Reye syndrome
Fabian Dupont is a freelance writer in the medical department. The human medicine specialist is already doing scientific work in Belgium, Spain, Rwanda, the USA, Great Britain, South Africa, New Zealand and Switzerland, among others. The focus of his doctoral thesis was tropical neurology, but his special interest is international public health and the comprehensible communication of medical facts.
More about the experts All content is checked by medical journalists.Reye's syndrome is a serious cellular disorder that can occur in young children and adolescents. It particularly affects the brain and liver and can be fatal. The exact causes have not yet been clarified. Reye's syndrome is particularly associated with flu, herpes and chickenpox viruses. Scientists also assume that drugs such as acetylsalicylic acid can trigger Reye's syndrome after a virus infection.
ICD codes for this disease: ICD codes are internationally recognized codes for medical diagnoses. They can be found, for example, in doctor's letters or on certificates of incapacity for work. G93
Reye syndrome: description
Reye's syndrome is a rare, serious, and potentially life-threatening disease of the brain and liver ("hepatic encephalopathy") in children. It occurs especially after a virus infection and the intake of acetylsalicylic acid (ASA). The exact connection is so far unclear. Reye's syndrome was observed, for example, after an infection with cold and flu viruses or chickenpox viruses. Viruses that cause gastrointestinal infections with diarrhea or vomiting can also be related to Reye's syndrome. The list of potentially involved viruses is possibly much longer.
Reye's syndrome was discovered in Australia in the 1970s. Shortly thereafter, many cases of severe liver and brain diseases in America were assigned to Reye's syndrome. However, it took a few years before the first suspicions about a connection with viral diseases and the pain reliever and fever drug acetylsalicylic acid arose. The result was widespread media awareness that acetylsalicylic acid should not be given to children. Although Reye's syndrome has actually been far less common since then, the connection between the virus, ASA and Reye's syndrome has never been clearly elucidated.
Reye syndrome: symptoms
Reye's syndrome often occurs in children when parents are actually thinking that the viral infection has been overcome. There is increased vomiting without nausea. The child becomes increasingly confused, restless, irritable or just limp and is more and more difficult to respond. Also, a child with Reye's syndrome can have a seizure and even fall into a coma at some point.
The reason for these symptoms is that Reye's syndrome increases intracranial pressure as fluid builds up in the brain (edema formation). The increased pressure affects important nerve centers and nerve tracts in the brain.
At the same time, Reye's syndrome causes damage and obesity to the liver. Their function is severely restricted, which leads to a large number of metabolic disorders with different symptoms.In addition to the nerve toxin ammonia, bilirubin also gets into the blood, which can cause the skin to turn yellow.
In general, the child looks seriously ill and urgently needs intensive medical care.
Reye syndrome: causes and risk factors
The exact causes of Reye's syndrome are not known. It is known, however, that Reye's syndrome damages the mitochondria. Mitochondria are often referred to as the cells' power plants because they are essential for generating energy. The malfunction of the mitochondria in Reye's syndrome is particularly evident in the cells of the liver and brain, but also, for example, in the muscles.
Salicylates like ASA can interfere with the metabolism of the mitochondria, making them more susceptible to further damage. This is how some experts explain the connection between ASD and Reye's syndrome. This connection, so accepted by the professional world, could never be scientifically proven unambiguously. The same goes for the assumption that certain viruses are the trigger for Reye's syndrome.
In addition to the viral infections, salicylates, and age, there may also be a genetic risk for the disease. Some people seem to be more prone to Reye's syndrome than others. The exact genetic causes are still unclear here.
Reye syndrome: examinations and diagnosis
The doctor first collects the medical history (anamnesis). To do this, he asks the child's parents, for example, whether the child recently had a virus infection and / or took salicylates. It is also important to describe the symptoms such as vomiting, possible seizures, and increasing confusion and restlessness. They are possible signs of brain involvement.
Depending on the extent of the disease, the liver in Reye's syndrome may be enlarged, which the doctor can determine by palpating the abdomen. A blood test may also reveal evidence of liver involvement.
Blood test
When the liver is damaged, certain substances such as liver enzymes (transaminases) and waste products, which the liver actually filters out of the blood and break down, increasingly enter the blood. Elevated liver enzyme values and an elevated ammonia level can indicate liver damage.
Since the liver is also responsible for the blood sugar level, a simple blood sugar test can provide quick information about the liver function - in Reye's syndrome, there may be low blood sugar (hypoglycaemia).
The liver is also involved in blood clotting. In Reye's syndrome, the blood clotting time can therefore be prolonged. This can be determined using the Quick value or the INR (international normalized ratio), determined using a blood sample.
Tissue sample
To confirm the diagnosis of “Reye's Syndrome”, the doctor can take a tissue sample (biopsy) from the liver and examine it for appropriate cell damage. The mitochondrial damage is particularly noticeable here. In addition, in Reye's syndrome there is an increased accumulation of fat in the cells. This is a sign that the liver can no longer adequately process the fat.
Other investigations
An ultrasound examination can also provide information about the condition of the liver. If the doctor suspects increased intracranial pressure, this will be checked with a computed tomography (CT) scan.
Reye's syndrome cannot easily be differentiated from other clinical pictures. The various symptoms can also be caused by other diseases, including those that are much more common than the rare Reye syndrome. For this reason, some more diagnostic tests are done to rule out meningitis, blood poisoning, or serious bowel disease, for example.
Reye syndrome: treatment
Reye's syndrome cannot be treated causally. The doctors can only try to alleviate the symptoms and ensure the patient's survival. Intensive medical treatment is necessary for this.
The focus is particularly on brain swelling (brain edema) and liver failure. With the help of medication that is given directly into the bloodstream and other measures (such as elevating the upper body), one can try to lower the pressure in the brain. If the liver is severely damaged, the organ must be supported in its tasks (such as filtering and regulating sugar metabolism). An excessively high ammonia level in the blood (hyperammonaemia) can be treated with medication (with sodium benzoate) or with dialysis (hemofiltration). In severe cases of liver damage, a liver transplant may be required.
The work of all organs of the body is interrelated. The kidneys and liver in particular form a well-coordinated team. If the liver is suddenly damaged, there is a risk of kidney failure (hepatorenal syndrome). With the help of medication, the medical team can maintain the urine output through the kidneys.
The heart and lung function are also closely monitored, because the damage to the brain may require artificial ventilation, for example.
Reye's syndrome: disease course and prognosis
Reye's syndrome is very rare, but it is usually rapid and severe. About 50 percent of patients die. Many survivors suffer permanent damage. After overcoming Reye's syndrome, brain damage often remains, which manifests itself, for example, in paralysis or speech disorders.
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