Also known as: Reye’s sequence, Reye-Morgan-Baral syndrome, Reye-Johnson syndrome. Synonyms: fatty liver-encephalopathy syndrome, white liver disease.
An acute disease of children under the age of 15 years, characterized by acute encephalopathy and fatty infiltration of the liver.
DescriptionAn acute disease of children under the age of 15 years, characterized by acute encephalopathy and fatty infiltration of the liver. Pancreas, heart, kidney, spleen and lymph nodes may also be affected. Symptoms usually appear after a respiratory tract infection, often chicken pox or influenza B. Hyperglycaemia is frequently a feature in the under five year olds. A main symptom is vomiting. Central nervous system disorders manifest by stupor, convulsions, and coma.
Some cases occur after febrile conditions in children who have received aspirin. The mortality may be as high as 80 %.
First described in 1929 by W. R. Brain, D. Hunter and H. M. Turnbull. Established as a clinical-pathological entity in 1963 by the Australian physicians D. K. Reye, G. Morgan, and J. Baral.
In 1963 Dr. George Johnson with colleagues published an investigation of an outbreak in 16 children with influenza B that developed neurological problems after the virus. 4 of the children had symptoms that were very similar to those of Reye’s.
Douglas Reye was the director of pathology at the Royal Alexandra Hospital for Children in Sydney when children with the disease were admitted in the 1950s and early 1960s. In the 1963 article Rey and his team desbribed 21 children, of whom 17 died within an average of 27 hours after admission. At autopsy, all were found to have brain swelling, a slightly enlarged, firm and uniformly bright yellow liver, and a change in the appearance of the kidneys. Rey and his colleagues believed this set of symptoms represented a distinct disease, which they called fatty degeneration of the viscera (internal organs) of unknown cause. Though they suspected that ingestion of drugs or poisons may have been responsible for the condition, an investigation into the children's homes revealed they had no access to these substances. ¨
In 1963, George Johnson, M.D., and his co-workers reported an epidemic of 16 fatal cases of an encephalitis-like disease occurring within a four-month period during an outbreak of influenza B in a small North Carolina community. Although children in this group were older than those studied by Reye, and their preceding illness was flu, it was subsequently theorized from Johnson's description of the symptoms and postmortem findings that several of the children who died may have also had the syndrome described by Reye. It became known as Reye-Johnson syndrome, though it's usually referred to as Reye (pronounced rye) syndrome.
BibliographyW. R. Brain, D. Hunter, H. M. Turnbull:
• Acute meningoencephalomyelitis of childhood. The Lancet, London, 1929, 1: 221.
R. D. K. Reye, Graeme Morgan, Jim Baral:
• Encephalopathy and fatty degeneration of the viscera. A disease entity in childhood.
The Lancet, London, 1963, 2: 749-752.
George M. Johnson, T. D. Scurletis, N. B. Carroll:
• A study of sixteen fatal cases of encephalitis-like disease in North Carolina children.
North Carolina Medical Journal, Winston-Salem, 1963, 24: 464-473.
• Reye Syndrome: The Decline of a Disease.
Publication no. (FDA) 94-1172, June 1994, Department of Health and Human Sercvices, Public Health
Service Food and Drug Administration