Reyes (Reye) Syndrome: Causes, Symptoms, Treatment, Risk Factors, Complications

Reyes (Reye) Syndrome is a rare, but an extremely serious pathological condition associated with swelling of liver and brain. Reyes (Reye) Syndrome usually affects children and teenagers with metabolic abnormalities who are recovering from some sort of a viral infection. In this article, we will read about the various causes, symptoms, and treatments rendered for this potentially serious medical condition called Reyes (Reye) Syndrome.

• Viral Infection-
  • Reyes (Reye) Syndrome is observed in children and adolescents following viral infection.¹
• Aspirin ¹–
  • Some studies have shown that aspirin can cause Reyes Syndrome.
  • Aspirin should never be given to adolescents who are recovering from chickenpox and other flu like illness.
• Metabolic Abnormalities²–
  • Illness causes metabolic abnormalities.
  • Aspirin should be used with caution in teenagers, especially those who have already been identified to have metabolic abnormalities and viral infection.
  • Additionally, the blood of the child becomes significantly acidic. There may also be swelling of brain resulting in seizures or loss of consciousness.
• Hypoglycemia-
  • In Reyes (Reye) Syndrome, there is a significant decrease in blood sugar levels of a child and elevation in ammonia levels.
• Brain Edema-
  • Metabolic disorder and acidosis in late stage causes cerebral edema resulting in encephalopathy¹
  • Serious symptoms observed, which needs immediate medical attention are confusion, seizures and loss of consciousness.
  • Brain stem disorder may follow brain edema causing fatal outcome.³
• Fatty Liver-
  • Delayed stage is associated with liver fatty deposits.
  • Irregular liver metabolism causes metabolic acidosis and hypoglycemia.

• Reyes (Reye) Syndrome Caused Due to Viral Infection³ –
  • Few cases of viral infection in children and adolescent end up in symptoms of Reyes (Reye) Syndrome.
• Reyes (Reye) Syndrome Caused Due to Aspirin⁴–
  • Aspirin when prescribed during viral infection may cause symptoms of Reyes (Reye) Syndrome.
  • Viral infection associated with Reyes (Reye) Syndrome is flu, chickenpox, influenza and upper respiratory tract infection.
• Fatty Acid Oxidation Dysfunctions-
  • The disorder is caused by enzyme deficiency, which is necessary to break down fatty acid.
  • The disorder is inherited metabolic dysfunctions where the body is unable to break down fatty acids by oxidation.
  • Aspirin may be influencing the fatty acid oxidation though the metabolic relation is yet not established.
• Reyes (Reye) Syndrome Caused Due to Toxins-
  • Effects of toxins may cause liver metabolic dysfunction though research yet has not been able to find the biological changes in cell or tissue.
  • Toxins such as pesticides and paint are associated with Reyes Syndrome.

• Age- Children and adolescents are at high risk of suffering from Reyes (Reye) Syndrome
• Liver Disease- Underlying liver disease may trigger Reyes (Reye) Syndrome in children and adolescents who suffer with viral infection and are treated with aspirin.
• Aspirin Treatment- Aspirin when prescribed for children and adolescents during viral infection can trigger Reyes (Reye) Syndrome.

The signs and symptoms of Reyes (Reye) Syndrome are usually observed about a week after a viral infection like influenza or chickenpox.

• Fever
• Weakness, lethargy and fatigue.
• Vomiting.
• Runny nose.
• Diarrhea.
• Irritability and anxious.

• Confusion
• Hallucination
• Seizures
• Unconscious
• Severe hypoglycemia
• Metabolic acidosis

• Blood Test-
  • There are no specific test to make a confirmatory diagnosis of Reyes (Reye) Syndrome.
• Spinal Tap-
  • Cerebrospinal fluid (CSF) is collected by spinal tap
  • CSF is studied for infection and metabolic diseases.
  • CFF study is indicated to rule out other causes of encephalopathy and seizure disorder.
• Liver Biopsy-
  • Liver biopsy is done to rule out diseases, which may cause hypoglycemia and metabolic acidosis.
  • Diagnose associated liver disorder.
• CT Scan /MRI (Magnetic Resonance Imaging):
  • Evaluate swelling of brain and liver, which is often seen with Reyes (Reye) Syndrome.
  • Rule out causes such as brain abscess, brain tumor or brain hemorrhage as a cause of symptoms like unconsciousness or seizure disorder.
  • Absence of brain disease supports diagnosis of Reyes Syndrome.
• Skin Biopsy:
  • This test is done to look for any Fatty Acid Oxidation disorders or other metabolic abnormalities.
  • Electroencephalogram (EEG)-
  • EEG is performed to evaluate seizure disorder.³

Patient suffering from Reyes (Reye) Syndrome is admitted in hospital for observation and treatment of encephalopathy and hypoglycemia. Symptoms of seizure disorder, severe hypoglycemia and encephalopathy need Intensive Care Unit treatment.

• Intravenous Fluids ¹–
  • Maintain normal serum glucose level
  • Monitor and correct metabolic acidosis
  • Correct electrolyte abnormalities.
• Diuretics-
  • Maintain normal urine output
  • Treat cerebral (brain) edema
  • Treat water retention
• Anti-Seizure Medications for Reyes (Reye) Syndrome –
  • Tegretol
  • Zarontin
  • Lamictal
• Bleeding Disorder-
  • Correct the cause of bleeding disorder
• Emergency Treatment-
  • Delay in medical attention can result in life threatening complication.

Majority of individuals with Reyes (Reye) Syndrome are able to survive but possibly may end up having permanent brain damage. If Reyes (Reye) Syndrome remains undiagnosed and is not appropriately treated then this diseases may prove fatal within a few days of the onset.

References: