Reye's Syndrome

Introduction to Wright's Syndrome Reye Syndrome is a group of syndromes characterized by cerebral edema and liver dysfunction caused by organ fat infiltration. It is also called encephalopathy with visceral steatosis syndrome. First reported by Rey in 1963, mostly in children or children between the ages of 6 months and 15 years, with an average age of 6 years, rare in adults. The cause of the disease is unknown, and it is believed that it is related to viral infections, such as influenza virus, Coxsackie virus, herpes virus, Epstein-Barr virus, etc., and may also be related to aflatoxin, salicylic acid preparation or environmental genetic factors. basic knowledge Sickness ratio: 0.0001% Susceptible population: Most children or children between 6 months and 15 years old, with an average age of 6 years, rare in adults Mode of infection: non-infectious Complications: cerebral edema

Cause of Reye's syndrome

Cause:

The cause of the disease is unknown, and it is believed to be related to viral infections, such as influenza virus, Coxsackie virus, herpes virus, Epstein-Barr virus, etc., may also be related to aflatoxin, salicylic acid preparation or environmental genetic factors, mitochondria after viral infection The structural and metabolic abnormalities lead to a series of biochemical changes that are the main basis for liver and brain lesions. The main pathological changes are mainly brain and liver. A large number of fat deposits are seen, fat droplets are filled in the cytoplasm, and mitochondrial swelling is observed under electron microscope. In a multi-morphological change, the internal hemorrhoids disappear, the matrix stretches in a strip shape, the particles become thicker, the glycogen is reduced, and in addition, there are fat infiltration in organs such as renal tubules, myocardium, pancreas, gastrointestinal tract, lung, spleen and lymph nodes.

Rui's syndrome prevention

Care should be taken to protect liver function and strengthen supportive care. Although the cause of Reye syndrome is unclear, studies have shown that aspirin or aspirin-containing drugs can cause this condition. The best prevention method you can do now is not to give your child aspirin. Prenatal screening avoids the birth of the child. Prenatal screening is mainly for diseases that currently have no good treatment, and the purpose is to prevent the birth of defective children. Usually in the 16th to 20th week of pregnancy, 2-3 ml of peripheral blood of pregnant women should be examined. If the risk of high risk is found (high risk factor exceeds 1/270), further amniocentesis should be taken to confirm the diagnosis.

Rui's syndrome complications Complications brain edema

Can be complicated by influenza, cerebral edema, liver dysfunction and other diseases. The mortality rate is 10%-40%, and the survivors may have residual sequelae such as mental retardation, epilepsy, paralysis and behavioral abnormalities. Can die from cerebral palsy and brainstem dysfunction. Liver function is abnormal without jaundice. Physical examination: liver mild to moderate increase. Most children have symptoms of hypoglycemia and hyperammonemia, and a few are associated with dehydration and metabolic acidosis.

Symptoms of Reye's syndrome Common symptoms Nausea and vomiting Respiratory failure Coma liver enlargement Drowsiness convulsions Dehydration

The disease is rapid onset, and its main clinical manifestations are:

1 There are often prodromal symptoms of upper respiratory tract and digestive tract infections within 2 weeks before the disease.

2 brain damage, the most prominent manifestation of this disease, when the current symptoms turn around, sudden vomiting and severe headache can occur suddenly, start to be irritated, mentally disturbed, lethargy, and then turn into convulsions, coma, and even brain rigidity State, can die due to respiratory failure.

3 liver damage, showing liver enlargement, accompanied by liver dysfunction, more jaundice and bleeding tendency.

4 most with hypoglycemia, a small number of dehydration and metabolic acidosis.

Inspection of Wright's syndrome

1. Increased serum transaminase, prolonged prothrombin time, elevated blood ammonia, plasma free fatty acids and short-chain fatty acids.

2. Cerebrospinal fluid examination pressure increased, but the number of cells and protein were within the normal range.

3. EEG examination showed moderate, severe diffuse abnormalities.

4. CT examination helps to rule out brain occupying lesions.

Diagnosis and identification of Wright's syndrome

diagnosis

According to the history of mild upper respiratory tract and digestive tract infection before pediatric disease, followed by progressive neurological symptoms, liver signs and hypoglycemia, the disease should be considered. The diagnosis of this disease depends on the liver biopsy. It can be seen that there are a lot of fat droplets in the liver cells, and the characteristic changes such as mitochondrial enlargement and the decrease or disappearance of the dense body are observed under the electron microscope.

Differential diagnosis

Attention should be paid to the identification of diseases such as epidemic cerebrospinal meningitis, Japanese encephalitis, purulent meningitis and sepsis.

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