![]() Aspirin is a medicine you can buy at stores such as Wal-Mart, CVS, Walgreens, and even Dollar General. It's revered as one of the most effective and cheap drugs to bring down fever and a range of pain, not including the heartache of breaking up with your partner. Aspirin is the cause of a rare and near-fatal disease: Reye-Johnson, also called Reye's Syndrome, also called RS. September is Reyes awareness month, observed to spread awareness of this terrifying disease and remind parents not to give themselves or their children aspirin to relieve pain or fever while suffering from a viral infection. The exact mechanism or the cause of RS is unknown. However, a study showed a significant cause-effect relationship between aspirin intake and RS in children. A review reports that death happens in approximately 30%- 40% of cases. Children 5 to 14 years of age, recovering from viral infections such as influenza and chicken pox, are more susceptible to RS. However, there are reported cases of children less than one year, teens, and adults with RS. History of Reye's Syndrome Douglas Reye, an Australian pathologist, and colleagues first documented RS in 1963 as an encephalopathy with fatty degeneration associated with an 80% mortality rate. In his study, Reye described 21 children who presented with symptoms such as vomiting, rapid breathing, reduced blood sugar levels, and elevated liver enzymes. Seventeen out of the 21 children died within three days of disease progression. In 1965, suggestions that aspirin hypersensitivity could lead to RS were put forward by physicians. To date, the exact cause is still unknown.In the United States, RS surveillance commenced in the early 1970s leading to strict warnings against aspirin use in children.
The number of cases reported worldwide peaked between the 1970s and 1980s. In 1977, 454 cases of RS were reported in the United States. The increase in RS incidence rates correlated with a peak in influenza B and chicken pox viral epidemics. Approximately 555 cases of RS were documented in the U.S in 1980. In 1986 warnings were issued against aspirin, after which the number of cases fell from 0.63 per 100,000. The number of cases dropped to an average of 36 per year between 1987 and 1993. Features and Symptoms of Reye's Syndrome The symptoms of RS develop after recovery from a viral illness of the upper respiratory tract and, in some cases, gastroenteritis. There are five stages of clinical progression in RS. The first stage involves severe vomiting, lethargy, confusion, and drowsiness. Patients are in a nearly unconscious state or stupor during the second stage. Rapid breathing, lack of response to stimuli, and sluggish pupils are other common symptoms during the second stage. By the third stage, the patient's consciousness is more depressed, also called obtundation. The patient enters into a deep coma in the 4th stage, followed by death in RS's fifth and final stages. Mechanism of Reye's Syndrome As early as 1983, studies showed that the primary cause of RS is damage to liver mitochondrial structure and function. In RS, the liver shows reduced mitochondrial numbers. Liver biopsies show enlarged liver, high-fat content, and changes in mitochondrial structure. The high-fat content could be attributed to dysregulation of the breakdown of fatty acids and increased uptake of fatty acids by the liver. Similar observations were documented for brain biopsies, with swelling of the brain. The swelling in the brain leads to increased pressure in the skull or intracranial pressure that alters the amount of oxygen delivered to cerebral tissue. Biochemical characterization showed RS defects in the urea cycle, glucogenesis, and fatty acid oxidation pathways. The defective pathways could lead to the accumulation of high levels of neurotoxins such as ammonia and fatty acids leading to encephalopathy. High ammonia levels increase brain lactic acid production in RS, leading to brain swelling. Survivors with elevated ammonia levels usually have neurological deficits that last for the rest of their lives. These complications include poor attention span, memory loss, hearing, vision, and motor skills, and speaking difficulties. A study showed that aspirin increases mitochondrial fatty acid oxidation due to mitochondrial injury and defective respiration. Diagnosis and Treatment Although RS progress is rapid and fatal, there are a few ways to diagnose it. Diagnosis is not always specific, as other diseases show similar symptoms. Testing usually begins with the analysis of blood and urine and tests for fatty acid oxidation disorders and metabolic disorders. Invasive diagnostic tests include a spinal tap or lumbar puncture, liver biopsy, CT scan or MRI, and skin biopsy to test for fatty acid disorders. Current treatment focuses on managing symptoms such as administering glucose and electrolyte solutions, medications that lower intracranial pressure, and vitamin K to reduce bleeding from liver damage. Awareness Although the number of cases has significantly reduced since 1963, educating parents and caregivers about the detrimental effects of aspirin use and RS is essential. In 1974, the National Reyes Syndrome Foundation was established to eliminate RS cases worldwide by spreading awareness. The foundation supports research and serves families and individuals dealing with RS. They advocate for the safe use of aspirin in schools and raise awareness through health fairs, newsletters, and student projects. Comments are closed.
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