REYES SYNDROME

-Reyes syndrome is acute encephalitis with accompanying fatty infiltration of the liver, heart, lungs, pancreas, and skeletal muscle. -Onset: occurs in children 1 to 18 years of age regardless of gender. -Cause: unknown, but usually occurs after a viral infection such varicella (chickenpox) or influenza that was treated with a salicylate such as acetylsalicylic acid (aspirin). Anticipatory guidance to parents and children about avoiding the use of aspirin during viral infections has led to almost total prevention of this syndrome. Pathophysiology, Etiology and Incidence Exposure to viral agent or toxin in at-risk children leads to liver cell damage with rising serum ammonia levels. The toxic serum ammonia levels results in cerebral dysfunction (encephalopathy, cerebral edema), fluid and electrolyte imbalances, and coagulopathies. The average age at onset is 6-7 years. Reyes syndrome might be related to administration of aspirin to children with viral disease. Assessment After seeming to recover from an initial viral illness, children become ill again 1 to 3 weeks later, with lethargy, vomiting, agitation, anorexia, confusion, and combativeness. Symptoms in adolescents may be so extreme that they mimic those of drug intoxication.As fatty droplets invade the liver, enzyme abnormalities, hypothrombinemia, hypoglycemia, and elevated blood ammonia occur. Although CSF findings remain normal, cerebral symptoms progress from confusion to stupor to deep coma, with seizures and respiratory arrest resulting from pressure on the brain stem. Laboratory diagnosis of Reyes syndrome is confirmed by elevated liver enzyme levels (alanine aminotransferase [ALT or SGPT] and aspartate aminotransferase [AST or SGOT]), elevated serum ammonia, normal direct bilirubin, delayed prothrombin time and partial thromboplastin time, decreased serum amylase, elevated blood urea nitrogen, elevated serum amylase, elevated short-chain fatty acids, and an elevated white blood count. A lumbar puncture is usually done to rule out other infection. CSF findings are normal, except for slightly elevated opening pressure. A skull CT scan or sonogram will be normal at first. Later, it will show cerebral edema and decreased ventricle size. A liver biopsy shows fatty infiltration, establishing a definitive diagnosis. Therapeutic Management Left untreated, Reyes syndrome is rapidly fatal. If it is diagnosed early and promptly treated, the child usually recovers quickly ad without any residual neurologic effects.

Reyes syndrome is not infectious. It is categorized by stages of involvement from 1 to 5, depending on the degree of the childs lethargy or coma. In stages 1 to 2, the child responds to stimuli but may exhibit lethargy or delirium and possibly combativeness. In stages 3 through 5, the child is unresponsive to stimuli with a progressively deepening coma. Frequent neurologic assessments are necessary to evaluate the childs status and possible progression to a more serious stage of involvement. Therapy is directed toward supporting respiratory function, controlling hypoglycemia, and reducing brain edema. Nursing Considerations Care is similar to that for any child with increasing ICP, with the potential addition of mechanical respiratory support. Accurate, continuous monitoring of neurologic and cardioresporatory status is essential because the childs condition can deteriorate suddenly. Fluid replacement is achieved with IV hypertonic solutions if ICP is not increased. Protect the child from coagulopathy-related injury.

Reference: Maternal and Child Health Nursing: Care of he childbearing and childrearing family Fifth edition by Adele Pilliteri (pages 1561) Maternal and Child Nursing Second edition by Mc Kinney, James, Murray and Ashwill (page 1528)