Disease Description (Causes and Symptoms)
Reye syndrome is an acute disease that occurs mainly in children shortly after viral infections such as influenza or chickenpox. It is characterized by hepatic microvesicular steatosis (fatty liver) and acute encephalopathy (cerebral edema). Although the exact mechanism has not been fully established, it is thought to be associated with metabolic abnormalities caused by mitochondrial dysfunction. Major symptoms include severe vomiting, altered consciousness (drowsiness and stupor → coma), confusion and hallucinations, agitation, and seizures (convulsions). If the condition progresses rapidly, it can be fatal due to liver failure and cerebral edema within hours to several days. Tests commonly show abnormalities in liver function and coagulation, including hypoglycemia, elevated blood ammonia, elevated AST/ALT, and prolonged prothrombin time. Cerebrospinal fluid often shows no signs of inflammation, which can help distinguish it from encephalitis.
Treatment Methods and Precautions
- Basic principles: There is no specific treatment, so intensive supportive care (symptomatic treatment) is essential. Rapid management at the intensive care unit level is required.
- Acute treatment
- Correction of hypoglycemia (intravenous glucose), and correction of electrolyte and acid-base imbalances.
- Management of increased intracranial pressure: head elevation, oxygen administration, short-term mechanical ventilation and assisted ventilation (if necessary), administration of hypertonic solutions (hyperosmolar saline) or mannitol, and intracranial pressure monitoring when necessary.
- Seizure control: administration of anticonvulsants.
- Correction of coagulation abnormalities: vitamin K, fresh frozen plasma (FFP), etc.
- Supportive treatment for organ failure such as liver failure and kidney failure (hemodialysis, liver support, etc.); evaluation for liver transplantation may be considered in acute end-stage liver failure.
- Medications and antipyretics
- Aspirin (salicylates) and salicylate-based medications are contraindicated in children because they are associated with the risk of Reye syndrome. Acetaminophen (antipyretic analgesic) should be used to relieve fever and pain in children (under a doctor’s instructions).
- Follow-up and rehabilitation
- Neurological follow-up examinations and rehabilitation are necessary because neurological sequelae may remain even after recovery.
- Risk of complications: Death caused by cerebral edema, permanent neurological damage, bleeding tendency (DIC), multiple organ failure, etc.
- Preventive recommendation: Do not use aspirin in children with acute viral illnesses (especially during influenza and chickenpox outbreaks).
Checklist Before an Outpatient Visit (Items to Bring and Things to Confirm)
- Items to bring
- A list of currently taken medications (including prescription drugs, over-the-counter drugs, and antipyretics) — clearly indicate whether aspirin or salicylate-based medications are being taken.
- A record of any recent fever, rash, chickenpox, or influenza diagnosis and when the symptoms began.
- A record of symptom changes: number of vomiting episodes, level of consciousness (drowsiness, stupor, confusion), and whether seizures occurred and when.
- Medical history: personal and family history of liver disease and metabolic disease, and vaccination status (chickenpox and influenza vaccinations).
- Recent test results, if available — blood tests, liver function tests, coagulation tests, imaging findings, etc.
- Emergency guidance before an outpatient visit (warning signs)
- Persistent or recurrent vomiting, decreased level of consciousness (reduced responsiveness), convulsions or difficulty breathing, or rapidly progressing coma require an immediate visit to the emergency room.
Examples of General Questions to Ask the Doctor
- What treatment plan is recommended for the child’s current condition (including the need for hospitalization or intensive care)?
- Which test values (liver enzymes, ammonia, blood glucose, coagulation values) are warning signs?
- What medications can be used, and what are the side effects of each?
- What is the schedule for follow-up tests after recovery, and what is the long-term prognosis (including the possibility of neurological sequelae)?
- Which medication should be used for reducing fever and relieving pain at home, and which medications must be avoided entirely?
Examples of Disease-Specific Questions
- It is certain that the child recently took aspirin or a salicylate-based medication. In that case, how will emergency testing and treatment proceed?
- Among the test results (e.g., ammonia, AST/ALT, blood glucose), which values are the key indicators for determining severity?