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Reye's syndrome

 
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Last reviewed: 04.07.2025
 
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In 1963, R. Reye et al. described acute fatty degeneration of internal organs (primarily the liver), occurring in combination with toxic encephalopathy, which was later called Reye's syndrome.

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What causes Reye's syndrome?

The pathogenesis of Reye's syndrome is based on congenital mitochondrial insufficiency. Congenital defect of enzymes involved in urea synthesis - the ornithine cycle (ornithine transcarbaminase, carbamine phosphate synthetase, etc.) is also important. The syndrome can be provoked by drugs (salicylates), hepatotropic poisons and other substances.

Symptoms of Reye's syndrome

A prodromal period is possible, proceeding as an acute respiratory viral infection or acute intestinal infection. Vomiting is often the main clinical symptom before the development of coma. A characteristic feature of coma in patients with Reye's syndrome is an early increase in muscle tone up to decerebrate rigidity. Almost all patients have a poorly controlled tonic convulsive seizure. The meningeal symptom complex is not fully expressed, is inconstant. Hepatomegaly is maximally expressed by the 5th-7th day of coma development. Signs of cerebral edema are characteristic.

The severity of neurological disorders by the depth of neurological disorders is divided into 4 degrees: I - somnolence, II - stupor, III - coma proper, IV - terminal coma. The damage can be of varying degrees of severity: for I degree, an increase in the activity of aminotransferases and ammonia levels is characteristic, for II degree - the addition of signs of insufficiency of protein-synthetic function (decreased levels of albumin, prothrombin, etc.), for III - the appearance of a hemorrhagic syndrome complex.

Diagnosis of Reye's syndrome

Laboratory findings: increased aminotransferase levels (2-5 times), short-term hyperammonemia (precedes coma), hypoglycemia (up to 2.5 mmol/l), hypofibrinogenemia without the presence of fibrin degradation products in the blood (no DIC), hypoalbuminemia, etc. Jaundice and hyperbilirubinemia are not typical.

Elevated levels of enzymes and ammonia persist for 3-4 days. To clarify the diagnosis of Reye's syndrome, a liver biopsy is often required. Children aged 3 months to 3 years are most often affected.

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