How is stage 2 Reye syndrome treated?

Updated: Apr 02, 2018
  • Author: Debra L Weiner, MD, PhD; Chief Editor: Kirsten A Bechtel, MD  more...
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Answer

The standard of care consists of continuous cardiorespiratory monitoring, placement of central venous lines or arterial lines to monitor hemodynamic status, urine catheters to monitor urine output, ECG to monitor cardiac function, and EEG to monitor seizure activity. Endotracheal intubation may be required at this stage to maintain the airway, control ventilation, and prevent increased ICP. Use rapid-sequence agents that minimize the chance of increasing ICP. Place a nasogastric tube to decompress the abdomen.

Hyperammonemia can contribute to cerebral edema and therefore must be corrected aggressively. The US Food and Drug Administration (FDA) has not approved any medication for treatment of hyperammonemia specifically due to Reye syndrome. However, sodium phenylacetate–sodium benzoate is FDA-approved for the treatment of acute hyperammonemia and associated encephalopathy in patients with deficiencies in enzymes of the urea cycle.

Administer ondansetron 1-2 mg IV during the first 15 minutes of the initial dose of sodium phenylacetate–sodium benzoate. If the ammonia level is higher than 500 µg/dL or if the patient’s condition fails to respond to the initial dose of sodium phenylacetate–sodium benzoate, start dialysis, preferably hemodialysis. (See Inborn Errors of Metabolism.)

Prevent increased ICP. Elevate the head to 30°, keep the head in a midline orientation, use isotonic rather than hypotonic fluids, avoid overhydration, and administer furosemide 1 mg/kg as often as every 4-6 hours to control fluid overload.


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