Reye syndrome

Reye syndrome is a hepatoencephalopathy (inflammation of the liver and brain), primarily affecting children, triggered by the use of Aspirin® or salicylates as a treatment for the fever of a viral infection, such as influenza or chickenpox.

It is rare in adults, and more frequent among children and adolescents, and typically manifests approximately one week after the onset of an upper respiratory tract infection, such as the flu or chickenpox. Additionally, it seems that there may be a genetic predisposition.

It is characterized by symptoms such as vomiting, behavioral changes, agitation, and delirium, which can rapidly progress to convulsions and coma, and ultimately may lead to the death.

Although it is typically linked to the use of Aspirin® or salicylates as a treatment for fever in viral infections, Reye syndrome can also be associated with the use of other medications such as metoclopramide (used for treating vomiting), zidovudine (used for treating HIV), and valproic acid (used for treating epilepsy), albeit in rare cases.

There are 4 stages in the Reye syndrome:

Stage 1:

• Persistent or continuous vomiting
• Signs of brain dysfunction:
  • Lack of energy or enthusiasm
  • Loss of energy
  • Drowsiness

Stage 2:

• Personality changes:
  • Irritability
  • Aggressive behavior

Stage 3:

• Disorientation:
  • Confusion
  • Irrational behavior
  • Combative

Stage 4:

• Delirium
• Convulsions and strange postures, with the arms fully extended and turned over the body, the legs straight and with the toes stretched outwards on the soles of the feet
• Coma

In babies, it is possible that vomiting is not present. They can suffer unspecific symptoms like diarrhea or irregular breathing.

If a child with a previous viral infection has been treated with Aspirin® or salicylates, it is important to conduct the following diagnostic tests:

• Measurement of blood sugar levels, which are typically low in such cases.
• Assessment of transaminase levels (elevated AST).
• Evaluation of serum ammonia levels, which are often found to be elevated.
• Lumbar puncture to study the cerebrospinal fluid.
• Brain scan or CT.

The treatment for Reye syndrome is very intensive, and therefore hospitalization is necessary.

As Reye syndrome is a highly complex and rare condition, it is recommended that it be managed by a hospital with extensive experience in treating the syndrome at a high level of care.

The treatment is focused on:

• Treating cerebral edema (accumulation of fluid in the head), which is the leading cause of death. Lumbar puncture should not be performed. Oxygen and other medications are used. Sometimes, it may be necessary to monitor breathing and occasionally provide assisted breathing.
• Effective treatment of elevated levels of ammonia in the blood (toxic substances) and provides some treatments to prevent further deterioration of liver function.
• Adequate nutrition and hydration: electrolyte and glucose solutions will be administered.
• Treating any possible complication: primarily blood clotting problems.

The recovery percentage is about 60%. The rest has a bad prognosis usually leading to the death.

The long-term consequences will be determined by the severity of the acute condition and the effectiveness of the individualized treatment approach.

The use of acetylsalicylic acid (Aspirin®) and other salicylates should be avoided as a treatment for fever or malaise resulting from viral infections, flu, or chickenpox in children and adolescents (under 18 years of age).

Instead, Paracetamol or Ibuprofen are safer alternatives for such patients.