Ray's syndrome

Quite rare and very life-threatening condition of the person which is characterized by development of fatty infiltration of a liver and the fast-progressing encephalopathy is called Ray's syndrome or Reja.

There is such state at children and teenagers, often aged from 4 up to 12 years, against the background of treatment of measles, flu, chicken pox and other fevers of a virus origin medicines as a part of which there is an acetylsalicylic acid (aspirin). According to statistical data, only several cases when Ray's syndrome was revealed at the adult are registered.

The state is followed by such manifestations as:

• Hyper ammonium;
• Increase more than three times the level of aspartate aminotransferase and alaninaminotranspherase in blood serum.

It is remarkable that at these manifestations bilirubin level in blood of the patient remains normal. Generalized damage of mitochondrions to which leads disturbance of b-oxidation of fatty acids and inhibition of oxidizing phosphorylation is the cornerstone of a state.

For the first time the syndrome was described in 1963 by the Australian pathologist Ralf Douglas Kenneth to Reja. The doctor fulfilling duties of the director of hospital department of pathology for that time found out that in 17 of 21 cases of children's death encephalopathy and fatty dystrophy of internals which and will be called later in his honor Ray's syndrome was the cause of a lethal outcome.

Injuries of a brain, block, convulsive frustration, a delay of intellectual development, damage of peripheral nerves and twitching of muscles can be effects of a state. In medical practice cases when Ray's syndrome arose at the same child twice were registered.

Ray's syndrome: pathogeny

Today the pathogeny of a syndrome of Ray remains up to the end not clear, however the accurate interrelation between reception of the drugs containing acetylsalicylic acid by patients with fever of a virus origin is revealed.

The researches conducted in 1980 in the USA showed that mortality among children because of Ray's syndrome considerably decreased when at the government level the decision to notify the public on harmful influence of the acetylsalicylic acid applied in the period of viral infections at children was made. Thus, it was succeeded to reduce considerably the frequency of emergence of a state and mortality among children, after 17 years in 1997 only 2 cases of a syndrome were recorded.

There are data according to which the pathogeny of a syndrome of Ray in certain cases was connected with transferring of a syndrome by the blood relative. Up to the end this tendency was not investigated, it is supposed that it is connected with heredity, or the state can be transferred from one relative to another.

Symptoms of a syndrome of Ray

Clinical manifestations vary depending on weight of a syndrome. Usually for the fifth or sixth day of a viral disease, and at chicken pox – on the fourth after emergence of rashes, the child suddenly has a nausea and not ukrotimy vomiting caused by reception in the anamnesis of disease ASK-soderzhashchikh of drugs.

These symptoms of a syndrome of Ray are followed by change of the mental status of the child varying from easy block and psychomotor excitement to episodes of a full disorientation and a deep coma.

The main symptoms of a syndrome of Ray at children under 3 years are:

• Breath disturbances;
• Increased fatigue and drowsiness;
• Increase in aspartate aminotransferase, ammonia and alaninaminotranspherase in blood serum;
• Spasms.

Lack of timely treatment of a state leads to bystry development of a coma, an apnoea, spasms, dekortikatsionny and detsebratsionny poses.

In 40% of the registered cases at sick children the increase in a liver which is seldom accompanied with jaundice was observed.

Prevention of a syndrome of Ray

Necessary measure of prevention of a state at children and teenagers is careful use ASK-soderzhashchikh of medicines with high temperature. Better in a similar situation to replace acetylsalicylic acid with the Ibuprofen or Paracetamol.

Diagnosis of a syndrome of Ray

Suspicions on Ray's syndrome at the doctor arise when without any obvious reasons the child has a vomiting and nausea, and also signs of sudden edematization of a brain appear. For reliable diagnosis of a state the doctor needs to carry out a lumbar puncture and a biopsy of a liver of the patient.

Ray's syndrome: treatment

It is the most important to bring the child to intensive care unit as the apnoea for which prevention artificial ventilation of the lungs is necessary is possible quickly. For treatment of a syndrome of Ray the doctor needs to possess the exact anamnestic information about the patient and his family to exclude inborn disbolism.

In a type of the fact that no treatment can stop development of a syndrome early diagnosis of a state and the intensive care directed to maintenance of the vital functions of an organism play the most important role.

For the warning of bleedings to intravenously sick enter electrolytes, liquids, vitamin K and glucose. Glycerin, corticosteroids and Mannitolum apply for the purpose of cerebral decompression. Liquid is appointed in a small amount as against the background of the developing wet brain it can play a fatal role.

Further the trachea intubation in order that the child could breathe, a hyperventilation – for measurement of intracerebral pressure, and active fight against wet brain follows. At treatment of a syndrome of Ray in certain cases control over arterial pressure and content in blood of gases, requires introduction to veins and arteries of catheters.

Ray's syndrome call a state at which encephalopathy and fatty infiltration of a liver, against the background of reception of acetylsalicylic acid quickly progresses at treatment of fevers of a virus origin at children and teenagers.