Potentially fatal process with impairment of multiple organs, but particularly the brain and the liver.
Cause unknown.
Associated with aspirin consumption in children with viral diseases.
Reye syndrome
A rapidly progressive encephalopathy.
Symptoms may include vomiting, personality changes, confusion, seizures, and loss of consciousness.
Liver toxicity is typical.
Jaundice usually does not occur.
Death occurs in 20–40% of those affected.
About a third of those who survive are left with a significant degree of brain damage and disability.
Risk factors:
Aspirin use in children
Viral infection
Inborn errors of metabolism
Treatment
Supportive care.
Children are most commonly affected.
Occurs in less than one in a million children a year.
Cause of Reye syndrome remains unknown.
It usually begins shortly after recovery from a viral infection, such as influenza or chickenpox.
About 90% of cases in children are associated with aspirin use.
Laboratory changes may include a high blood ammonia level, low blood sugar level, and prolonged prothrombin time.
Hepatomegaly May occur.
Prevention- avoiding the use of aspirin in children
When aspirin was withdrawn for use in children a decrease of more than 90% in rates of Reye syndrome was seen.
Treatment is supportive, and early diagnosis improves outcome.
Mannitol may be used to help with the associated brain swelling.
In children use of aspirin only recommended in Kawasaki disease.
Reye syndrome progresses through five clinical stages:
Stage I is manifested by a rash on palms of hands and feet, heavy vomiting, generalized lethargy, confusion, nightmares, headache and no fever.
Stage II is manifested by stupor, hyperventilation, fatty liver, hyperactive reflexes
Stage III is manifested by the continuation of Stage I and II symptoms, with possible coma, cerebral edema and rarely, respiratory arrest.
Stage IV associated with deepening coma, dilated pupils with minimal response to light, minimal but still present liver dysfunction.
Stage V has a rapid onset following stage IV, with deep coma, seizures, multiple organ failure, flaccidity, hyperammonemia (above 300 mg/dL of blood), death
The majority of the surviving patients have various metabolic disorders, particularly a fatty-acid oxidation disorder medium-chain acyl-CoA dehydrogenase deficiency.
The Reye syndrome results from damage to cellular mitochondria, at least in the liver.
Aspirin could cause or exacerbate mitochondrial damage.
The Centers for Disease Control and Prevention (CDC), the U.S. Surgeon General, the American Academy of Pediatrics (AAP) and the Food and Drug Administration (FDA) recommend that aspirin and combination products containing aspirin not be given to children under 19 years of age during episodes of fever-causing illnesses.
Differential diagnosis:
Various inborn metabolic disorders
Viral encephalitis
Drug overdose
Poisoning
Head trauma
Liver failure due to other causes
Meningitis
Kidney failure
Shaken baby syndrome
Cases of Reye syndrome in adults are rare, and recovery of adults with the syndrome is generally complete, within two weeks of onset.
Causes fatty liver with minimal inflammation and severe encephalopathy with brain swelling.
May be associated with hepatomegaly.
Recommended that individuals under the age of 19 years should not be given to aspirin for episodes of fever associated illness.
Has been rarely documented in adults, with usual complete recovery of brain and liver function within 2 weeks of the illness.
Symptoms related to mitochondrial damage in the liver.
5 stages of disease: Stage I-severe vomiting, lethargy, mental impairment and nightmares.
Stage II-stupor, hyperventilation, fatty liver and hyperactive reflexes.
Stage III-continuation of stages I and II, possible coma, possible cerebral edema, and rarely respiratory arrest.
Stage IV-deepening coma, large pupils with minimal light response. minimal but persistent hepatic dysfunction.
Stage V-deep coma, seizures, respiratory failure, flaccidity, extremely high ammonia levels and death.
Mortality 30% in cases in the U.S. from 1981-1997.
Mortality 30% in cases in the U.S. from 1981-1997.
Differential diagnosis-inborn metabolic disorders, viral encephalitis, poisoning, head trauma, meningitis, and renal failure.
About 2 cases per year reported in the U.S.