What are the Symptoms of Reyes Syndrome
After Ralph D Douglas Kenneth Reye (1912-1978), Australian pediatrician
Synonym: Reye-Morgan-Baral Syndrome
English: Reye's syndrome, white liver disease
The Reye syndrome is an acute encephalopathy that often occurs 3-5 days after infection of the upper respiratory tract and use of certain medications. Reye's syndrome is a pediatric disease.
The treatment of childhood viral infections with the anti-inflammatory drug ASS is considered to be the cause of the occurrence of Reye's syndrome. Therefore, the treatment of febrile illnesses with ASA in children and adolescents should only be carried out after a strict indication.
Reye's syndrome is a rare disease by following the precautions. The numbers vary from 1-5 cases to one million children under 18 in a year, depending on the source. The mortality is up to 40%.
Reye's syndrome is caused by a malfunction of the mitochondria. Characteristic changes in the mitochondria (loss of the cristae) are found in the liver, skeletal muscles and brain. There are also an increased number of peroxisomes in the cells of the liver.
The metabolism of the mitochondria is impaired:
- Carbamoyl phosphate synthetase, an important enzyme in the urea cycle, has a reduced activity. As a result, the neurotoxic ammonia accumulates.
- The pyruvate dehydrogenase and enzymes of the respiratory chain cytochrome oxidase are also not fully functional. There is an increased anaerobic metabolism. The resulting lactate leads to acidosis.
- The beta-oxidation stops, long-chain fatty acids accumulate.
The liver is subject to fatty degeneration (steatosis hepatis). Ammonia accumulates and leads to the formation of edema in the brain, which leads to the clinical picture of encephalopathy.
Reye's syndrome usually occurs up to the age of 10.
The initial stage of Reye's syndrome is characterized by:
In about a third of patients, the full encephalopathic picture of Reye's syndrome develops as a result:
5.2 Differential Diagnosis
In terms of differential diagnosis, the following should be considered:
The intensive care therapy is symptomatic and supportive. There are no causal therapy concepts.
The patient is intubated and sedated (barbiturates). The intracranial pressure must be monitored invasively; osmotic diuretics such as mannitol can be used to reduce it. Hyperammonemia can be treated with peritoneal dialysis.
The full picture of Reye's syndrome leads to death in over three quarters of all cases. Early therapy in the early stages of the disease can significantly reduce this high mortality.
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