Reye’s syndrome is a biphasic disease that rapidly causes brain encephalopathy among infants and children. It was named after by Australian pathologist Douglas Reyes who first described the clinical and pathological features of this syndrome in detail. It is characterized by the onset of viral-associated illness like a common cold, respiratory infection, and gastrointestinal infection. After resolving completely, again the disease reoccurs with encephalopathy variable severity such as vomiting, mental confusion, seizure, a neurological problem like delirium and sometimes it may lead to coma and death. The symptoms of this disease characteristically appear within a week i.e., after the onset of a viral fever. This disease infrequently occurred during 1950’s and considered as a rare disease. But later in 60’s and 70’s the case becomes complicated as the infection is associated with the virus and caused death in childhood.
What is the Cause of Reye’s Syndrome?
Many death cases reported and those who survived have severe complications such as brain injury. Mannitol decreased the brain injury and treatment was effective. The cause of the disease is still not yet known. Reye’s syndrome affected children under age 10 and mostly 90% of the cases associated with the use of aspirin. Because of adequate aspirin doses over sufficient time caused a hepatotoxic effect. Live injury showed a significant increase in aminotransferase and alanine transferase enzyme. As a result, the mitochondrial dysfunction occurs which initiated the viral infection in children. The mitochondria are the key energy source in the cell. The dysfunction of the cell caused a block in Na, K, and water transport and major defects observed in the urea cycle. Fat deposition, cerebral edema, and encephalopathy are the manifestation of the Reye’s syndrome.
Exogenous Medication
The two toxins most commonly associated with Reye’s syndrome. One is aspirin and the second is an emulsifying agent used in Spruce budworm spray. It was the organophosphate insect repellent better known as fenitrothion in the market used worldwide. It was referred to as “poison mist” in an article called “the enemy above.’ It was proved using an animal model that this emulsifier inhibited the immunity cells. Many published referring numerous agent associated hepatotoxic by affirming solid evidence such as acetylsalicylic acid, acetaminophen, and many non-steroidal anti-inflammatory analgesics.
Antecedent Viral Infections and Genetic Risk
Influenza A, B, chicken pox are some of the viral illness found in Reye’s syndrome. A vast range of viruses such as adenoviruses, cytomegalovirus, Epstein-Barr virus, mumps, Coxsackie A and B, dengue, herpes simplex, respiratory syncytial virus, parainfluenza, rubella, polio, and echoviruses also reported being associated with Reye’s syndrome.
Dual viral infections are also thought to be important in its causation. It has been considered as a seasonal disease because of its high incidence viral infection during winter and spring. In those days diagnosis and awareness of the disease outbreaks are poor and hence this disease considered as a rural and semi-urban disease. Many cases identified within families members and the medical review says genetic inborn errors of metabolism are associated with risk. Fatty acid oxidation disorder is a genetic disease where person unable to metabolize fatty acid is also associated with Reye’s syndrome.
Initial Signs and Symptoms
Reye’s syndrome described into five stages based on the signs and symptoms. Stage 5 is the most dangerous where victims show multiorgan failure, coma, and death occurs. Infants show rapid breathing and diarrhea is common under age 3. Among children and teenagers, persistent vomiting, insomnia, fatigue, short-tempered, violent behavior, misperception, disorientation, hallucinations, and paralysis in the arms and legs are seen. Swelling occurs in the brain which causes the seizures. Early identification and hospitalization help the victims from death.
Also Read:
- Reyes (Reye) Syndrome: Causes, Symptoms, Treatment, Risk Factors, Complications
- How Can Aspirin Cause Reye’s syndrome?