Reye's syndrome

Definition
Reye's or Reye syndrome is a rare but extremely serious illness involving inflammation of the brain and involving the collection of fatty deposits in the liver and other organs. When Reye's syndrome occurs, it nearly always follows an acute viral infection. Children of all ages can develop Reye's syndrome, but those between ages 4 and 12 are most vulnerable. Reye's syndrome occurs rarely in adults.

Causes/associated factors
The cause of Reye's syndrome is unknown, but it has been linked to various viruses, including varicella (the virus that causes chickenpox), influenzae A and B, echovirus 2, coxsackievirus A and Epstein-Barr virus. About 90 percent of Reye's cases are associated with a viral respiratory tract infection.
In 1980, it became clear that children who were treated with aspirin during flu-like illnesses and chickenpox had a higher risk of developing Reye's syndrome. Thanks to immediate warnings about the danger of giving aspirin to children with such illnesses, the incidence of Reye's syndrome has declined dramatically.

Reye's syndrome is not contagious, but cases sometimes cluster in certain geographic areas. Reye's syndrome can develop at any time, but more cases have been reported from December through April, when influenza is most prevalent.


Signs/symptoms
Symptoms of Reye's syndrome can be divided into five stages.
Stage I: Early signs/symptoms
Suspect Reye's syndrome when a child begins vomiting three days to three weeks following the onset of influenza, chickenpox or an upper respiratory infection. The vomiting may become increasingly severe over an eight- to 12-hour period. Other early symptoms may include:

listlessness
drowsiness
Note: For infants, vomiting does not always occur, and other symptoms may not be typical.

Stage II: Medical emergency
If Reye's syndrome progresses to the second stage, consider it a medical emergency. Symptoms may include:

hyperventilation (rapid breathing)
irritability or combativeness
disorientation or confusion
delirium
increased reflex action
Stages III and IV: Medical emergency
More advanced symptoms may include:

seizures
deepening coma
Stage V: Extreme emergency
The final stage of Reye's syndrome is considered an extreme medical emergency. Symptoms may include:

coma
seizures
paralysis (loss of control over body movements)
respiratory arrest

Diagnosis
In some cases, Reye's syndrome is tough to distinguish from other medical conditions such as encephalitis, meningitis, diabetes, drug overdose or poisoning. Because Reye's syndrome can progress quickly to delirium and coma, however, early diagnosis is crucial. The doctor will ask questions about the child's medical history and current symptoms and do a physical exam. Blood samples will be studied in the lab to confirm the diagnosis. High levels of ammonia and acid in the blood, low levels of sugar, elevated liver enzymes and decreased blood clotting ability help confirm a diagnosis of Reye's syndrome.
A lumbar puncture (taking a sample of cerebrospinal fluid from a space in the spinal cord) may be done to measure pressure in the brain as well as examine the cerebrospinal fluid. An electroencephalogram (EEG, a recording of the electrical activity of the brain) may help the doctor detect brain swelling or lack of blood flow. Sometimes, a liver biopsy (taking a tissue sample for further evaluation) is needed to help rule out other forms of liver disease.


Treatment
Reye's syndrome can quickly become a medical emergency. Immediate treatment is essential. Although there is no cure for Reye's syndrome, the likelihood of full recovery is much greater when it's treated in the early stages.
Treatment depends on the severity of the illness. In most cases, the child is hospitalized in an intensive care unit. The goals of treatment are to protect the brain from irreversible damage due to swelling, correct chemical changes, and prevent serious respiratory and cardiac complications.

Fluids and medications will be given intravenously (through a vein). If the blood is not clotting normally, plasma (the liquid part of blood), platelets (substances in the blood that help blood to clot) and vitamin K (which also helps reduce bleeding) may also be needed. Vital signs will be monitored frequently, and diagnostic tests will be repeated to monitor the progress of the disease. Noise and other forms of stimulation will be limited to help prevent a further increase in the pressure in the brain. Sedative medications may also be given. If necessary, the child may be intubated to help him or her breathe.


Complications
The survival rate for Reye's syndrome is about 70 percent, although this may improve with new advances in intensive care. Recovery depends on the severity of swelling in the brain. Recovery is more likely when treatment begins early. Some people, however, are left with varying degrees of permanent brain damage. When the condition progresses rapidly and the person enters a coma, outcomes are poor. Without treatment, the condition may be fatal within a few days.

Prevention
Always check with your doctor before giving your child any medications. Do not give aspirin or products containing aspirin to children or teens age 19 or younger when they have a fever or fever-causing illness, according to the U.S. Surgeon General, the U.S. Food and Drug Administration and the Centers for Disease Control and Prevention.
To reduce fever in children and teens, use acetaminophen instead of aspirin. Read labels carefully -- acetylsalicylate, acetylsalicylic acid, salicylic acid, phenyl salicylate, salicylamide and salicylate are other words for aspirin. Many over-the-counter medications contain aspirin. If you're not sure of the ingredients in a product, check with your doctor or a pharmacist.

Occasionally, children are prescribed aspirin products to treat rheumatoid arthritis or other medical conditions. This type of treatment requires careful medical monitoring. Children who take aspirin for a chronic condition are recommended to have a yearly flu shot to decrease the risk of influenza and the associated risk of Reye's syndrome.

Here's a list of common over-the-counter medications that may contain aspirin. This is not a complete list. Remember to read the label of any medication before giving it to your child. If you're not sure of the ingredients, check with your doctor or a pharmacist.

Alka-Seltzer products
Anacin products
Ascriptin products
Aspergum
Bayer products
BC products
Buffaprin
Bufferin products
Buffinol
Cama Arthritis Pain Reliever
Coricidin D
Doan's products
Dristan products
Ecotrin
Emperin Aspirin tablets
Excedrin products
Goody's Extra Strength or Headache Powders
Halfprin
Norwich Aspirin
Pamprin
Pepto-Bismol
Sine-Off Sinus Medicine
St. Joseph Adult Chewable Aspirin
Ursinus Inlay-Tabs
Vanquish caplets