Reye syndrome, named after Australian pediatrician Ralph Douglas Reye, is an acute metabolic disorder associated with damage to the brain and liver. Reye syndrome primarily affects children.
What is Reye syndrome?
Reye syndrome usually occurs as a result of a previous viral infection, specifically influenza or chickenpox. About a week after the actual illness subsides, there is severe and frequent vomiting and high fever. The children often seem restless, sometimes even hyperactive and easily irritable. Blood sugar levels may drop sharply. In severe and advanced cases, seizures, spasticity, clouding of consciousness and unconsciousness and even coma may occur. About two-thirds of patients with severe courses experience edema of the brain. In principle, Reye’s syndrome can occur at any age; however, children between the ages of four and twelve are most commonly affected. The disease occurs with indiscriminate frequency in girls and boys. Reye’s syndrome is an extremely rare disorder, but it can have serious consequences and absolutely requires medical treatment.
Causes
In Reye syndrome, damage occurs to the mitochondria, which in turn severely affects the metabolism of certain organs. As a result, there is hyperacidity of the body and accumulation of ammonia and fatty acids in the liver, which can lead to liver failure. The accumulation of ammonia can lead to the formation of cerebral edema. Although the exact cause of Reye’s syndrome is not known, it has been observed frequently in cases where patients with viral infections have been treated with acetylsalicylic acid-containing drugs. Since this association became known, a general decrease in Reye’s syndrome has been observed as a result of appropriately adjusted treatment recommendations. However, the exact relationship between acetylsalicylic acid and Reye’s syndrome has not yet been clarified; a genetic disposition is suspected. In children younger than two years of age who develop Reye syndrome, a congenital metabolic disorder is usually assumed to be the cause.
Symptoms, complaints, and signs
Reye syndrome is a functional disorder of the cellular apparatus. The disease mostly occurs only in children and adolescents. The liver and brain are mainly affected. Reye’s syndrome can take a fatal course. Investigations into the causes have not yet been completed. Scientists assume that the pathogens of influenza or chickenpox may be responsible, as well as certain drugs. Reye’s syndrome occurs in conjunction with viral infections that have subsided. The first sign is frequent vomiting. Nausea, on the other hand, does not occur. The young patients appear restless and confused, are powerless and hardly responsive. Seizures are also typical of the disease. It is not uncommon for patients to fall into a coma. The accumulation of fluid leads to increased intracranial pressure. This affects important nerve tracts. Likewise, fatty degeneration of the liver occurs. The accompanying dysfunction can lead to various metabolic impairments. One indication is hypoglycemia, for example. The skin often turns yellowish. After a blood sample has been taken, a prolonged blood clotting time will be observed. The symptomatology resembles blood poisoning or meningitis and is therefore, without a detailed examination, not easy to differentiate.
Diagnosis and course
Because Reye syndrome is extremely rare-about 50 cases are thought to occur annually in the United States-it is often not recognized. Also, symptoms are relatively nonspecific and are often misinterpreted and misdiagnosed as meningitis or inborn errors of metabolism. Diagnosis can be made on the basis of blood and urine tests. These reveal elevated liver enzymes, blood clotting disorders, elevated levels of ammonia, and often low blood glucose levels. To confirm the diagnosis, imaging techniques or a liver biopsy are used, which reveal changes in the mitochondria and fatty degeneration of the liver. Brain waves can be used to detect an increase in intracranial pressure.In addition, the previous intake of acetylsalicylic acid-containing medications in the case of a viral infection is important from a medical history point of view. If left untreated, Reye’s syndrome is a life-threatening disease that is fatal in about a quarter of cases. About 30% of patients suffer neurological sequelae such as speech or learning problems. Reye syndrome is not contagious.
Complications
Reye syndrome in itself is a very serious complication of viral infection and usually affects children between the ages of four and ten. In up to 50 percent of all cases, patients die from severe complications, particularly affecting the brain and liver. Unfortunately, there is no curative therapy. Treatment consists only of alleviating the severe symptoms to ensure the survival of those affected. In many cases where children have survived, they are left with neurological disorders that result from brain damage. Thus, paralysis, speech disorders or mental limitations may remain for life. The main feature of Reye syndrome is liver and brain damage. The liver develops into a fatty liver, which is severely limited in its function. Eventually, liver failure may even occur, which in severe cases may require liver transplantation. Since the functions of the liver and kidneys are closely linked, kidney damage and even kidney failure can also occur. At the same time, the brain is affected. The intracranial pressure increases due to fluid accumulation (cerebral edema). Cerebral edema is responsible for the most severe complications of Reye’s syndrome. In about 60 percent of affected children, the full-blown Reye’s syndrome develops, which is characterized not only by liver dysfunction but also by confusion, irritability, seizures, and impaired consciousness up to and including coma. Three-quarters of those with full-blown Reye syndrome do not survive the disease.
When should you see a doctor?
Reye syndrome should always be evaluated and treated by a physician. There is usually no self-healing with this disease. Because the condition is a genetic disease, it cannot be completely cured. However, the affected person is entitled to genetic counseling to ensure that Reye’s syndrome is not passed on to the next generation. A doctor should be consulted if the affected person suffers from severe nausea or even vomiting over a long period of time. Patients are often confused or barely responsive and are unable to communicate with other people. Persistent hypoglycemia may also indicate Reye’s syndrome and should be investigated. In the worst case, if Reye’s syndrome is left untreated, the affected person may suffer from blood poisoning or inflammation of the meninges, which can also be fatal. First and foremost, a general practitioner should be consulted in case of symptoms. Further treatment is provided by a specialist.
Treatment and therapy
There is no specific causative therapy for Reye’s syndrome. Treatment is limited to relief of acute symptoms and damage control with regard to severe progressions such as liver failure or coma. This requires intensive inpatient treatment under close medical supervision. The supply of fluids and nutrients can be ensured by means of a cannula. If necessary, the intracranial pressure is lowered by specific drugs. In some cases, artificial respiration of the patient is necessary. Reye’s syndrome is a medical emergency. If this condition is suspected, medical attention should be sought as soon as possible, as prompt medical intervention can prevent progression of the disease and thus reduce the risk of sequelae.
Prevention
That Reye’s syndrome has been observed in association with the administration of acetylsalicylic acid-containing medications, these drugs (eg, aspirin) should not be administered to children and adolescents with febrile illnesses, if possible. Other medications are available for fever reduction and pain control that are not associated with the risk of Reye’s syndrome. The pediatrician should be consulted about these.
Follow-up
Reye syndrome is considered incurable. Only symptomatic follow-up can be given to reduce the various symptoms and increase the chances of survival.Drug treatment is important to reduce the inflammation and swelling of the brain. In this case, elevation of the upper body is recommended as a supportive measure. Since liver damage limits its function, in severe cases it may be necessary to artificially support metabolism and blood clotting. This is done by administering sodium benzoate to lower the ammonia level in the blood and by peritoneal dialysis. In particularly severe cases, a liver transplant may be necessary. The kidney must also be treated with medication to maintain urine output and prevent kidney failure. Furthermore, the function of the remaining organs, such as the heart and lungs, must also be monitored, as artificial respiration may be necessary due to the damage to the brain. The disease has a variety of effects and can also affect other organs and cause permanent paralysis and speech disorders. Therefore, regular check-ups with a doctor are strongly advised to check the progress of Reye’s syndrome. Unfortunately, the prognosis is rather poor. More than half of those affected die and the survivors have lifelong severe neurological impairments. However, early detection of the disease and subsequent therapy can increase the chances of survival.
Here’s what you can do yourself
Reye’s syndrome is a medical emergency. If signs of the acute metabolic disorder appear, call 911 immediately. Until medical help arrives, the child or adolescent should be sedated. Parents should reassure the affected person and ensure that he or she does not lose consciousness. If the disorder is congenital, the emergency physician must be informed. If necessary, appropriate emergency medication should be administered. After initial treatment, the sufferer must be cared for in hospital. Accompanying rest and sparing apply. In addition, the causes of the acute metabolic disorder should be determined. Since the condition occurs predominantly in children, a consultation with the pediatrician is necessary. The physician can carry out suitable regular progress checks and explain the disease to the child in an age-appropriate manner. After a medical emergency, secondary damage to the liver and brain may occur. In addition, neurological disorders may remain, which, accompanied by physiotherapy, must be treated by regular exercise. The affected child must not come into contact with possible triggers. Parents should review and, if necessary, change medication to prevent possible triggers such as acetylsalicylic acid from entering the body. In cases of inborn errors of metabolism, the use of appropriate medications should also be avoided.
