Quite rare and very dangerous to human life condition characterized by the development of fatty liver and rapidly progressive encephalopathy, known as Reye's syndrome or Reye.
There is a condition in children and adolescents, often between the ages of 4 and 12 years old, on the background of the treatment of measles, influenza, chicken pox and other viral fevers drugs, of which consists of acetylsalicylic acid (aspirin). According to statistics registered only a few cases where Reye's syndrome is diagnosed in adults.
State accompanied by such manifestations as:
- Raising more than three times the level of aspartate aminotransferase and alanine aminotransferase in serum.
It is noteworthy that in these manifestations bilirubin levels in the blood of the patient is normal. At the heart of the state is generalized mitochondrial damage, which results in violation of the b-oxidation of fatty acids and the inhibition of oxidative phosphorylation.
For the first time the syndrome was described in 1963 by Australian pathologist Ralph Douglas Kenneth Reyes. The doctor, who served at the time acting director of the hospital pathology department, found that in 17 out of 21 child deaths cause of death was encephalopathy and fatty degeneration of internal organs, which later would be called in his honor Reye's syndrome.
The consequences of the state can be brain damage, retardation, seizure disorders, mental retardation, damage to peripheral nerves and muscle twitching. In medical practice there were cases when Reye's syndrome occurs in one and the same child twice.
Reye's syndrome: pathogenesis
Today the pathogenesis of Reye's syndrome is not fully clear, however found a clear relationship between the intake of drugs containing acetylsalicylic acid sick with a fever of viral origin.
Conducted in 1980, the US studies have shown that the mortality rate among children due to Reye's syndrome has decreased significantly when the government level, it was decided to inform the public about the harmful effects of acetylsalicylic acid used during viral infections in children. Thus, we managed to significantly reduce the incidence of the condition and the mortality rate among children after 17 years in 1997, there were only 2 cases of the syndrome.
There is evidence that the pathogenesis of Reye's syndrome in some cases was associated with the transfer of the syndrome blood relative. By the end of this trend it has not been investigated, it is believed that it is related to heredity, or condition may be passed from one family member to another.
Symptoms of Reye's syndrome
Clinical symptoms vary depending on the severity of the syndrome. Usually on the fifth or sixth day of viral disease and varicella - the fourth after the appearance of the rash, the child suddenly there is no nausea and vomiting tamable caused by taking a history of the disease ASA-containing medicines.
These symptoms of Reye's syndrome accompanied by a change in mental status of the child, range from mild lethargy and agitation to episodes of complete disorientation and deep coma.
The main symptoms of Reye's syndrome in children under 3 years are:
- Fatigue and drowsiness;
- Image aspartate, ammonia and serum alanine aminotransferase;
Lack of timely treatment of a condition resulting in rapid development of coma, respiratory arrest, seizures, and dekortikatsionnym detsebratsionnym postures.
In 40% of reported cases of sick children, an increase in the liver, rarely accompanied by jaundice.
Prevention of Reye's syndrome
A necessary measure to prevent the state of children and adolescents is a careful use of ASA-containing drugs in the presence of heat. It is best in this situation to replace aspirin ibuprofen or paracetamol.
Diagnosis of Reye's syndrome
Reye's syndrome is suspected by a doctor occur in cases where no obvious reason the child has vomiting and nausea, as well as there are signs of a sudden development of cerebral edema. For accurate diagnosis of the condition the doctor has to spend a lumbar puncture and biopsy of the liver of the patient.
Reye's Syndrome: Treatment
Most important to quickly deliver the baby in the NICU, as there may be cessation of breathing, which is needed to prevent ventilation. For the treatment of Reye's syndrome, you must have your doctor accurate anamnestic information about the patient and his family, to exclude inborn error of metabolism.
Since that stop the development of the syndrome can not no cure, early diagnosis of the condition and aimed at maintaining vital functions intensive care are the most important.
To prevent bleeding, the patient is administered intravenously electrolytes liquid vitamin K and glucose. Glycerol, mannitol and corticosteroids used to reduce intracranial pressure. Liquid administered in small quantities, as the background of the developing cerebral edema, it can play a fatal role.
Followed by tracheal intubation, in order that the child could not breathe, hyperventilation - to measure intracranial pressure, and the active struggle with brain swelling. In the treatment of Reye's syndrome in certain cases, to control blood pressure and blood gases, necessary to introduce in the vein and artery catheters.
Reye's syndrome is a condition in which rapidly progressive encephalopathy and fatty liver, in patients receiving aspirin in the treatment of viral fevers in children and adolescents.