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At a glance

Possible life-threatening multifactorial syndrome resulting from a previous viral infection treated by aspirin and a genetic sensibility. Dehydration, intracranial hypertension, and liver insufficiency must be considered. Presentation includes seizures, vomiting, lethargy, irrational behavior, and delirium. Tachypnea, jaundice, fever, and coma suggest end-stage of disease. Reye Syndrome appears usually 3 to 5 days after the onset of chickenpox rash.

Synonyms

Fatty Liver with Encephalopathy; Reye-Johnson Syndrome.

History

First described by Douglas Reye, an Australian pathologist, in 1963.

Incidence

Rare (1:1,000,000 in the general population); incidence has decreased since 1993, but not been eradicated.

Genetic inheritance

There is no known genetic association with this condition.

Pathophysiology

Reye Syndrome is a two-phase illness because it is almost always associated with a previous viral infection, such as influenza, cold, or chickenpox. It tends to appear with greatest frequency during January, February, and March. It has been proposed that Reye Syndrome develops from the interaction of a viral illness, genetic susceptibility to the disease, and exposure to chemicals, such as salicylates, pesticides, and aflatoxin. Abnormal accumulations of fat begin to develop in the liver and other organs of the body, which can cause a severe increase of intracranial pressure. Unless diagnosed and treated successfully, death is common, often within a few days. Salicylates continue to be present in “herbal” remedies. A toxic synergism between salicylates and acetaminophen/paracetamol has been postulated.

Diagnosis

Syndrome occurs in children and teens (96%). Diagnosis is made by clinical signs (persistent or recurrent vomiting, listlessness, personality changes present during or soon after a viral illness) and by elimination of other diagnoses such as metabolic illness, meningitis, encephalitis, diabetes, drug overdose, sudden infant death, toxic ingestion, head trauma, renal or hepatic failure poisoning, that could imitate Reye Syndrome.

Clinical aspects

Persistent or continuous vomiting; signs of brain dysfunction; listlessness, loss of pep and energy; drowsiness can be observed first and then irritability, aggressive behavior, disorientation, confusion, irrational behavior delirium, convulsions, coma. Beyond these neurological signs, it is possible to observe respiratory disturbances such as hyperventilation. Anemic episodes and hypoglycemia are common.

Precautions before anesthesia

Evaluate liver function (echography, computed tomography [CT] scan, laboratory tests: coagulation, serum glutamic-oxaloacetic transferase [SGOT] and serum glutamic-pyruvic transaminase [SGPT] [increase generally], albumin); respiratory function (clinical, chest radiograph, arterial blood gas analysis); hydration status of the patient (clinical, electrolytes values, urea, creatinine); neurological function (clinical, CT).

Anesthetic considerations

Indication of anesthesia during the evolution of a Reye Syndrome has to be exceptional. Steady state should be raised before starting. Careful monitoring of respiratory function should be done (SpO2 [arterial oxyhemoglobin saturation], ETCO2 [end-tidal ...

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