Reye Syndrome

Reye Syndrome

Reye ‘s syndrome, named after the Australian pediatrician Ralph Douglas Reye, is an acute metabolic disease that is associated with damage to the brain and liver. Reye’s syndrome primarily affects children.

What is Reye Syndrome?

Reye’s syndrome always occurs as a result of a previous viral infection, particularly influenza or chickenpox. About a week after the actual illness has subsided, severe and frequent vomiting and a high fever occur. For about globule cell anemia, please visit bittranslators.com.

The children often appear restless, sometimes even hyperactive and easily irritable. The blood sugar level can drop sharply. In severe and advanced cases, seizures, spasticity, clouding of consciousness and unconsciousness up to coma can occur. Around two-thirds of patients with severe forms suffer edema in the brain.

In principle, Reye’s syndrome can occur at any age; however, children between the ages of four and twelve are mostly affected. The disease occurs equally often in girls and boys. Reye’s syndrome is an extremely rare condition, but it can have serious consequences and medical treatment is essential.

Causes

Reye’s syndrome causes damage to the mitochondria, which in turn severely affects the metabolism of certain organs. As a result, the body becomes acidic and ammonia and fatty acids accumulate 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 more frequently in cases where patients with viral infections have been treated with drugs containing acetylsalicylic acid. Since this connection became known, a general decline in Reye’s syndrome has been recorded through appropriately adapted therapy recommendations.

However, the exact connection between acetylsalicylic acid and Reye’s syndrome has not yet been clarified; a genetic disposition is suspected. In children under the age of two who develop Reye’s syndrome, a congenital metabolic disorder is usually assumed to be the cause.

Symptoms, Ailments & Signs

Reye’s syndrome is a functional disorder of the cellular apparatus. The disease usually occurs only in children and adolescents. Mainly the liver and the brain are attacked. Reye’s syndrome can be fatal. Investigations into the causes are not yet complete.

Scientists assume that the causative agents of influenza or chickenpox can be responsible as well as certain drugs. Reye’s syndrome occurs in association with resolving viral infections. The first sign of this is frequent vomiting. Nausea, on the other hand, does not occur. The small 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 pathways. Fatty liver also occurs.

The associated dysfunction can lead to various impairments of the metabolism. One indication is hypoglycaemia, for example. The skin often turns yellowish. After a blood draw, a prolonged blood clotting time will be noticed. The symptoms are similar to blood poisoning or meningitis and are therefore not easy to distinguish without thorough examination.

Diagnosis & History

Since Reye’s syndrome is extremely rare – in the USA it is assumed that there are around 50 cases a year – it often goes undetected. The symptoms are also relatively unspecific and are often misinterpreted and misdiagnosed as meningitis or congenital metabolic disorders.

Diagnosis can be made using blood and urine tests. Elevated liver enzymes, blood clotting disorders, an increased concentration of ammonia and often a blood sugar level that is too low are found here. Imaging methods or a liver biopsy are used to confirm the diagnosis, in which changes in the mitochondria and fatty degeneration of the liver are detected.

The increase in intracranial pressure can be detected on the basis of the brain waves. The previous intake of drugs containing acetylsalicylic acid in the case of a viral infection is also important in terms of the anamnestic history.

If left untreated, Reye’s syndrome is a life-threatening disease that ends fatally in around a quarter of cases. About 30% of patients suffer neurological sequelae such as language or learning problems. Reye’s syndrome is not contagious.

Complications

Reye’s syndrome 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, the patients die from serious complications that particularly affect the brain and liver. Unfortunately, there is no curative therapy.

Treatment consists simply of relieving the severe symptoms to ensure survival of those affected. In many cases where the children have survived, neurological disorders resulting from brain damage remain. Paralysis, speech disorders or mental limitations can remain for life. The main features of Reye’s syndrome are liver and brain damage. The liver develops into a fatty liver, which is severely restricted in its function.

Finally, liver failure can even occur, which in severe cases also makes a liver transplant necessary. Since the functions of the liver and kidneys are closely linked, kidney damage and even kidney failure can occur. At the same time, the brain is affected. The intracranial pressure increases due to fluid accumulation (cerebral oedema).

Cerebral edema is responsible for the most serious complications of Reye’s syndrome. Reye’s syndrome develops in around 60 percent of affected children, which, in addition to liver dysfunction, is also characterized by confusion, irritability, seizures and disturbances of consciousness up to and including coma. Three-fourths of people with full-blown Reye syndrome do not survive the disease.

When should you go to the doctor?

Reye’s syndrome should always be evaluated and treated by a doctor. This disease usually does not heal itself. Since the disease is a genetic disease, it cannot be completely cured. However, those affected are also entitled to genetic counseling so that the Reye syndrome is not passed on to the next generation.

A doctor should be consulted if the person concerned suffers from severe nausea or even vomiting over a longer period of time. The patients are often confused or hardly responsive and cannot communicate with other people. Permanent low blood sugar can also indicate Reye’s syndrome and should be examined. In the worst case, the person affected with untreated Reye’s syndrome can suffer from blood poisoning or inflammation of the meninges, which can also be fatal. In the first place, a general practitioner should be consulted in the event of symptoms. Further treatment is carried out by a specialist.

Treatment & Therapy

There is no specific, causal therapy for Reye syndrome. Treatment is limited to relieving the acute symptoms and limiting the damage with regard to severe forms such as liver failure or coma.

This requires intensive medical inpatient treatment under close medical supervision. A cannula can be used to ensure the supply of liquid and nutrients. If necessary, the intracranial pressure is lowered with specific medication. In some cases, artificial respiration of the patient is necessary.

Reye’s syndrome is a medical emergency. If this disease is suspected, medical help should be sought as soon as possible, since timely medical intervention can prevent the disease from progressing and thus reduce the risk of subsequent damage.

Prevention

Since Reye ‘s syndrome has been observed in connection with the administration of drugs containing acetylsalicylic acid, these drugs (e.g. aspirin) should not be administered to children and adolescents with febrile illnesses if possible. There are other drugs available to reduce fever and relieve pain that do not carry the risk of Reye’s syndrome. The pediatrician should be consulted for this.

Aftercare

Reye’s syndrome is considered incurable. Follow-up care can only be symptomatic to reduce the various symptoms and increase the chances of survival. Drug treatment is important to reduce inflammation and swelling in the brain. Elevated upper body support is recommended here.

Since the liver damage impairs its function, it may be necessary in severe cases to artificially support the metabolism and blood clotting. This is done by giving sodium benzoate to lower the level of ammonia in the blood and by peritoneal dialysis. In particularly severe cases, a liver transplant may be necessary.

The kidneys also require 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, since artificial respiration may be necessary due to damage to the brain.

The disease has a variety of effects and can also affect other organs and cause permanent paralysis and speech disorders. Regular check-ups with the doctor are therefore urgently recommended in order to check the course of Reye’s syndrome.

Unfortunately, the prognosis is rather poor. Over half of those affected die, and those who survive have lifelong severe neurological impairment. However, early detection of the disease and subsequent therapy can increase the chances of survival.

You can do that yourself

Reye’s syndrome is a medical emergency. If signs of the acute metabolic disorder appear, the emergency doctor must be called immediately. The child or young person must be sedated until medical help arrives. Parents should reassure the victim and make sure they do not lose consciousness. If it is a congenital disorder, the emergency doctor must be informed. Appropriate emergency medication should be administered if necessary.

After the initial treatment, the patient has to be treated in the hospital. This is accompanied by rest and protection. In addition, the causes of the acute metabolic disorder should be determined. Since the condition occurs mainly in children, a discussion with the pediatrician is necessary. The doctor can carry out appropriate regular follow-up checks and inform the child about the disease in an age-appropriate manner.

After a medical emergency, there can be consequential damage to the liver and brain. In addition, neurological disorders can remain, which, in addition to physiotherapy, must be treated with regular exercise. The affected child must not come into contact with possible triggers. Parents should check the medication and change it if necessary so that possible triggers such as acetylsalicylic acid do not enter the body. In the case of congenital metabolic disorders, the use of corresponding drugs should also be avoided.

Reye Syndrome