Reye 's syndrome, Reye's syndrome (acute liver failure and encephalopathy, "white liver disease") is a rare, but very dangerous, often life-threatening acute condition that occurs in children and adolescents (usually aged 4-12 years) with treatment of viral fever origin ( flu , measles , chickenpox ) drugs containing acetylsalicylic acid , and characterized by rapidly progressing encephalopathy (due to cerebral edema) and the development of fatty liver . Reye's syndrome is accompanied by hyperammonemia [3] , an increase in the level of AST , ALT in the blood serum (more than 3 times) with a normal level of bilirubin .
| Reye's Syndrome | |
|---|---|
| ICD-10 | G 93.7 |
| ICD-10-KM | |
| ICD-9 | 331.81 |
| ICD-9-KM | |
| Diseasesdb | 11463 |
| Medlineplus | 001565 |
| eMedicine | emerg / 399 |
| Mesh | D012202 |
Reye's syndrome is based on generalized mitochondrial damage due to inhibition of oxidative phosphorylation and impaired β-oxidation of fatty acids .
The syndrome was first described by Australian pathologist Douglas Ray ( en: Douglas Reye ) in 1963 [4] .
Content
Clinical manifestations
- 5-6 days after the onset of the viral disease (with chickenpox - 4-5 days after the rash appears) nausea and indomitable vomiting suddenly develop, accompanied by a change in mental status (varies from mild inhibition to deep coma and episodes of disorientation, psychomotor agitation). A history of taking acetylsalicylic acid or ASA-containing drugs in order to lower the temperature.
- In children under 3 years of age, the main signs of the disease can be respiratory failure, drowsiness and cramps, and in children of the first year of life, there is tension of a large fontanel.
- In the absence of adequate therapy, a rapid deterioration of the patient's condition is characteristic: the rapid development of coma , decerebration and decortication poses, convulsions , respiratory arrest.
- An increase in the liver is noted in 40% of cases, but jaundice is rare.
- An increase in AST, ALT, and ammonia in the blood serum of patients is characteristic.
Forecast
Mortality in children with Reye's syndrome is 20-30%, but it is less than 2% in children with a mild course of the disease and more than 80% with a deep coma. The prognosis depends on the severity of changes in brain functions and the rate of their progression; on how high the intracranial pressure is , and on the amount of ammonia in the blood (a high ammonia content indicates a significant inhibition of liver function). Children who overcome the acute phase of the disease usually recover completely. If convulsions occur, some signs of brain damage may later appear, such as mental retardation, convulsive disorders, muscle twitches, or damage to peripheral nerves. Reye's syndrome in rare cases occurs in one child twice.
Prevention
You should not give your child aspirin if he has the flu, measles, or chicken pox. Use caution when prescribing acetylsalicylic acid at high temperature in children under 12 years of age. In this situation, it is recommended to replace acetylsalicylic acid with paracetamol or ibuprofen . Call your doctor right away if your child has signs: vomiting, severe headache, lethargy, irritability, delirium , respiratory distress, stiff arms and legs, coma.
In order to prevent the development of Reye's syndrome, parents should be given antipyretic and analgesic drugs that do not contain salicylates. [5] .
Treatment
No treatment can stop the development of Reye's syndrome. Early diagnosis and intensive care aimed at maintaining vital functions, including blood circulation and breathing, are vital. Fluids, electrolytes and glucose are injected intravenously to avoid cerebral edema, as well as vitamin K , which prevents bleeding. To reduce intracranial pressure, mannitol and corticosteroids (for example, dexamethasone ) are prescribed. The baby's breathing can be supported by a ventilator . In some cases, the introduction of catheters into arteries and veins is required to control the gas content in the blood and blood pressure .
See also
Hepatic coma - a more common case
Notes
- ↑ Disease Ontology release 2019-05-13 - 2019-05-13 - 2019.
- ↑ Monarch Disease Ontology release 2018-06-29sonu - 2018-06-29 - 2018.
- ↑ Hyperammonemia (Russian) // Wikipedia. - 2017-06-21.
- ↑ RD Reye, G. Morgan, J. Baral, Encephalopathy and fatty degeneration of the viscera. A disease entity in childhood , Lancet , 2 (7311), 1963, pp. 749–752
- ↑ Medical Encyclopedia. - 2009 .-- S. 183-185.