Reye syndrome
Background
- Potentially fatal syndrome
- Associated with aspirin use during viral illness, especially influenza, varicella
- Multiorgan system disease, particularly effects brain and liver
- Classic early symptoms: rash, vomiting, liver damage (without jaundice typically)[1]
Clinical Features
- Stage I
- Stage II
- Stupor
- Hyperventilation
- Fatty liver (found by biopsy)
- Stage III
- Possible coma
- Possible cerebral edema
- Rarely, respiratory arrest
- Stage IV
- Deepening coma
- Dilated pupils with minimal response to light
- Minimal hepatic dysfunction
- Stage V
- Deep coma
- Seizures
- Multisystem organ failure[7]
- Flaccidity
- Hyperammonemia (above 300mg/dL of blood)
- Death
Differential Diagnosis
- Shaken baby syndrome
- Head trauma
- Viral encephalitis
- Meningitis
- Drug overdose or poisoning
- Inborn errors of metabolism
Evaluation
- CBC
- Chem 10
- ABG
- LFTs
- Ammonemia
- Acetaminophen level (rule out coingestion/alternate cause of liver failure)
- ASA level (rule out overdose)
- Fingerstick (can be hypoglycemic)
- UDS
- LP
- ECG
- EEG (coma)
Management
ABCs
- Consider intubation if stage II or higher
- Ensure IV access with fluid resus
- Monitor neuro status closely, treat signs of cerebral edema aggressively
Consultations
- Consider NSG Consult for cerebral edema
- Consider GI consult for liver biopsy
- Consider metabolic disorders
Also See
References
- ↑ Suchy, FJ, el al.; Sokol, RJ; Balistreri, WF (2007). Liver Disease in Children. Cambridge: Cambridge University Press. ISBN 0-521-85657-4.