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Ray's syndrome


Ray's syndrome, syndrome to Reja (acute hepatic encephalopathy, a white hepatic disease) — the rare, but very dangerous, often life-threatening acute state arising at children and teenagers (is more often at the age of 4-12 years) against the background of treatment of fever of a virus origin (flu, measles, chicken pox) the drugs containing acetylsalicylic acid, and characterized by quickly progressing encephalopathy (owing to brain hypostasis) and development of fatty infiltration of a liver. Ray's syndrome is followed by a giperammoniyemiya, increase in level of nuclear heating plant, ALT in blood serum (more than by 3 times) at the normal level of bilirubin.

Symptoms of the Syndrome of Ray:

    * In 5-6 days after the beginning of a viral disease (at chicken pox — for 4-5 day after emergence of rashes) the nausea and pernicious vomiting which is followed by change of the mental status suddenly develop (varies from easy block to a deep coma and episodes of a disorientation, psychomotor excitement). In the anamnesis — reception of acetylsalicylic acid or ASK-soderzhashchikh drugs for the purpose of decrease in temperature.
    * Children up to 3 years the main symptoms of a disease can have a breath disturbance, drowsiness and spasms, and at children of the first year of life tension of a big fontanel is noted.
    * In the absence of adequate therapy the prompt aggravation of symptoms of the patient is characteristic: bystry development of a coma, detserebratsionny and dekortikatsionny poses, spasms, apnoeas.
    * Increase in a liver is noted in 40% of cases, however jaundice is observed seldom.
    * Increase in nuclear heating plant, ALT, ammonia in blood serum of patients is characteristic.

Reasons of the Syndrome of Ray:

Generalized damage of mitochondrions owing to inhibition of oxidizing phosphorylation and disturbance of β-oxidation of fatty acids is the cornerstone of Ray's syndrome.

The syndrome is for the first time described by the Australian pathologist Douglas to Reja, English Ralph Douglas Kenneth Reye in 1963.

Предполагаемые механизмы нарушения b-окисления липидов при синдроме Рея

Estimated mechanisms of disturbance of b-oxidation of lipids at Ray's syndrome

Treatment of the Syndrome of Ray:

No treatment can stop development of a syndrome of Ray. The early diagnosis and an intensive care directed to maintenance of vital signs including blood circulation and breath, are vital. Intravenously enter liquids, electrolytes and glucose, and also vitamin K which warns bleedings. To reduce intracranial pressure, appoint Mannitolum, corticosteroids (for example, dexamethasone) or glycerin. Breath of the child can be supported by the medical ventilator. In certain cases introduction of catheters to arteries and veins is required to control the content of gases in blood and arterial pressure.

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