Reye's syndrome

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Related to Reyes Syndrome: Raynaud's syndrome

Reye's Syndrome

 

Definition

Reye's syndrome is a disorder principally affecting the liver and brain, marked by rapid development of life-threatening neurological symptoms.

Description

Reye's syndrome is an emergency illness chiefly affecting children and teenagers. It almost always follows a viral illness such as a cold, the flu, or chicken pox. Reye's syndrome may affect all the organs of the body, but most seriously affects the brain and liver. Rapid development of severe neurological symptoms, including lethargy, confusion, seizures, and coma, make Reye's syndrome a life-threatening emergency.
Reye's syndrome is a rare illness, even rarer now than when first described in the early 1970s. The incidence of the disorder peaked in 1980, with 555 cases reported. The number of cases declined rapidly thereafter due to decreased use of aspirin compounds for childhood fever, an important risk factor for Reye's syndrome development. Because of its rarity, it is often misdiagnosed as encephalitis, meningitis, diabetes, or poisoning, and the true incidence may be higher than the number of reported cases indicates.

Causes and symptoms

Reye's syndrome causes fatty accumulation in the organs of the body, especially the liver. In the brain, it causes fluid accumulation (edema), which leads to a rise in intracranial pressure. This pressure squeezes blood vessels, preventing blood from entering the brain. Untreated, this pressure increase leads to brain damage and death.
Although the cause remains unknown, Reye's syndrome appears to be linked to an abnormality in the energy-converting structures (mitochondria) within the body's cells.
Reye's syndrome usually occurs after a viral, fever-causing illness, most often an upper respiratory tract infection. Its cause is unknown. It is most often associated with use of aspirin during the fever, and for this reason aspirin and aspirin-containing products are not recommended for people under the age of 19 during fever. Reye's syndrome may occur without aspirin use, and in adults, although very rarely.
After the beginning of recovery from the viral illness, the affected person suddenly becomes worse, with the development of persistent vomiting. This may be followed rapidly by quietness, lethargy, agitation or combativeness, seizures, and coma. In infants, diarrhea may be more common than vomiting. Fever is usually absent at this point.

Diagnosis

Reye's syndrome may be suspected in a child who begins vomiting three to six days after a viral illness, followed by an alteration in consciousness. Diagnosis involves blood tests to determine the levels of certain liver enzymes, which are highly elevated in Reye's syndrome. Other blood changes may occur as well, including an increase in the level of ammonia and amino acids, a drop in blood sugar, and an increase in clotting time. A liver biopsy may also be done after clotting abnormalities are corrected with vitamin K or blood products. A lumbar puncture (spinal tap) may be needed to rule out other possible causes, including meningitis or encephalitis.

Treatment

Reye's syndrome is a life-threatening emergency that requires intensive management. The likelihood of recovery is greatest if it is recognized early and treated promptly. Children with Reye's syndrome should be managed in an intensive-care unit.
Treatment in the early stages includes intravenous sugar to return levels to normal and plasma transfusion to restore normal clotting time. Intracranial pressure is monitored, and if elevated, is treated with intravenous mannitol and hyperventilation to constrict the blood vessels in the brain. If the pressure remains high, barbiturates may be used.

Prognosis

The mortality rate for Reye's syndrome is between 30-50%. The likelihood of recovery is increased to 90% by early diagnosis and treatment. Almost all children who survive Reye's syndrome recover fully, although recovery may be slow. In some patients, permanent neurologic damage may remain, requiring physical or educational special services and equipment.

Prevention

Because Reye's syndrome is so highly correlated with use of aspirin for fever in young people, avoidance of aspirin use by children is strongly recommended. Aspirin is in many over-the-counter and prescription drugs, including drugs for headache, fever, menstrual cramps, muscle pain, nausea, upset stomach, and arthritis. It may be used in drugs taken orally or by suppository.
Any of the following ingredients indicates that aspirin is present:
  • aspirin
  • acetylsalicylate
  • acetylsalicylic acid
  • salicylic acid
  • salicylate

Key terms

Acetylsalicylic acid — Aspirin; an analgesic, antipyretic, and antirheumatic drug prescribed to reduce fever and for relief of pain and inflammation.
Edema — The abnormal accumulation of fluid in interstitial spaces of tissue.
Mitochondria — Small rodlike, threadlike, or granular organelle witin the cytoplasm that function in metabolism and respiration.
Teenagers who take their own medications without parental consultation should be warned not to take aspirin-containing drugs.

Resources

Organizations

National Reye's Syndrome Foundation. P.O. Box 829, Bryan, OH 43506-0829. (800) 233-7393. 〈http://www.bright.net/∼reyessyn〉.

Reye's syndrome

 [rīz]
an acute, potentially fatal disease of childhood, characterized by severe edema of the brain and increased intracranial pressure, hypoglycemia, and fatty infiltration and dysfunction of the liver. The cause is unknown, but it is almost always associated with a previous viral infection. In the United States, the most frequently reported viral diseases present prior to the development of Reye's syndrome are influenza type B and chickenpox (varicella). At least 14 other viruses are known to have preceded Reye's syndrome. The relationship between these infections and the typical pathologic changes in the brain and liver is not yet clear. However, there is an association between the administration of aspirin for acute febrile illnesses, particularly influenza and varicella, and the subsequent development of Reye's syndrome.

Reye's syndrome was once among the ten major causes of death in children over one year of age. A massive public health campaign was undertaken to warn of the danger of giving aspirin to children with influenza or varicella, and Reye's syndrome now occurs only rarely. Some scientists believe that the disorder was misdiagnosed in the past and that it has always been a major viral disease of children. The prognosis for the disease has been greatly improved with better recognition in its earliest stages and improved modes of therapy.
Symptoms. Within several hours to several days after a viral infection the child suddenly develops persistent vomiting, begins to show signs of encephalopathy, and becomes listless, lethargic, and unusually quiet. As intracranial pressure increases the child may become more agitated, irritable, and delirious. In its severest form the disorder causes convulsions and coma. There are no meningeal or focal neurologic signs present, and the cerebrospinal fluid is normal.
Treatment. Treatment is aimed at the correction of the hypoglycemia, acidosis, and electrolyte imbalance. Measures are employed to reduce intracranial pressure and to correct metabolic abnormalities. There is no specific medication or treatment that will cure the disease. The child must be monitored continuously, and the fluid imbalances and increased intracranial pressure must be corrected as soon as possible to avoid permanent brain damage. The sooner treatment is initiated the better the prognosis.



Reduction in the mortality rate, which has been reported as low as 25 per cent and as high as 80 per cent, is believed to be the result of increased awareness of the disease, earlier diagnosis, and improved modes of therapy.

Additional information about Reye's syndrome and medical facilities equipped to handle the disease can be obtained by calling or writing to the National Reye's Syndrome Foundation, P.O. Box 829, Bryan, OH 43506, (419) 636–2679.

Reye's syndrome

(rīz, rāz) or

Reye syndrome

(rī, rā)
n.
An acute disorder characterized by encephalopathy and an accumulation of fatty deposits in the liver, resulting in vomiting, disorientation, and coma and occurring mainly in children following a viral infection such as chickenpox or influenza. It is linked to the use of aspirin and other salicylates.

Reye's syndrome

[rāz′]
Etymology: Ralph D.K. Reye, Australian pathologist, 1912-1978
a combination of acute encephalopathy and fatty infiltration of the internal organs that may follow acute viral infections. This syndrome has been associated with influenza B, chickenpox (varicella), the enteroviruses, and the Epstein-Barr virus. It usually affects people under 18 years of age, characteristically causing an exanthematous rash, vomiting, and confusion about 1 week after the onset of a viral illness. In the late stage there may be extreme disorientation followed by coma, seizures, and respiratory arrest. Laboratory tests reveal greater than normal amounts of SGOT and SGPT, bilirubin, and ammonia in the blood. A specimen obtained by liver biopsy shows fatty degeneration and confirms the diagnosis. Mortality varies between 20% and 80%, depending on the severity of symptoms. The cause of Reye's syndrome is unknown; however, there appears to be an association with the administration of aspirin. Therefore aspirin is given only if prescribed by a physician for any condition in infants or children. Aspirin should not be given in cases of chickenpox or suspected influenza. No specific treatment is available. Insulin, antibiotics, and mannitol may be given. Blood gases, blood pH, and blood pressure are monitored frequently. Intensive supportive nursing care with meticulous monitoring of all vital functions and prompt correction of any imbalance are of extreme significance in the outcome of this syndrome.
observations Reye's syndrome is staged by the characteristics manifested. In stage I, the child is usually quiet, lethargic, and drowsy, with episodes of vomiting. Pupil reactions are brisk, and commands are followed. Serum lab values show evidence of liver dysfunction; EEG is type 1. In stage 2, there is evidence of deep lethargy, confusion, delirium, and combativeness. Reflexes are hyperreflexic, and hyperventilation is present. Pupillary reactions are sluggish. In stage 3, senses are obtunded, and light coma is present with seizure activity and decorticate rigidity. Pupillary light reaction is still intact. EEG is type II. In stage 4, the coma deepens with seizure activity and decerebrate rigidity. There is loss of oculocephalic reflexes, and pupils are fixed. EEG is type III or IV, and there is evidence of brain dysfunction. In stage 5, there is deep coma, loss of deep tendon reflexes, respiratory arrest, fixed and dilated pupils, and type IV EEG. Definitive diagnosis is established by liver biopsy or by a threefold rise in levels of serum aspartate aminotransferase, serum alanine aminotransferase, or serum ammonia. Survivors may exhibit a neuropsychological deficit.
interventions Deterioration is generally rapid and requires early diagnosis and aggressive treatment. Care in stages 1 and 2 is largely supportive, with frequent monitoring and evaluation of neurological status. Management of cerebral edema and increased intracranial pressure is a key focus in stages 3 through 5 and is monitored with an ICP monitor and treated by administration of mannitol, glycerol, and/or hyperventilation via endotracheal tube and ventilator. Pancuronium bromide is used to immobilize the child on a ventilator. Seizures are managed by IV phenytoin. NG tubes, urinary catheters, and peripheral IVs are placed to manage fluids. An arterial catheter is placed to permit continuous blood pressure measurement and monitor blood gases. A central venous catheter permits monitoring of blood volume and cardiac function and administration of hypertonic solutions. A pulmonary artery catheter may be inserted to monitor pulmonary artery pressure and cardiac output. A cooling blanket may be indicated to manage temperature. If increased intracranial pressure fails to respond to treatment, a decompressive craniotomy may be indicated. Prevention is targeted around routine vaccinations for influenza and varicella and avoidance of aspirin in children.
nursing considerations Nursing interventions during acute disease are continuous and intensive. Careful evaluation is required for neurological, cardiac, and respiratory systems. Management is required for multiple lines and tubes, including peripheral and central IV lines, arterial lines, CVP lines, ICP monitors, retention catheters, NG tubes, and endotracheal tubes. Management of ventilator settings is required. Careful fluid management and intake and output is required to reduce cerebral edema and prevent dehydration. Careful supportive care, including careful hygiene, positioning, and passive range of motion, is required to maintain skin integrity and prevent sequelae of immobility. Sensory stimulation is needed for the unconscious child. Emotional support for parents is essential because the sudden severity and intensity of the disease and the aggressive treatment is a source of extreme anxiety and fear. Prevention is targeted around education about maintaining routine vaccinations for influenza and varicella and the avoidance of aspirin in children.

Reye's syndrome

A severe childhood disorder that may follow a virus infection such as chickenpox, rubella, influenza or a herpes simplex, or echovirus infection. Reye's syndrome features HEPATITIS and dangerous swelling of the brain that may lead to coma and death. The condition is strongly associated with the use of aspirin and this should no longer be routinely given to children. Treatment involves removal of fluid from the brain. (Ralph Douglas Kenneth Reye, 1912–77, Australian physician).