How is stage 3-5 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

Continuously monitor ICP, central venous pressure, arterial pressure, or end-tidal carbon dioxide. Perform endotracheal intubation if the patient is not already intubated.

Treat increased ICP by following standard guidelines, which, in addition to correction of hyperammonemia, proper positioning of the head, and appropriate fluid management (see above), include the following:

  • Ventilation to maintain the partial pressure of carbon dioxide in the normal range

  • Aggressive management of fever to prevent the increased cerebral metabolism and increased cerebral blood flow resulting from hyperpyrexia

  • Analgesia and sedation to alleviate agitation or prepare for painful interventions

  • Paralytic agents to control shivering

  • If other measures fail, mannitol 20% solution dosed at 0.25-0.5 g/kg IV infused over 10-20 minutes as often as every 6-8 hours, [10] or hypertonic saline 3% dosed at 3-5 mL/kg over 3-30 minutes [11] (For additional information, see Mannitol and ICP Monitors.)

  • Induced barbiturate coma and hypothermia are controversial

Treat seizures with phenytoin 10-20 mg/kg IV as a loading dose, followed by 5 mg/kg/day IV divided every 6 hours or fosphenytoin dosed as 10-20 mg/kg phenytoin equivalents.

Correct coagulopathy (prothrombin time >16 seconds). The data on treatment of coagulopathy in Reye syndrome, like those on most etiologies of coagulopathy in children, are limited. Options include fresh frozen plasma (FFP), cryoprecipitate, platelets, vitamin K, and exchange transfusion.

FFP 10-15 mL/kg every 12-24 hours provides rapid correction and volume expansion and should be administered, particularly if active bleeding is present or if invasive procedures (eg, ICP monitoring device placement or liver biopsy) are required. If the fibrinogen level is lower than 100 mg/dL, cryoprecipitate 10 mL/kg every 6 hours should be considered instead of FFP because cryoprecipitate has a higher concentration of fibrinogen.

If invasive procedures are to be performed, platelets should also be given as needed to restore the platelet count to a value higher than 50,000/µL. Vitamin K 1-10 mg IV may be administered instead of FFP or cryoprecipitate if the need for correction is not an emergency. Exchange transfusion is rarely required. (See Consumption Coagulopathy.)

Reye syndrome has been successfully treated with liver transplantation. [12]


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