Reye's syndrome

Reye's syndrome is a rare, but dangerous disease that can even threaten life if it is started. The disease is characterized by the development of edematous syndrome in the brain and in the liver, in the future - fatty dystrophy. This syndrome was first described in 1963 in The Lancet by Dr. Douglas Reye along with colleagues Graham Morgan and Jim Baral. Most often he appeared in patients with chickenpox, influenza type B and other viral infections.

In the course of research, it was found that the development of the syndrome is more likely in blood relatives of those people who were ill with this disease. It was not yet possible to identify what exactly this is connected with.

[1], [2], [3]

Epidemiology

Reye's syndrome is found exclusively in children.

[4], [5], [6], [7], [8]

Causes of the reye syndrome

The disease develops because of hypersensitivity of children to drugs that contain acetylsalicylic acid (it is the cause of this disease). Such agents are prescribed in the case of fever and high fever - for example, in case of influenza, chickenpox gastroenteritis or a common acute respiratory disease.

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

As a consequence, the liver develops the so-called hepatic fatty infiltration. Along with this, aseptic inflammation develops in the brain with the development of edema. That is why this syndrome is also called acute hepatic encephalopathy or, more rarely, white hepatic disease.

[9], [10], [11]

Pathogenesis

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

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

[12], [13], [14], [15], [16], [17],

Symptoms of the reye syndrome

In the clinic, there are two variants of the course of the disease: classical and atypical.

With classical aspirin-associated Reye syndrome, the signs of the disease develop more often in children older than 5 years, have a two-phase course, usually accompanied by prodromal phenomena, the use of aspirin in conventional therapeutic dosages, has a two-phase course.

Clinical signs of Reye's syndrome appear after a viral infection (influenza, chickenpox) and not a long (on average 3 days) latent period.

With lesions of the brain and liver, there are such signs:

• Neurological symptoms (the patient does not understand where he is, does not recognize anyone, is not able to answer even the simplest questions, aggression, apathy);
• Accelerated and shallow breathing with the development of hyperventilation;
• There is a strong aggression, even to causeless attacks;
• The patient begins cramps, he falls into a coma from 24 hours to 3 days, sometimes up to several weeks.

First signs

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

Among the first symptoms of Ray's syndrome:

• The sudden nausea that accompanies regular vomiting;
• Lethargy and lack of energy;
• Strange behavioral reactions - irritability, some inhibition in actions, problems with speech;
• Constant drowsiness.

[18], [19]

Reye syndrome in children and newborns

Reye syndrome usually appears in adolescents or children who took aspirin during the treatment of a viral disease. It disrupts the functional activity of the brain, liver, and kidneys. Without conducting prompt prompt treatment, the disease can lead to death.

In infants up to the age of 5, Reye's syndrome manifests itself in atypical form (the so-called rhea-like syndrome), accompanied by congenital anomalies of metabolism.

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

Reye's syndrome in adults

Typically, Rhea's syndrome is affected by small children and adolescents, but this disease affects adults in very rare cases.

Stages

The syndrome of Ray passes through five stages:

Step I

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

Stage II

• Stupor.
• Hyperventilation.
• Heavy dystrophy of the liver (by biopsy results).
• Hyperactive reflexes.

Stage III

• Symptoms of stages I and II.
• A coma is possible.
• Possible cerebral edema.
• Rarely, stop breathing.

Stage IV

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

Stage V

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

[20], [21], [22], [23], [24], [25]

Complications and consequences

Possible consequences and complications of Ray's syndrome:

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

[26], [27], [28], [29]

Diagnostics of the reye syndrome

It is rather difficult to diagnose Reye's syndrome, since he does not have any specific symptoms, peculiar only to him. That is why the risk of making mistakes when diagnosing a doctor is quite high. It should be noted that before the examination the doctor should be informed about all the medications that the patient took during the treatment of the viral disease.

[30], [31], [32],

Analyzes

Analyzes are conducted to identify possible abnormalities in the oxidation of fatty acids, as well as the detection of other metabolic disorders.

For the syndrome of Ray is characterized by the following indicators of the blood test:

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

There is also a procedure for lumbar puncture - the patient takes a sample of fluid from the spinal cord. It is conducted in order to exclude the presence of other diseases in which there are neurological manifestations such as encephalitis and meningitis. With Reye's syndrome, the number of white blood cells in the smear should not exceed 8 / μl.

Procedure of liver biopsy - to exclude diseases in which liver damage occurs, a sample of liver tissue is taken.

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

In the process of taking the analysis, the patient is anesthetized, then a small puncture in the right side of the abdomen is carried out with a special needle and a small piece of liver is taken.

When biopsies show a diffuse fatty dystrophy of liver cells, deposited glycogen inflammatory cell infiltrates are absent.

[33], [34], [35], [36]

Instrumental diagnostics

In the diagnosis of Reye's syndrome, instrumental methods of investigation are also used. For example, it is a computer tomography, an electroencephalogram, and also an MRI.

Thanks to MRI and CT of the head, it is possible to exclude possible disorders in the brain activity, manifestation of which is lethargy and a change in the behavior of the patient. This allows for differential diagnosis with tumors, various aneurysms, hemorrhages in the brain, and abscesses.

Differential diagnosis

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

[37], [38], [39],

Treatment of the reye syndrome

Treatment of Ray's syndrome is carried out permanently. If the disease is severe, the patient can be sent to the intensive care unit. In the hospital, the patient will be under constant control - his vital signs will be carefully monitored.

Treatment is directed, first of all, to the removal of cerebral edema, correction of metabolic disorders and bleeding disorders.

Treatment procedures can be as follows:

• Infusion of medications intravenously (through the dropper, the electrolyte and glucose solutions prescribed by the doctor can be administered);
• Anticonvulsant medications to prevent the prevention of possible convulsive seizures;
• Diuretics - diuretics relieve the body of excess fluid, thereby reducing intracranial pressure;
• Treatment of possible bleeding due to acute fatty liver disease. It is possible to use vitamin K, as well as transfusion to the patient platelet mass and plasma.

If the patient has problems with self-breathing, artificial ventilation is carried out. In severe cases, liver transplantation can be performed

Medications

In the course of treatment, diuretics and anticonvulsants are often prescribed.

With Reye's syndrome, treatment with homeopathic remedies, alternative medicine and herbs is not recommended.

Prevention

As prevention of the disease, there is only one method - the refusal to use acetylsalicylic acid and its medicines containing in the treatment of young children, as well as adolescents. For example, you should not use such drugs as Citramon or Ascofen. It is also not recommended to use medicines that contain salicylates or salicylic acid.

[40], [41], [42], [43]

Forecast

Reye's syndrome can have a favorable prognosis if the disease is in mild form - 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 progress of hepatological and metabolic disorders. Important factors are also the level of ammonia in the blood and the degree of cerebral edema. In the case of confluence of the patient into a deep coma, the probability of subsequent death increases to 80%.

If the patient was able to endure the acute stage of the disease, he will begin to recover. Later, when seizures occur, symptoms of damage to brain cells may appear - convulsive disorders, muscle twitching, impaired peripheral nerves, mental retardation is possible. In rare cases, this disease can occur twice in one person.

[44], [45], [46]