Posted in Pediatrics

Reye’s syndrome is a rare but very serious condition that involves acute encephalopathy, cerebral edema and fatty liver failure. The pathophysiology is not fully understood but what we do know points to mitochondrial injury secondary to viral illness and the use of aspirin in young children. Note that it’s not salicylate overdose that leads to Reye’s syndrome, but simply aspirin usage. In a study conducted in 2018, 80 percent of pediatric patients who developed Reye’s syndrome had taken aspirin in the prior three weeks. Note that many times the aspirin use is directly related to symptom treatment of viral illnesses.

Reye’s syndrome most commonly affects children ages 5 to 16, especially those with a recent history of viral illness. Data spanning 1980 -1997 from the Centers for Disease Control and Prevention (CDC) showed that a recent history of influenza was present in 73% of cases, while varicella infections were present in 21% of cases, and 14% of cases were preceded by gastroenteritis infections. 

Without treatment, Reye’s syndrome can be fatal and death is usually due to cerebral edema. Children can also suffer long-term neurological deficits that range from mild to severe. Though the organs affected the most are the brain and liver, any organ can be affected and the complications of Reye’s syndrome are widespread. In addition to generalized neurological impairments and increased intracranial pressure, complications can include fluid and electrolyte imbalances, hypoglycemia, hyperammonemia, coagulopathies (including DIC), seizures, GI bleeds, pancreatitis, renal failure, respiratory failure, and sepsis.

Though mortality from Reye’s syndrome has decreased drastically since 1980, the condition still has an average mortality rate of 21% according to the Cleveland Clinic. Early recognition and treatment are crucial for avoiding long-term complications and reducing the risk of death.

Now that you have some background knowledge about Reye’s syndrome, let’s dive into caring for these patients using the Straight A Nursing LATTE method.

L: How does the patient LOOK?

When symptoms of Reye’s syndrome manifest can vary, but symptoms typically appear about three to five days after the viral illness onset. However, some studies show symptoms can appear up to three weeks after the illness.

The neurological symptoms that are the hallmark of Reye’s syndrome are due to increased intracranial pressure and elevated ammonia levels secondary to liver dysfunction. Initial symptoms are typically persistent nausea coupled with an acute change in mental status such as confusion, weakness, visual changes, hearing changes and lethargy. 

As the condition worsens, intracranial pressure increases and symptoms can include progressively worsening LOC, tachypnea, tachycardia, seizures, weakness or paralysis in the arms/legs, fixed and dilated pupils, posturing, and coma. In infants, signs of increased ICP are a high-pitched cry and bulging fontanel.

Though the liver is the other key organ affected by Reye’s syndrome, hepatomegaly without jaundice is present in less than half of the cases.

A: How do you ASSESS the patient?

When Reye’s syndrome is suspected, it’s important to inquire about recent viral illnesses and aspirin use. It is also important to ask about a history of Kawasaki disease, as a key treatment for this condition is aspirin. Other important assessments include:

  • Neurological assessment, including the patient’s Glasgow Coma Scale score. If the child is 2 years of age or younger, use the Pediatric Glasgow Coma Scale instead. 
  • Assess for signs of increased ICP such as decreased LOC, headache, vomiting, visual or hearing changes, weakness, and confusion. In infants, a high-pitched cry and bulging fontanel are signs of increased ICP.
  • Continuous ICP monitoring utilizing an intracranial catheter (only utilized in the critical care environment).
  • Measure intake and output every one to two hours as the child is at high risk for dehydration secondary to vomiting, diarrhea, and the use of osmotic diuretics. The patient will likely need an indwelling catheter.

T: What TESTS will be conducted?

Reye’s syndrome is typically diagnosed by excluding other conditions such as encephalopathy, meningitis, liver disease, inborn errors of metabolism (IEMs), and adverse drug reactions.

  • Liver function tests – AST and ALT are drastically elevated while bilirubin is normal
  • Ammonia level – Higher serum ammonia levels may be associated with poorer prognosis
  • Liver biopsy – The biopsy will show fatty changes to the liver
  • Prothrombin time – A prolonged prothrombin time is supportive of a diagnosis of Reye’s syndrome
  • Lumbar puncture – May be conducted to rule out other causes for the symptoms, such as meningitis. The procedure will show elevated pressure and the CSF will have normal protein levels and WBCs less than 8
  • Skin biopsy – Metabolic disorders and fatty acid oxidation disorders can be diagnosed with a skin biopsy
  • Abdominal ultrasound – May be utilized to assess hepatomegaly
  • Imaging studies –  CT or MRI will show edema as intracranial pressure increases
  • Ongoing labs – Liver enzymes, coagulation studies, serum glucose and electrolytes. If other organs are affected, associated labs will be closely monitored.

T: What TREATMENTS are provided? 

Reye’s syndrome is typically treated in the critical care environment with supportive care to decrease intracranial pressure, maintain hemodynamic stability, prevent hypoglycemia, support respiratory function, and address coagulopathy.

Specific treatments to decrease intracranial pressure include:

  • Administration of osmotic diuretics such as mannitol or hypertonic saline.
  • Administration of dexamethasone to reduce cerebral edema
  • Maintain head in neutral position with HOB elevated
  • Avoid severe flexion at the hips and tight or restrictive clothing
  • Fluid restriction in some cases
  • Decompressive craniotomy in severe cases
  • Mechanical ventilation with hyperventilation (used short-term)
  • Maintain normothermia (often requires external cooling measures)
  • Sedation

Other supportive treatments may consist of: 

  • Dextrose infusion to prevent hypoglycemia
  • Vitamin K, platelets or fresh frozen plasma to prevent bleeding
  • Replace electrolytes as needed
  • Replace fluids as needed to maintain euvolemia
  • Administer lactulose to remove excess ammonia levels (severe hyperammonemia may require dialysis)
  • Anticonvulsants may be utilized to prevent seizure
  • Enteral feeding via a nasogastric tube

E: How do you EDUCATE the family?

The key education point for Reye’s syndrome is to avoid giving aspirin to children unless advised to do so by a physician. Alternatives for fever and pain management are acetaminophen and ibuprofen. Teach parents to read all medication labels to look for aspirin and salicylates, which may go by various names such as “salicylic acid” or “methyl salicylate.” Remind parents they can always consult with their pharmacist or pediatrician before administering any prescription or OTC medications.

Because Reye’s syndrome can be fatal if untreated, teach parents to always seek medical attention if they notice the signs and symptoms in their child. Make sure they know what to watch for and that a full recovery is possible if Reye’s syndrome is caught and treated early.

Maximize your learning about Reye’s syndrome by listening to this information in episode 257 of the Straight A Nursing podcast. Tune in wherever you get your podcast fix, or straight from the website here.

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REFERENCES

Chapman, J., & Arnold, J. K. (2022). Reye Syndrome. https://www.ncbi.nlm.nih.gov/books/NBK526101

Chornomydz, I., Boyarchuk, O., & Chornomydz, A. (2017). REYE (RAY’S) SYNDROME: A PROBLEM EVERYONE SHOULD REMEMBER. Georgian Medical News, 272, 110–116.

Cleveland Clinic. (n.d.). Reye’s Syndrome: Symptoms, Causes, Treatment & Outlook. Cleveland Clinic. https://my.clevelandclinic.org/health/articles/6088-reyes-syndrome

Gallucci, M., Smith, J. D., Limbucci, N., Rossi, A., Demaerel, P., Krings, T., D’Amico, A., & Micheli, C. (2012). Pediatric Inflammatory Diseases. The Neuroradiology Journal, 15.

Magrum, B. G., & Pickworth, K. K. (2020). Aspirin rechallenge in an adult patient previously diagnosed with Reye syndrome. American Journal of Health-System Pharmacy, 77(2), 123–127. https://doi.org/10.1093/ajhp/zxz276

Mayo Clnic. (n.d.). Reye’s syndrome—Symptoms and causes. Mayo Clinic. https://www.mayoclinic.org/diseases-conditions/reyes-syndrome/symptoms-causes/syc-20377255

Medscape. (n.d.). Salicylates | Drug, OTCs & Herbals | Medscape Reference. https://reference.medscape.com/drugs/salicylates

National Organization for Rare Disorders. (n.d.). Reye Syndrome. NORD (National Organization for Rare Disorders). https://rarediseases.org/rare-diseases/reye-syndrome/

Pribožič, L., Tanšek, M. Ž., Herga, P., Osredkar, D., Osredkar, S. R., Vidmar, I., Lampret, B. R., Klemenčič, S., Bratina, N., Battelino, T., & Groselj, U. (2021). Reye Syndrome with Severe Hyperammonemia and a Good Neurological Outcome. The American Journal of Case Reports, 22, e932864-1-e932864-6. https://doi.org/10.12659/AJCR.932864

Raab, C. (n.d.). Reye Syndrome—Pediatrics. Merck Manuals Professional Edition. https://www.merckmanuals.com/professional/pediatrics/miscellaneous-disorders-in-infants-and-children/reye-syndrome