Ray's syndrome

Reye's syndrome is a rare but dangerous disease that can even be life-threatening if left untreated. The disease is characterized by the development of edema in the brain and liver, and later fatty degeneration. This syndrome was first described in 1963 in The Lancet by Dr. Douglas Reye and his colleagues Graham Morgan and Jim Baral. It most often appeared in patients with chickenpox, influenza type B, and other viral infections.

During the research it was found that the development of the syndrome is more likely in blood relatives of those people who suffered from this disease. It has not yet been possible to determine what exactly this is connected with.

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Epidemiology

Reye's syndrome occurs exclusively in children.

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Causes Ray syndrome

The disease develops due to hypersensitivity of children to medications containing acetylsalicylic acid (it is the cause of the disease). Such medications are prescribed in case of fever and high temperature - for example, with flu, chickenpox, gastroenteritis or common acute respiratory infections.

Once in the body, this acid can lead to the destruction of cell membrane elements, disruption of fatty acid metabolism and water-electrolyte balance, and an increase in aminotransferases and/or ammonia in the blood serum.

As a result, the so-called hepatic fatty infiltration develops in the liver. At the same time, aseptic inflammation develops in the brain with the development of edema. That is why this syndrome is also called acute hepatic encephalopathy or, less commonly, white liver disease.

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Pathogenesis

Clinical studies have shown that Reye's syndrome is based on mitochondrial destruction. In the pathogenesis, an important role is played by a violation of beta-oxidation of fatty acids due to exogenous (infectious diseases, taking certain medications) or endogenous (congenital pathologies accompanied by metabolic disorders) causes, which leads to a decrease in the level of Acetyl-CoA and the activity of pyruvate carboxylase. Because of this, glycogen production decreases, which leads to the development of hypoglycemia.

In addition, the activity of malate and succinate dehydrogenase decreases in the citrate cycle, and the activity of cytochrome oxidase decreases in the respiratory chain. There is a disruption of the activity of enzymes in the mitochondrial section of the urea cycle - ornithin transcarbamylase, as well as carbamyl phosphate synthetase.

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Symptoms Ray syndrome

The clinic distinguishes two variants of the disease: classical and atypical.

In classical aspirin-associated Reye's syndrome, signs of the disease develop most often in children over 5 years of age, have a two-phase course, are usually accompanied by prodromal phenomena, and are taken with aspirin in normal therapeutic doses.

Clinical signs of Reye's syndrome appear after a viral infection (flu, chickenpox) and a short (on average 3 days) latent period.

When the brain and liver are affected, the following symptoms occur:

• Neurological symptoms (the patient does not understand where he is, does not recognize anyone, is unable to answer even the simplest questions, aggression, apathy);
• Rapid and shallow breathing with the development of hyperventilation;
• Strong aggression is shown, up to and including unprovoked attacks;
• The patient begins to have convulsions and falls into a coma lasting from 24 hours to 3 days, sometimes up to several weeks.

First signs

When the first signs of the disease begin to appear depends on the severity of the organ damage. Usually, the syndrome first appears 5-6 days after the onset of the viral disease. If it appears with chickenpox, the symptoms may begin earlier - on the 4th day after the rash appears.

Among the first symptoms of Reye's syndrome:

• Sudden onset of nausea, accompanied by regular, severe vomiting;
• Lethargy and lack of energy in the child;
• Strange behavioral reactions – irritability, some inhibition in actions, problems with speech;
• Constant drowsiness.

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Reye's syndrome in children and newborns

Reye's syndrome usually occurs in teenagers or children who have taken aspirin as part of a treatment for a viral disease. It disrupts the functional activity of the brain, liver, and kidneys. Without prompt and timely treatment, the disease can lead to death.

In infants under 5 years of age, Reye's syndrome manifests itself in an atypical form (the so-called Reye-like syndrome), accompanied by congenital metabolic anomalies.

The initial manifestations of the disease in children under 2 years of age are hyperventilation and diarrhea.

Reye's syndrome in adults

Reye's syndrome usually affects young children and adolescents, but adults are extremely rarely affected by this disease.

Stages

Reye's syndrome goes through five stages:

Stage I

• Rash on the palms of the hands and feet.
• Persistent severe vomiting.
• General lethargy.
• Confusion of consciousness.
• Nightmares.
• Headaches.

Stage II

• Stupor.
• Hyperventilation.
• Fatty liver disease (based on biopsy results).
• Hyperactive reflexes.

Stage III

• Symptoms of stages I and II.
• Coma is possible.
• Cerebral edema is possible.
• Rarely, respiratory arrest.

Stage IV

• Deepening of coma.
• Dilated pupils with minimal reaction to light.
• Minimal liver dysfunction.

Stage V

• Deep coma.
• Cramps.
• Multiple organ failure.
• Lethargy.
• Hyperammonemia (above 300 mg/dL blood).
• Death.

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Complications and consequences

Possible consequences and complications of Reye's syndrome:

• cardiovascular collapse with signs of acute respiratory failure, conduction disturbances (arrhythmia), sudden cardiac arrest;
• aspiration pneumonia;
• DIC syndrome;
• diabetes insipidus;
• septic condition;
• paraplegia and paralysis of the legs and arms;
• acute gastrointestinal;
• coma.

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Diagnostics Ray syndrome

Reye's syndrome is quite difficult to diagnose, as it does not have any specific symptoms. That is why the risk of making a mistake when making a diagnosis is quite high. It should be noted that before the examination, the doctor must be informed about all medications that the patient took during treatment for the viral disease.

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Tests

Tests are performed to identify possible disturbances in the process of fatty acid oxidation, as well as to detect other metabolic disorders.

The following blood test results are characteristic of Reye's syndrome:

• increased levels of transaminases (ALT, AST), ammonia in the blood;
• prolongation of prothrombin time;
• decreased blood glucose levels (hypoglycemia).

A lumbar puncture procedure is also performed - a sample of fluid is taken from the patient's spinal cord. It is performed to exclude the presence of other diseases in which neurological manifestations are observed - such as encephalitis and meningitis. In Reye's syndrome, the number of leukocytes in the smear should not exceed - 8 /mcl.

Liver biopsy procedure - to rule out diseases that cause liver damage, a sample of liver tissue is taken.

Liver biopsy is performed mainly in children under 1 year of age, with frequent relapses or atypical signs of the syndrome (without prodrome and vomiting).

During the analysis process, the patient is given anesthesia, then a small puncture is made in the right side of the abdomen with a special needle and a small piece of liver is taken.

Biopsy reveals diffuse fatty degeneration of liver cells, deposited glycogen, and no inflammatory cellular infiltrates.

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Instrumental diagnostics

Instrumental methods of examination are also used in diagnosing Reye's syndrome. For example, this is a computer tomography, electroencephalogram, and MRI.

MRI and CT of the head can exclude possible disturbances in brain activity, the manifestation of which are lethargy and changes in the patient's behavior. This allows for differential diagnostics with tumors, various aneurysms, hemorrhages in the brain, and abscesses.

Differential diagnosis

Since the signs of Reye's syndrome are not specific and can occur with other diseases, they can only be diagnosed by exclusion. Reye's syndrome is differentiated from neuromuscular diseases, viral infections and side effects of various drugs, as well as toxins, which can cause encephalopathy and hepatopathy.

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Treatment Ray syndrome

Treatment of Reye's syndrome is carried out in hospital. If the disease is severe, the patient may be sent to the intensive care unit. In the hospital, the patient will be under constant supervision - his vital signs will be closely monitored.

Treatment is aimed primarily at relieving cerebral edema, correcting metabolic disorders and blood clotting disorders.

The treatment procedures may be as follows:

• Intravenous administration of medications (electrolyte solutions and glucose prescribed by the doctor can be administered through a drip);
• Anticonvulsant medications to prevent possible seizures;
• Diuretics - diuretics rid the body of excess fluid, thereby reducing intracranial pressure;
• Treatment of possible bleedings arising from acute fatty liver disease. In this case, it is possible to use vitamin K, as well as transfuse the patient with platelet mass and plasma.

If the patient has problems with independent breathing, artificial ventilation of the lungs is performed. In severe cases, a liver transplant may be performed.

Medicines

During treatment, diuretics and anticonvulsants are often prescribed.

In case of Reye's syndrome, treatment with homeopathic preparations, traditional medicine and medicinal herbs is not recommended.

Prevention

There is only one method for preventing the disease - refusal to use acetylsalicylic acid and medicines containing it when treating small children and adolescents. For example, you should not use such drugs as Citramon or Askofen. It is also not recommended to use medicines that contain salicylates or salicylic acid.

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Forecast

Reye's syndrome may have a favorable prognosis if the disease is mild - the probability of death in this case is only 2%. But it should be understood that this depends on the severity of damage to the functional activity of the brain, as well as the rate of progression of hepatological and metabolic disorders. Important factors are also the level of ammonia in the blood and the degree of cerebral edema. If the patient falls into a deep coma, the probability of subsequent death increases to 80%.

If the patient has managed to survive the acute stage of the disease, he will begin to recover. Later, when convulsions appear, symptoms of damage to brain cells may appear - convulsive disorders, muscle twitching, disturbances in the functioning of peripheral nerves, mental retardation is possible. In rare cases, such a disease can appear twice in one person.

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