Reye syndrome
| Classification according to ICD-10 | |
|---|---|
| G93.7 | Reye syndrome |
| ICD-10 online (WHO version 2019) | |
The Reye's syndrome is a disease in which acute damage to the brain ( encephalopathy ) and a fatty degeneration of the liver in the foreground. It is a rare condition that mostly affects children between the ages of four and nine, but can occur in all ages. The disease is fatal in up to 25% of cases, and neurological disorders such as language and learning difficulties remain in around 30% . It was first described in 1963 by the Australian pathologist Ralph Douglas Kenneth Reye (1912–1977) as a separate entity .
Symptoms
Reye's syndrome occurs on average one week after viral infections (influenza, varicella, herpes) have subsided. The intake of salicylates (e.g. aspirin) can also be the cause. Symptoms of acute liver insufficiency (enlargement of the liver, increase in liver enzymes, coagulation disorders) and encephalopathy (impaired consciousness, cerebral seizures) appear. The affected child usually begins to vomit suddenly, has a fever , is restless and irritable, and hypoglycaemia occurs. According to Huttenlocher , Reye's syndrome can be divided into different stages with different prognoses. In severe cases, the child is plagued by violent cramps and may even fall into a coma (stage III according to Huttenlocher). In 60% of children, cerebral edema (stage IV) develops as the disease progresses .
The corresponding warning in the product information on acetylsalicylic acid (ASA) -containing medicinal products reads:
“Acetylsalicylic acid should only be used in children and adolescents with febrile illnesses on medical advice and only if other measures are not effective. If these illnesses lead to prolonged vomiting, this can be a sign of Reye's syndrome, a very rare but life-threatening disease that requires immediate medical treatment. "
Emergence
Damage to the mitochondria is pathogenetically relevant, especially for metabolically active organs such as the liver , skeletal muscles and the brain . Furthermore, the number of peroxisomes in the liver cells is increased.
The following mitochondrial enzymes / metabolic functions are impaired:
- Pyruvate dehydrogenase and enzymes of the respiratory chain in all organs → increased anaerobic metabolism → lactate formation → acidification of the body.
- Carbamoyl phosphate synthetase, especially in the liver → urea cycle impaired → the "nerve poison" ammonia accumulates.
- Beta-oxidation (fatty acid breakdown) in the liver and muscles → lack of energy and accumulation of fatty acids.
It comes to the "fatty degeneration" of the liver ( fatty liver inflammation ). Ammonia accumulates and leads to increased internal pressure in the brain , the so-called brain edema .
The actual cause of Reye's syndrome is unknown. However, the number of reported cases in the United States, for example, fell in line with the decline in the use of salicylates for febrile illnesses following warnings from medical professionals, the public and on drug packages. An association of previous viral infections with the intake of ASA or other salicylates is therefore assumed. A genetic predisposition is also discussed .
Diagnosis
- Blood test, urine, toxins
- Brain waves
- Imaging procedures
- Liver biopsy
- Medication history
therapy
There is no causal therapy. Treatment is symptomatic in the form of intensive medical monitoring and therapy:
- Sedation (psychological depressant drugs)
- Ventilation
- osmotic diuretics (e.g. mannitol ) used to lower pressure in the brain
- Peritoneal dialysis for ammonia detoxification
Individual evidence
- ↑ RDK Reye, G. Morgan, J. Baral: Encephalopathy and fatty degeneration of the viscera: a disease entity in childhood. In: Lancet. 1963; 2, pp. 749-752. (English)
- ↑ AllEx. The compendium for the 2nd ÄP. Volume B: Pediatrics. Stuttgart 2012, ISBN 978-3-13-146951-9 , pp. 565-566.
- ↑ AllEx. The compendium for the 2nd ÄP. Volume B: Pediatrics. Stuttgart 2012, ISBN 978-3-13-146951-9 , pp. 565-566.
- ↑ Aspirin SPC. (Fachinformation), July 2007 via fachinfo.de
- ^ ED Belay, JS Bresee, RC Holman, AS Khan, A. Shahriari, LB Schonberger: Reye's syndrome in the United States from 1981 through 1997. In: N Engl J Med. 1999 May 6; 340 (18), p. 1377 -1382. PMID 10228187 (English).
