Dr Razaq Dawar
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Reye Syndrome
Reye syndrome is a rare but life-threatening disorder characterized by:
• Acute encephalopathy (brain dysfunction/swelling)
• Fatty degeneration of the liver
It mainly affects children and teenagers, usually after a viral illness such as influenza or chickenpox.
Cause and Pathogenesis
Reye syndrome is strongly associated with the use of Aspirin during viral infections in children.
The exact mechanism is not fully understood, but aspirin may damage mitochondria, leading to:
• Impaired fatty acid metabolism
• Fat accumulation in the liver
• Increased ammonia levels
• Cerebral edema (brain swelling)
Common Triggering Viral Infections
• Influenza
• Varicella (chickenpox)
• Viral upper respiratory infections
Clinical Features
Symptoms usually begin a few days after the viral illness.
Early Symptoms
• Persistent vomiting
• Nausea
• Weakness
• Irritability
Neurological Symptoms
• Confusion
• Agitation
• Personality changes
• Drowsiness
• Seizures
• Coma in severe cases
Investigations
Typical findings include:
• Elevated AST and ALT
• Increased serum ammonia
• Hypoglycemia
• Prolonged PT/INR
• Metabolic acidosis
CSF
Usually normal, helping differentiate it from meningitis or encephalitis.
Liver Biopsy
Shows microvesicular fatty change without inflammation.
Differential Diagnosis
• Meningitis
• Encephalitis
• Poisoning
• Inborn errors of metabolism
• Hepatic failure
Management
There is no specific cure; treatment is mainly supportive in ICU.
Supportive Treatment
• Maintain airway and ventilation
• IV fluids and glucose
• Correct electrolyte imbalance
• Control raised intracranial pressure
• Treat seizures
Drugs Used
• Mannitol for cerebral edema
• Anticonvulsants if seizures occur
Complications
• Brain herniation
• Permanent neurological damage
• Respiratory failure
• Death
Prevention
Children with viral infections should avoid Aspirin unless specifically prescribed for special conditions.
For fever control, safer alternatives include:
• Paracetamol
• Ibuprofen (in appropriate age groups)
Prognosis
Early diagnosis and aggressive supportive care improve outcomes. Severe neurological involvement is associated with poorer prognosis.
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