What Is Reye's Syndrome: Causes and Treatments

What is Reye’s syndrome?

Reye's syndrome is a rare but serious condition that can cause swelling of the liver and brain. Even though this condition can occur at any age, it is mostly reported to affect children and teenagers, especially those who are trying to recover from viral infections, such as chickenpox and flu. 

Few of the symptoms of Reye's syndrome are seizure attacks, confusion, and loss of consciousness in some cases, which may need emergency medical attention. If this condition is diagnosed and treated in a timely manner, then it can significantly help in the successful recovery of the patient. 

The use of aspirin has been linked with Reye’s syndrome. Thus, aspirin must not be given to children and teenagers who have viral infections to avoid the risk of illness. It is still best to consult a doctor before giving any type of medication to your child. 

Risk Factors

Below are the known risk factors for Reye's syndrome:

• Children with a recent history of viral infection, particularly chickenpox, flu, or viral gastroenteritis. 
• Those who have taken aspirin to control their fever. 
• Those who have an undiagnosed metabolic disorder or fatty acid oxidation disorder (FAOD).

Symptoms

When children develop Reye’s syndrome, their blood sugar level would drop along with a sharp increase in the level of ammonia and acidity in their blood. At the same time, there would be swelling of the liver and brain, which may lead to seizures or convulsions and a loss of consciousness. 

The symptoms usually appear about three to five days from the start of the viral infection. The condition can also occur due to a cold or an upper respiratory infection. The first few signs of Reye's syndrome in young children are rapid breathing and diarrhea. On older children and teenagers, there would be persistent vomiting and lethargy. 

Other additional signs and symptoms of Reye's syndrome, which can become serious:

• Seizures
• Irritability
• Aggression
• Extremely feel tired or sleepy
• Confusion
• Loss of consciousness
• Hallucinations
• Leg or arm paralysis (in certain cases)

Emergency medical treatment and hospitalization should be done as soon as any of the above-mentioned signs and symptoms are observed. 

Causes

The exact cause of Reye’s syndrome has not been identified yet. However, there are several factors that play a role in the development of this syndrome. It has been seen that the use of aspirin for treating viral infections, such as chickenpox or influenza (flu) in children or teenagers is known to cause Reye’s syndrome, especially in individuals who have an underlying fatty acid oxidation disorder.

Fatty acid oxidation disorder is basically a group of inherited metabolic disorders, wherein the body becomes unable to break down its fatty acids since there is a missing enzyme or an enzyme that does not work as it normally should. The doctor would need to carry out a screening procedure to identify the presence of any fatty acid oxidation disorder present in the patient.

In a few individuals, Reye’s syndrome has been identified as an underlying metabolic condition that is mostly exposed by certain viral infections. Environmental factors are also known to play a role in triggering this condition. They include the individual’s exposure to certain toxins, which can include insecticides, any kind of paint thinner, and herbicides.

Few over-the-counter medications are also known to contain salicylate, which is quite similar to the one found in aspirin. Few of the examples are, bismuth subsalicylate and products that are known to contain oil of wintergreen, which are often in the form of topical medication. It is important not to give these medications to children, especially those who have or had a viral infection. These medications should also be avoided for at least several weeks even after the child has received vaccination for chickenpox.

Diagnosis

There are no specific tests carried out for Reye’s syndrome. The screening of this medical condition would begin from carrying out a blood test as well as a urine test. There would also be testing carried out for fatty acid oxidation disorder as well as other metabolic disorders.

In certain cases, there are more invasive diagnostic tests, which are needed to evaluate other possible causes of liver issues. These tests are also used to determine any form of neurological abnormalities. Few examples of the tests are mentioned below:

• Liver Biopsy: This procedure would help the doctor rule out any other conditions, which can be affecting the patient's liver. A liver biopsy involves the use of a needle, which would be inserted through the skin on the upper right side of the abdomen and then into the liver. The doctor would then remove a small sample of the liver tissue, which would then be sent to the laboratory for analysis.

• Lumbar Puncture or Spinal Tap: This particular procedure is helpful for the doctor to identify or to rule out other medical conditions that have the same signs or symptoms. Other conditions may include meningitis or encephalitis in certain cases. In a spinal tap procedure, the doctor would insert a needle through the lower back and into the space, which is below the end of the spinal cord. The doctor would then remove a small sample of the cerebrospinal fluid, which would then be sent to the laboratory for further analysis.

• Skin Biopsy: If the doctor wants to determine any fatty acid oxidation disorder or any metabolic disorder, then a skin biopsy may be performed. The doctor would take a small skin sample for further analysis in the laboratory. This procedure is usually carried out in the doctor's office with the help of local anesthesia.

• CT Scan: A CT scan is an imaging technique that shows a detailed 2D image of the brain.

Treatment

This condition is usually treated in the hospital. In severe cases, patients would need to be closely monitored and treated in the intensive care unit (ICU). Moreover, there is no absolute effective treatment for Reye’s syndrome. The main goal of the treatment would be aimed at reducing the effects caused by a metabolic dysfunction.

The main goal of the doctors would be to manage the patient's electrolyte imbalance as well as to reduce the swelling of the brain if any. It becomes difficult to predict on which of the patients would have a progressive illness, but doctors may recommend the use of certain medications, which are mostly aimed at reducing the serum ammonia level in the body since the level of ammonia present in the body is also known to cause brain swelling. 

Hemodialysis or kidney dialysis can also be done in patients who have Reye's syndrome to remove the toxins that are believed to be the cause of brain swelling. 

Other forms of treatment may include:

• Diuretics: The doctor can give these medications to lower intracranial pressure as well as to increase the production of urine. 

• Intravenous fluids: The doctor can provide glucose as well as an electrolyte solution, which can be administered through an intravenous line (IV).

• Medications to prevent bleeding: If the child suffers from bleeding due to liver abnormalities, then the doctor may need to carry out treatment by giving vitamin K, platelets, and plasma.

If the child is having issues with breathing, then he or she may need the assistance of a breathing machine called a ventilator. Doctors would ensure that the child stays hydrated as much as possible and bring back the electrolyte levels back to normal. They would also evaluate the patient's cardiorespiratory (heart and lung) status.

The functioning of the liver would also be carefully monitored from time to time. Children with seizure attacks or convulsions would be given an appropriate medication to control the attacks as well as their side effects. The individual may also be given insulin to increase the metabolism of glucose in the body. Corticosteroids would be given to reduce the swelling of the brain.

A better outcome is expected if the condition is properly diagnosed along with prompt treatment. If the condition is not diagnosed in a timely manner and the child progresses to the advanced stage of Reye’s syndrome, irreversible permanent damage to the brain may occur.

Prevention

Nowadays, Reye’s syndrome has become a less common illness due to a significant decline in the use of aspirin in children. If a child has a headache, it would be best to use acetaminophen for treatment instead of aspirin. However, give the correct dosage of acetaminophen to your child since too much of it is not good for the body and may cause liver damage.

If your child's fever or headache is not relieved through the use of acetaminophen, consult a doctor immediately.