The cause of Reye's syndrome remains unknown; however, a link was found with the use of aspirin or other salicylates in children and adolescents who have a viral infection such as influenza, chicken pox or the common cold. The increased risk of contracting Reye's Syndrome is one of the main reasons that aspirin is not recommended for use in people under the age of 16.

The syndrome is named for Dr R. Douglas Reye, who, along with Dr George Johnson, published the first study of the syndrome in 1963, though the disease was first diagnosed as a unique illness in 1929. In 1980 studies in Ohio, Michigan and Arizona by Starko et al pointed to the use of aspirin during an upper respiratory tract infection and chicken pox as a potential indicators for the syndrome. A decrease of the use of aspirin in children during the 1980s resulted in a corresponding decrease in the number of cases of Reye's syndrome, dramatically in children under 10 (Arrowsmith et al 1987). However, it is worth noting that a decrease in the number of cases has also been observed in countries where children's aspirin is still in use. Further case studies have revealed 19 viruses in conjuction with salicylates, pesticides and aflatoxin as potential factors contributing to the disease.

Presentation

Symptoms and signs

Reye's syndrome progresses through two stages :

Stage I
- Persistent or continuous vomiting and/or nausea, except for children under two who may have diarrhea or hyperventilate.
- Signs of brain dysfunction appear : listlessness, loss of energy, lethargy, drowsiness
Stage II
- Personality changes : irritability and aggressive behavior
- Disorientation : confusion, irrational and combative behavior
- Delirium, convulsions and coma

Features

Early diagnosis is vital, otherwise death or severe brain damage may follow.

The disease causes hepatic steatosis with minimal inflammation and severe encephalopathy (with swelling of the brain). Jaundice is NOT usually present. The liver may become slightly enlarged and firm, and there is a change in the appearance of the kidneys. (Suchy, Frederick, Ed: J Liver Disease in Children. Mosby, St. Louis.1994. Chapter 36.

Prognosis

In adults who survive the acute illness the recovery is generally complete, with liver and brain function returning to normal within two weeks of the illness. In children however, permanent brain damage is possible, especially in infants, and ranges from mild to severe.

Differential diagnosis

Causes for similar symptoms include

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Reye's syndrome
From Nursing, 12/1/99 by Doire, Terry Lee

GRUMPY AND LETHARGIC, Carol Moore, 12, arrives at your emergency department with her mother. Mrs. Moore says that Carol had a mild case of the flu a week ago and seemed to be recovering. But yesterday, she vomited repeatedly and started sleeping a lot, waking up very irritable. As you escort Carol to a stretcher, she starts screaming and resists being examined.

What's the situation?

Mrs. Moore tells you that Carol can't swallow pills, so she took chewable aspirin for her fever during the flu. You take Carol's vital signs: respirations, 24; pulse, 110; and temperature, 980 F (36.70 Q. Carol is disoriented but follows commands, her pupillary reaction is brisk, and her oculocephalic reaction is normal. You see no evidence of jaundice.

What's your assessment?

Based on Carol's history and signs and symptoms, you suspect Reye's syndrome, characterized by acute encephalopathy and liver failure. It can rapidly progress to coma and death.

The classic scenario is a child who's taken aspirin after a viral illness during the winter-but Reye's syndrome can affect anyone at any time of year, with or without aspirin use. The lethargy and personality changes that occur early in Reye's syndrome are caused by impaired neurologic function secondary to cerebral edema.

Treatment is primarily supportive. Your top priority is to address the cerebral edema and protect Carol from permanent neurologic damage.

What must you do immediately?

Carol is breathing on her own, so you administer 4 liters/minute of supplemental oxygen via nasal cannula and attach a pulse oximetry probe to her finger so you can monitor her SPO^sub 2^ Start an intravenous infusion of 10% dextrose in 0.9% sodium chloride solution: Hypoglycemia is common in Reye's syndrome. Draw blood for liver function tests, coagulation studies, blood glucose level, and routine chemistries. Also obtain a blood specimen to check Carol's serum salicylate level. Prepare Carol for a thorough neurologic examination, lumbar puncture, and cerebrospinal fluid (CSF) analysis to rule out other infections, such as encephalitis and meningitis. In some cases, a liver biopsy may be ordered for a definitive diagnosis. Obtain a more in-depth health history from Mrs. Moore.

Abnormal lab values include aspartate aminotransferase and alanine aminotransferase of 200 units/liter (normal is 0 to 40 units/liter), low blood glucose, prolonged prothrombin and partial thromboplastin times, elevated blood urea nitrogen and creatinine, and elevated serum ammonia. The CSF analysis rules out other infections.

What should be done later?

Carol is admitted to the pediatric intensive care unit, where her neurologic status and serum lab studies will be closely monitored. Carol will receive corticosteroids to reduce brain swelling. She may receive small amounts of insulin to increase glucose metabolism and vitamin K to aid in coagulation. If her condition worsens, she may need to be intubated and mechanically ventilated.

Carol is discharged home 10 days later with no permanent neurologic damage. Explain to Mrs. Moore that children under age 19 shouldn't take aspirin or other products containing salicylates during a febrile illness. Inform her that antinausea and cold medications may contain salicylates.

Advise her to check with Carol's health care provider before giving any medication when Carol is feverish. She can also contact the National Reye's Syndrome Foundation, Inc., at 1-800-233-7393 for more information or visit its Web site at http://www.bright.net/~reyessyn.

BY TERRY LEE DOIRE, RN,C, BSN

Nurse-Manager, Labor and Delivery Unit * Baptist Medical Center * Jacksonville, Fla. Adjunct Instructor * Florida Community College at Jacksonville * Jacksonville * Jacksonville, Fla.

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