Reye’s syndrome – causes, diagnostics, intensive treatment, how to reduce the risk

Proven content

Reye’s syndrome is a very rare but extremely serious disease, affecting mainly children between 4 and 12 years of age, although it also occurs in newborns and adults. Therefore, it can meet anyone, regardless of age, with the same probability as men and women. Often the cause is incorrect treatment of flu and colds in children.

More frequent occurrence of this syndrome has been reported in siblings, twins and children from married couples who are closely related spouses. Children get sick most often in the autumn-winter and winter-spring periods, because then they are more likely to suffer from viral infections of the respiratory and gastrointestinal tract. Most often, the disease is a complication of a viral infection treated with acetylsalicylic acid, which is found in many preparations with various trade names, the most popular of which is aspirin. The risk of Reye’s syndrome increases as you give your child aspirin to lower the fever. Then, the mitochondria are severely damaged, most likely due to the disturbance of the metabolism of cells infected with the virus. The child develops non-inflammatory encephalopathy (brain damage) and liver failure.

The syndrome was first described in 1963 in Australia by R. Douglas Reye, who presented a description of 21 children with encephalopathy and steatosis of internal organs. Previously, it was classified as meningitis or drug poisoning. In the 70s, the incidence of Reye’s syndrome was high, ranging from 0,8 to 6 / 100 annually. people of the study population. Since then, a progressive decline in the incidence has been observed, most likely due to the less frequent use of aspirin in children.

Of unknown cause

Possible causes of the disease:

1. Viral infections:

1. influenza, parainfluenza, herpes, shingles, rubella, Coxsackie, CMV, EBV, adenovirus, rhinoviruses

2. Drugs:

1. aspirin – its relationship with the development of Reye’s disease has been confirmed in several epidemiological studies conducted in various parts of the world. These studies showed that Reye’s syndrome developed in <0,1% of children using aspirin, and it was observed that aspirin was used before in 80% of patients with Reye's syndrome. The results of these studies changed the recommendations for the use of aspirin in children
2. valproic acid used to treat epilepsy
3. antiemetics, mainly phenothiazine

3. Other:

1. insecticides
2. pesticides
3. color
4. aflatoxins from fungi
5. Margosa oil

The literature also describes congenital disorders of metabolism, the clinical picture of which is similar to Reye’s syndrome. They include, among others fatty acid oxidation defects, urea cycle disorders, carbohydrate metabolism disorders or organic acidosis.

It can be life threatening

The course of Reye’s syndrome is two-phase.

Phase I.

Symptoms of upper respiratory tract infection, symptoms of chickenpox or rubella (mainly in older children). Gastrointestinal disturbances (most often in infants) may also occur.

Phase II.

Rapid vomiting from the 3rd to the 6th day, then symptoms of various degrees of damage to the nervous system. This may include excitement, delirium, convulsions, and coma. Muscle tension is usually increased and manifests itself in an arched back bend or neck stiffness. You may experience breathing difficulties and an increase in heart rate. Increasing intracranial pressure, as a result of cerebral edema, often causes various degrees of impairment of neurological functions. The disease can last from 6 to 10 days, ending with complete recovery, leaving behind permanent neurological defects, and even death (in 50% of cases).

Laboratory tests show:

1. increase in liver enzymes (AST and ALT)
2. increase in ammonia concentration
3. decreased glucose levels
4. decrease in the prothrombin index
5. increase in leukocytosis in blood counts
6. the non-inflammatory nature of the cerebrospinal fluid
7. fatty liver assessed by histopathology

It is found after excluding other diseases

A wide variety of conditions can follow a course similar to Reye’s syndrome, such as meningitis, encephalitis, hepatitis, drug and toxin poisoning, and metabolic diseases. Moreover, there are no diagnostic tests that would enable us to diagnose the disease with certainty. Therefore, the diagnosis of Reye’s syndrome follows the exclusion of other, more probable, causes of the observed disorders. The diagnosis of the disease is based on the following: typical biphasic, usually jaundice-free course, non-inflammatory nature of the cerebrospinal fluid, the result of histopathological examination of the liver biopsy, exclusion of other causes of acute encephalopathy and liver failure.

It is extremely important that the parents carefully monitor a child with a respiratory tract infection in order to initiate appropriate treatment as early as possible in the event of alarming symptoms. Disturbing symptoms that should prompt parents to consult a doctor include: violent vomiting after taking aspirin, child’s irritability, convulsions, excessive sleepiness, confusion, inadequate answers to questions, confusion or coma. The presence of Reye’s syndrome should always be considered when looking for the cause of violent vomiting with disturbed consciousness in a child who has had a viral infection and was treated with aspirin.

Intensive treatment

Treatment of Reye’s syndrome should take place in a hospital setting, often in intensive care units, as it requires very careful monitoring of the patient’s condition and often intensive therapeutic activities. It aims to improve the child’s general condition and prevent complications, which may be serious. Therapy here mainly consists of rehydration and the use of symptomatic medications (antipyretic, antiemetic). If the patient’s vital signs are not improved, mannitol, furosemide and dexamethasone are used to lower intracranial pressure and prevent impairment of neurological functions.

How to reduce the risk

At the moment, a causal treatment for Reye’s syndrome has not been developed as we still do not know what the root cause is. Therefore, the principles of prevention of this life-threatening disease are particularly important. Scientists have shown a link between viral infection treated with aspirin and the development of Reye’s syndrome and limited the indications for its use in children. Aspirin in children can only be prescribed in exceptional circumstances, such as Kawasaki disease. In addition to these cases, you should not use aspirin and other medications that contain it in children under 12 years of age, especially during a viral infection. Instead, paracetamol is recommended, which also has anti-pyretic and analgesic properties and is safer than aspirin.

Acetylsalicylic acid is a drug that is readily available and widely advertised as safe and effective in fighting cold and flu symptoms. You can buy them over the counter, even in supermarkets. To check whether the preparation does not contain acetylsalicylic acid, read the information leaflets carefully. Avoiding its use in children will reduce the risk of Reye’s syndrome.

Text: lek. Jagoda Kumik