Reye’s Syndrome (Reye-Johnson Syndrome) : Symptoms, Causes, Diagnosis & Treatment

Reye’s Syndrome (Reye-Johnson Syndrome): Symptoms, Causes, Diagnosis & Treatment

Reye’s syndrome also known as Reye-Johnson syndrome is a rare disorder that can cause serious liver and brain damage. If not treated immediately, it may lead to permanent brain injury or death.

Reye-Johnson syndrome is a rapidly progressive encephalopathy which causes death in 20–40% of those affected and about a third of those who survive are left with a significant degree of brain damage. About 90% of cases in children are associated with aspirin use and often the liver is enlarged.

When aspirin was withdrawn for use in children a decrease of more than 90% in rates of Reye’s syndrome was seen. Children are the most commonly affected individuals. Reye’s affects less than one in a million children per year. Fewer than 5 thousand cases per year are reported in India.

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How Does Reye’s Syndrome Affect Your Body?

Reye-Johnson syndrome affects all organs of the body but is most harmful to the brain and the liver. Reye’s syndrome causes an acute increase of pressure in the brain and often massive accumulations of fat in the liver and other organs.

What Are The Causes of Reye’s Syndrome?

The exact cause of Reye’s syndrome is unknown. However, there are certainly other causes that may influence this disease to occur.

  • Reye’s syndrome can be triggered by using aspirin to treat a viral illness or infection in children and teenagers who have an underlying fatty acid oxidation disorder.
  • Exposure to certain toxins such as insecticides, herbicides and paint thinner may also contribute to Reye’s syndrome.

What Are The Risk Factors of Reye’s Syndrome?

  • Using aspirin to treat a viral infection, such as flu, chickenpox or an upper respiratory infection in children puts them at risk.
  • Having an underlying fatty acid oxidation disorder may increase the risk of Reye’s syndrome.
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What Are The Symptoms of Reye’s Syndrome?

Reye’s syndromes passage through five stages. Symptoms of Reye’s syndrome at stage I includes:

  • Rash on palms of hands and feet.
  • Persistent, heavy vomiting.
  • Lethargy
  • Confusion
  • Nightmares
  • Headaches

Symptoms at stage II includes:

  • Stupor
  • Hyperventilation
  • Fatty liver (found on biopsy)
  • Hyperactive reflexes

Symptoms at stage III includes:

  • Continuation of Stage I and II symptoms
  • Possible coma
  • Possible cerebral edema
  • Rarely, respiratory arrest

Symptoms at stage IV includes:

  • Deepening coma
  • Dilated pupils with minimal response to light
  • Minimal but still present liver dysfunction

Symptoms at stage V includes:

  • Deep coma
  • Seizures
  • Multiple organ failure
  • Flaccidity
  • Hyperammonemia
  • Death
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How is Reye’s Syndrome Diagnosed?

There’s no specific test for Reye’s syndrome.

  • Screening tests – Blood and urine tests, as well as testing for fatty acid oxidation disorders and other metabolic disorders, are done to look for Reye’s.
  • Spinal tap (lumbar puncture) – A spinal tap can help identify or rule out other diseases with similar signs and symptoms, such as meningitis or encephalitis.
  • Liver biopsy – A liver biopsy can help identify or rule out other conditions that may be affecting the liver.
  • Imaging tests – Computerized tomography (CT) scan or magnetic resonance imaging (MRI) can help identify or rule out other causes of behavior changes or decreased alertness.
  • Skin biopsy – A skin biopsy may be required to test for fatty acid oxidation disorders or metabolic disorders.
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How To Prevent And Control Reye’s Syndrome?

  • Avoid giving aspirin to children and teenagers, instead, give acetaminophen or ibuprofen to get relief from fever and pain.
  • Children with known fatty acid oxidation disorders should not be given aspirin or aspirin-containing products.

Treatment of Reye’s Syndrome – Allopathic Treatment

Treatment of Reye’s is supportive. Medications used are:

  • Intravenous fluids – Glucose and an electrolyte solution may be given through IV tube.
  • Diuretics – This may be used to decrease intracranial pressure and increase fluid loss through urination.
  • Mannitol – This medicine may be used to help with brain swelling.

Treatment of Reye’s Syndrome – Homeopathic Treatment

No known homeopathic treatment is available at present.

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Reye’s Syndrome – Lifestyle Tips

Instead of aspirin, give acetaminophen or ibuprofen to your child for pain and fever.

What Are The Recommended Exercises For a Person With Reye’s Syndrome?

No specific exercise has been recommended for patients with Reye’s syndrome.

Reye’s Syndrome & Pregnancy – Things to Know

  • Daily low-dose aspirin use in pregnancy is considered safe and is associated with a low possibility of serious maternal, or fetal complications.
  • Low-dose aspirin prophylaxis is recommended in women at high risk of preeclampsia and should be initiated between 12 weeks and 28 weeks of gestation and continued daily until delivery.
  • Low-dose aspirin prophylaxis should be reviewed for women with more than one of several moderate risk factors for preeclampsia.
  • In the absence of high-risk factors for preeclampsia, the use of prophylactic low-dose aspirin is not allowed to use for the prevention of early pregnancy loss, fetal growth restriction, stillbirth, or preterm birth.

Common Complications Related to Reye’s Syndrome

Most children who have Reye’s syndrome survive, although varying degrees of permanent brain damage are possible.

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