologist]

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