Thursday, February 19, 2009

Reye's Syndrome

Today we admitted a child with clinical manifestations of fever, rashes, and hepatomegaly (liver enlargement). The doctor's initial diagnosis was Hepatitis. But as they assessed the child deeper and laboratory reports came, they finally diagnosed her with Reye's Syndrome. This is one of the rare cases we encounter here in the hospital.

So what is Reye's syndrome? It is a potentially fatal disease that causes numerous detrimental effects to many organs, especially the brain and liver. It is associated with aspirin consumption by children with viral diseases such as chickenpox. So it is very important to remember: not to give your children Aspilets during a viral infection.

The disease causes fatty liver with minimal inflammation, and severe encephalopathy (with swelling of the brain). The liver may become slightly enlarged and firm, and there is a change in the appearance of the kidneys. Jaundice (yellowish discoloration of the skin, sclera, and mucous membrane) is not usually present. Early diagnosis is vital, otherwise death or severe brain damage may follow. The cause of this disease is still unknown, that is why they call it a syndrome, to cover the umbrella of symptoms being manifested by the disease process.

The treatment of Reye's syndrome varies. Since it is an acute, rapidly progressive disease, it should be treated as a medical emergency, and time is of the utmost importance. The chance of recovery is greatly increased when it is treated in its earliest stages. To date there is no cure for the disease. Successful management of the disease depends on early diagnosis. Therapy is primarily directed to protect the brain against irreversible damage by reducing the brain swelling. Medical management includes IV infusion of 10% Glucose in maintenance salt solution, maintain airway and brain oxygenation, and IV mannitol.

People with Reye's Syndrome require the services of an intensive care unit and physicians and nurses experienced in the treatment of the disease. A person with Reye's Syndrome should be transferred to a teaching hospital or a children's hospital. If this is not possible, immediate phone consultation with a teaching hospital or children's hospital. The majority of individuals with Reye's Syndrome are children; however, cases have been reported in adults.

If Reye's Syndrome is suspected, two liver function tests should be done immediately: SGOT (SAT) & SGPT (ACT). Abnormal SGOT and SGPT strongly suggest a diagnosis of Reye's Syndrome. Immediate further diagnostic testing will give a definite diagnosis.

References: Wikipedia and National Reye's Syndrome Foundation.

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