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Patient handout

Reye syndrome

PRODUCTION

1. Your condition

This handout is for reye syndrome. Your care team identified this based on: intractable vomiting + altered ms in child / adolescent 3-7 days after viral illness (especially influenza b / varicella) (belay nejm 1999 pmid 10228187).

Other reasons your team may use this plan: progressive encephalopathy + behavior change + lethargy in child post-viral (belay nejm 1999); aspirin or salicylate-containing medication (pepto-bismol, oil of wintergreen) given during viral illness in child < 19 yr (belay nejm 1999); elevated ast/alt > 3× uln + ammonia > 1.5× uln + minimal jaundice in child — reye-syndrome differential (cdc surveillance criteria).

2. Your medications

Take these medications exactly as prescribed. Do not stop or change a dose without talking to your provider.

MedicationStarting doseHowWhenWhat it does
PICU admission + early transfer to pediatric liver transplant centerMandatory PICU + early transfer to facility with pediatric liver transplant capability; involve pediatric hepatology, metabolism, genetics, neurology, neurosurgery, transplant teamsBelay NEJM 1999 PMID 10228187 — 31% case-fatality even with treatment; early transplant-center transfer is mandatory
IEM workup in parallel with treatmentPlasma amino acids + acylcarnitine profile + urine organic acids + acylglycines + carnitine + VLCFA + biotinidase; consult metabolism / geneticsBelay NEJM 1999 — since 1980 aspirin warning, IEM mimics far more likely than true Reye; do not delay workup
mannitol0.25-1 g/kg IV q4-6h PRN for ICP crisisIVPRN q4-6hICS pediatric severe TBI consensus — hyperosmolar therapy for cerebral edema in PALF / Reye / metabolic encephalopathy (AAP Red Book 2024-2027, Lexicomp Peds)
hypertonic saline 3% NaCl3-5 mL/kg IV bolus over 10-15 min for ICP crisis; may follow with continuous infusion 0.1-1 mL/kg/h titrated to serum Na 145-155 mEq/LIVbolus + infusionICS pediatric severe TBI consensus — preferred over mannitol when hypovolemic / hypotensive (Lexicomp Peds; AAP Red Book 2024-2027) — listed as non_pharm because 3% NaCl is a compounded concentration not a single RxCUI
glucoseD10W or D25W 5 mL/kg IV bolus for hypoglycemia, then D10W continuous infusion GIR 6-8 mg/kg/min titrated to glucose 100-200 mg/dLIVbolus then continuousReverses hypoglycemia + suppresses lipolysis in suspected FAO defects (e.g., MCAD) — D10W at GIR 6-8 mg/kg/min is the standard metabolic-crisis backbone (AAP Red Book 2024-2027, Lexicomp Peds)
phytonadione (vitamin K1)0.3 mg/kg IV/IM/SC slow IV (max 10 mg single dose); for vitamin-K-dependent coagulopathy from hepatic synthetic dysfunctionIV/IM/SCsingle dose; may repeat per INRReverses vitamin-K-dependent coagulopathy from hepatic synthetic dysfunction; slow IV due to anaphylactoid risk (AAP Red Book 2024-2027, Lexicomp Peds)
fresh frozen plasma + cryoprecipitateFFP 10-15 mL/kg IV for INR > 1.5 with bleeding OR pre-procedure; cryoprecipitate 1 unit / 5-10 kg for fibrinogen < 100IVper coagulopathy + bleedingAAP transfusion guidance — blood-product reversal for active bleeding with hepatic synthetic dysfunction
lactulose1 mL/kg PO/PR q2-4h titrated to 2-3 soft stools/dayPO/PRq2-4h titratedReduces serum ammonia via gut acidification + ammonia trapping; first-line for hepatic encephalopathy (AAP Red Book 2024-2027, Lexicomp Peds)
lorazepam0.1 mg/kg IV (max 4 mg per dose)IVq5 min x 2AES 2016 Glauser PMID 26900382 — first-line acute seizure abortive; routes to peds.status_epilepticus.v1 if recurrent
levetiracetam20-40 mg/kg IV load over 15 min, then 30-60 mg/kg/day divided BIDIV/POBID after loadAES 2016 Glauser PMID 26900382 — levetiracetam preferred (no hepatic toxicity); AVOID valproate (worsens mitochondrial dysfunction + hepatotoxicity); AVOID phenytoin (hepatic enzyme inducer) (AAP Red Book 2024-2027, Lexicomp Peds)

Plan: Reye syndrome — supportive ICU bundle (NO specific antidote) (Belay NEJM 1999 PMID 10228187)

4. When to seek emergency care

Call 911 or go to the nearest emergency room right away if you have:

  • Aspirin or salicylate-containing medication (Pepto-Bismol, oil of wintergreen) given to child < 19 yr during viral illness (influenza, varicella) — sentinel risk feature for Reye syndrome (Belay NEJM 1999 PMID 10228187; CDC)
  • Lovejoy/Hurwitz stages 3-5: stage 3 obtunded + decorticate posturing; stage 4 deepening coma + decerebrate + brainstem dysfunction; stage 5 flaccid + apneic + areflexic + isoelectric EEG (Belay NEJM 1999)(life-threatening)
  • Ammonia > 45 mcg/dL (~ 26 mcmol/L) — relative risk 3.4 for death in Reye syndrome (Belay NEJM 1999)
  • Hypoglycemia (glucose < 60 mg/dL infant or < 70 mg/dL child) in suspected Reye / Reye-like illness (Belay NEJM 1999; AAP)
  • Any Reye-like illness in 2020s — IEM mimics (FAO defects, urea cycle disorders, organic acidemias) far more common than true Reye syndrome since 1980 aspirin warning (Belay NEJM 1999)
  • Seizure in Reye syndrome — cerebral edema, hypoglycemia, hyperammonemia, electrolyte derangement; routes to peds.status_epilepticus.v1 if refractory (AES 2016 Glauser PMID 26900382)
  • Coagulopathy with INR > 1.5 in suspected Reye syndrome (hepatic synthetic dysfunction) (Belay NEJM 1999)
  • King's College criteria met OR PALF criteria met for liver transplant listing in Reye / IEM-induced ALF (Belay NEJM 1999; PALF cohort)(life-threatening)

5. Follow-up

Pediatric hepatology + neurology (cognitive impairment in significant fraction of survivors per Belay 1999) + genetics + metabolism (lifetime IEM monitoring even if initial workup negative) + family education (NEVER give aspirin to children < 19 yr unless specifically prescribed) + school re-entry plan (Belay NEJM 1999; CDC)

6. Sources

Guideline: Belay NEJM 1999 PMID 10228187 (CDC surveillance Reye syndrome 1981-1997) + CDC clinical case definition + AES 2016 Glauser PMID 26900382 (status epilepticus cross-reference) + ICS pediatric severe TBI consensus + PALS 2020

  1. pubmed.ncbi.nlm.nih.gov/10228187
  2. pubmed.ncbi.nlm.nih.gov/26900382