What Is Reye Syndrome
What Is Reye Syndrome ?
Reye syndrome causes inflammation of the brain and impairment of liver function. This rare, but potentially fatal, disorder is associated with certain viral infections in children and the use of the drug aspirin.
- The typical symptoms include a viral infection followed by nausea and severe vomiting, lethargy, confusion and in some cases, coma.
- The exact cause is not known, but certain viral infections and the use of the drug aspirin may be the trigger.
- Reye syndrome is diagnosed from the typical symptoms, blood tests and a liver biopsy.
- The severity and the extent of damage suffered by the brain determine the prognosis, with the rate of mortality ranging from 2% to 80% depending on the stage of the illness.
- This condition is treated mainly by lowering the excess pressure in the brain and treating the other symptoms present.
Even though the exact reason for Reye syndrome is not known, it is often found to occur following certain viral infections such as a flu caused by the influenza virus or chicken pox which is caused by the virus varicella. It is found to occur mostly in children who are given the drug aspirin to bring down the fever caused by these viral infections. Aspirin is no longer given to children in view of the increased chance of it being a trigger for Reye syndrome, except to treat certain specific disorders such as Kawasaki disease and juvenile idiopathic arthritis. Those children who require aspirin therapy are vaccinated against viral infections such as chicken pox and influenza to avoid the risk of developing Reye syndrome.
With the decline in the use of aspirin in children, the incidence of Reye syndrome has become much less frequent, with only about 2 cases occurring in a year. It usually affects children below 18 years of age, and is more prevalent in winter season and the latter part of fall.
Symptoms
Reye syndrome can be mild or severe. It usually starts with the typical symptoms of an upper respiratory tract infection of viral origin, influenza, or occasionally of chickenpox. After 5 days to a week, the child suddenly has nausea and severe vomiting. The child’s condition deteriorates rapidly, with lethargy, disorientation and confusion setting in, often within the same day. Seizures may occur, and the child may fall into a coma which progresses steadily. Liver function may become impaired, leading to problems with blood clotting which may cause bleeding. Ammonia levels in the blood may increase excessively due to loss of liver function.
Diagnosis
Reye syndrome is suspected when children suddenly have vomiting, and start displaying symptoms of encephalopathy or brain dysfunction. Blood tests and a biopsy of the liver tissue are done to rule out other possible causes and to confirm Reye syndrome. The condition is categorized into 5 stages according to its severity, with the mildest form as stage I, and the severest as stage V.
Prognosis
The prognosis greatly depends on several factors such as the extent of damage inflicted on the brain by the inflammation, the duration of the brain dysfunction, the blood ammonia level, the pressure in the brain, and how fast the coma progresses. The mortality rate varies according to the stage of the disease severity and progression; the stage I carrying a 2 % risk and children in coma in the stages IV and V having 80%. If the children survive the critical phase, they may recover completely, but those who have suffered severe symptoms may sustain brain damage which can result in muscular weakness, intellectual disabilities or seizures.
Treatment
Reye syndrome has no particular treatment; mainly the symptoms are treated after admitting the affected child in the intensive care unit. If liver is affected, the child may have problems with clotting of blood. In such cases, fresh frozen plasma or vitamin K is given to help blood clotting. Endotracheal intubation is done to lower the pressure in the brain and reduce swelling. In this procedure, a tube is placed in the windpipe, and a high breathing rate is induced. Raising the head of the bed, restricting fluid intake, and giving mannitol or other drugs which help the body remove excess water, are other measures taken to reduce the swelling. The pressure in the brain may be monitored with a device placed in the head. Dextrose is administered to maintain normal blood sugar levels.
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Yasser Elnahas
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