What is Reye's Syndrome?

Reye's syndrome (RS) is a serious adverse drug reaction with a high mortality rate. This disease is a rare disease in children recovering from a viral infection (such as influenza, cold or chicken pox). Taking salicylic acid drugs (such as aspirin) is an important cause. Extensive mitochondrial damage is its pathological basis. Reye's syndrome affects all organs of the body, but it is most harmful to the liver and brain. If left untreated, it can quickly lead to liver and kidney failure, brain damage, and even death.

Basic Information

nickname: Reye syndrome
English name: Reye Syndrome
Visiting department: Pediatrics
Common causes: The cause is unknown and may be related to infection, taking salicylic acid drugs, poisoning, genetics, etc.

Common symptoms: Constant vomiting, diarrhea, tiredness, poor mental health, exacerbation of the disease, restlessness, hyperexcitation, unconsciousness, convulsions or epilepsy, and even coma

Causes of Reye Syndrome

The cause is unknown, but studies have found that taking salicylic acid drugs (such as aspirin) in people with viral infectious diseases can develop the disease. The cause of the disease is not completely clear. Considered related to the following factors:

Infection

Most children have symptoms of viral infections, such as respiratory infections and digestive tract infections, 1-7 days before the illness. They have also isolated influenza virus, parainfluenza virus, coxsackie virus, herpes virus, EB from Reye syndrome patients. Virus, chickenpox virus, etc. In addition, Reye syndrome such as purulent meningitis and bacterial dysentery can also occur after some bacterial infections. However, there is no evidence that the disease is caused by direct infection.

2. Drugs

A large number of clinical reports have found that children who take salicylate (aspirin) during viral infections are more likely to develop this disease. In view of this, Britain, the United States and other countries have reduced or stopped the application of salicylic acid preparations for children. In 2000, Castels-VanDaeleM and others reported that the incidence of Reye syndrome in Michigan, the United States, decreased with the decrease in the use of aspirin. Studies have found that children with systemic lupus erythematosus and rheumatoid arthritis who are long-term aspirin patients are susceptible to Reye syndrome, and the incidence is significantly reduced after use. The above studies have suggested that aspirin is related to this disease, but the relationship between aspirin and Reye is not yet clear. In addition, the use of other drugs can also cause the same symptoms as Reye syndrome, such as the anti-epileptic drug valproate.

3 toxins

Aflatoxin, organic pesticides and other poisoning patients can develop the same clinical phenotype as Reye syndrome.

4. Genetic metabolism

Some children have a family history, suggesting that the disease may be related to genetic factors.

Clinical manifestations of Reye's syndrome

Symptoms of the disease may appear during viral disease, but more often after a week or two. The child usually vomits at first. Other early symptoms include diarrhea, fatigue, and poor mental health.

As the disease worsens and affects the brain, the child may become restless, hyper-excited, unconscious, convulsed or epileptic, or even unconscious.

Wright syndrome

Blood routine

Most white blood cell counts increased significantly, and the classification was mainly based on increased neutrophils.

2. Liver function test

Serum alanine aminotransferase increased, prothrombin time prolonged.

3. Blood biochemical examination

Blood ammonia, plasma free fatty protein acids, and short-chain fatty protein acids are elevated. Most of the blood sugar is reduced, and a small number of children have normal blood sugar.

4. Cerebrospinal fluid examination

Except for headaches with elevated pressure, the number of cells and protein were mostly within the normal range.

5. EEG examination

Shows severe diffuse abnormalities.

6. Imaging examination

Skull CT and MRI examinations online can help rule out brain occupying lesions.

7. Liver biopsy

The diagnosis of this disease relies on a biopsy of the liver. It can be seen that there are a large number of fat droplets in liver cells, and characteristic changes such as mitochondrial enlargement and reduction or disappearance of dense bodies under electron microscope.

Wright's syndrome diagnosis

Diagnosis can be confirmed based on medical history, clinical manifestations, and auxiliary examinations.

Differential diagnosis of Reye's syndrome

Wright's syndrome is often misdiagnosed, in part because the disease is so uncommon and may be misdiagnosed as encephalitis, meningitis, diabetes, overdose or poisoning.

Wright syndrome treatment

Treatment principle

The emphasis is on symptomatic treatment of metabolic disorders, control of cerebral edema, reduction of intracranial pressure, and control of convulsions. It mainly deals with the two basic pathophysiological changes of the disease, namely cerebral edema and liver failure, for treatment, monitoring and evaluation.

2. Intensive care

Cardiopulmonary and intracranial pressure must be monitored for patients with severe encephalopathy and abnormalities should be detected in time. Because the disease changes rapidly, it can often suddenly change from mild to severe, so those who are less ill should also be closely monitored.

3. Treatment of brain lesions

Control of cerebral edema is the focus of treatment of this disease. While reducing intracranial pressure, the perfusion pressure of the brain must be maintained. Reduce intracranial pressure: use osmotic diuretics, 20% mannitol intravenously, once every 4 to 6 hours at the beginning. Fast urine and dexamethasone can be applied simultaneously. Monitoring intracranial pressure: subarachnoid or epidural manometers can be used to maintain intracranial pressure below 20mmHg. Monitor blood gas: keep the airway open, prevent hypoxemia and hypercapnia, to avoid aggravating cerebral edema. Maintain normal blood pressure: to ensure that the perfusion pressure in the brain is above 50mmHg. Cerebral perfusion pressure = mean arterial pressure-intracranial pressure. If cerebral perfusion pressure is too low, it will cause cerebral hypoxia and aggravate cerebral edema. Cerebral edema often requires limiting fluid intake.

4. Reduce blood ammonia

Can be given with vinegar enema, 10-20ml each time, and then diluted twice with sterile saline to retain the enema. Oral 50% lactulose suspension. Supply enough heat (30 ~ 40cal / kg). Those who have the condition can also use abdominal dialysis, fresh blood or plasma exchange therapy to reduce blood ammonia. Arginine infusion, neomycin orally or enema to reduce ammonia production, citrulline can convert ammonia to urea. Sodium glutamate is added intravenously to glucose to correct hyperammonemia. Protect liver function and strengthen supportive therapy. Some people have used blood exchange therapy to achieve certain results.

5. Prevention and treatment of bleeding

Cholesterol vitamin K can be given to help the synthesis of prothrombin, but also can be infused with coagulation factors or fresh plasma.

6. Correct hypoglycemia

Hypoglycemia must be corrected in time, and 10% to 20% glucose is intravenously injected to maintain blood glucose at 150 to 200 mg / dl. When blood glucose reaches slightly above normal levels, insulin can be added to reduce the breakdown of free fatty acids.

7. Correct metabolic disorders

Maintain water-electrolyte and acid-base balance.

8. Other

Due to the dangerous condition of Wright's syndrome, different children may experience different symptoms and complications of multiple organ damage. Necessary symptomatic and supportive treatment should be given in time.