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Diseases » Reye's Syndrome » Wikipedia
 

Reye's Syndrome in Wikipedia

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This article is licensed under the GNU Free Documentation License. It uses material from the Wikipedia article "Reye's syndrome". (Source - Retrieved 2006-09-07 14:06:15 from https://en.wikipedia.org/wiki/Reye%27s_syndrome)

Introduction

Reye's syndrome is a potentially fatal disease that causes numerous detrimental effects to many organs, especially the brain and liver.

Causes

The cause of Reye's syndrome remains unknown. There is speculation as to whether Reye's syndrome is a single disease or a group of disorders. Many studies indicate that a significant percentage of cases in young children are rediagnosed later and redesignated as other disorders or conditions -- as high as 25% in the UK and 50% in Australia. [[1]] Some studies reported a correlation between use of aspirin or other salicylates in children and adolescents who have a viral infection such as influenza or chicken pox. However, many of the studies were criticized for relying too heavily on the naturally biased recall of caregivers and were also validly criticized for including a substantial number of misdiagnosed cases that were actually metabolic disorders, drug reactions, other illnesses or conditions related to infections. It is important to note that no studies established a causal link to aspirin or salicylates. The vast majority of children who take aspirin while ill with a virus such as chicken pox or flu do not develop Reye's Syndrome and many children who develop the syndrome did not take aspirin or salicylates. It stands to reason then that other factors, perhaps genetic or biological, must also be involved. [[2]] Strikingly, in US studies, 93% of cases for which racial data is available occur in caucasians [[3]]. Nevertheless, the symptoms of Reye's Syndrome appear to involve damage to cellular mitochodria, at least in the liver, and a number of ways that aspirin could also cause or exacerbate mitochondrial damage may exist. An increased risk of contracting Reye's Syndrome is one of the main reasons that aspirin has not been recommended for use in those under the age of 16, the age group for which the risk of lasting serious effects is highest. Reye's Syndrome occurs in all age groups, not just the young, but adults do not appear to be as vulnerable to permanent neural or liver damage. There is a recorded case of a 59 year-old with Reye's Syndrome but only 2% of all cases occur in adults over 20. [[4]] (N.B.,unlike in the UK and Australia, the definition of the disorder in the US by the CDC restricts Reye's Syndrome to pediatric cases.)


The syndrome is named after Dr R. Douglas Reye, who, along with fellow Australians Dr. Graeme Morgan and Dr. Jim Baral, published the first study of the syndrome in 1963 in the British medical journal, The Lancet (2:749-52), though the disease was first diagnosed as a unique illness in 1929. Also in 1963, Dr. George Johnson, along with colleagues, published a study about cases with a remarkably similar profile that his team observed occuring in South Carolina following an influenza B outbreak. Formally, the disorder is known as Reye-Johnson Syndrome, although it is more commonly called Reye's Syndrome. In 1980, studies in Ohio, Michigan and Arizona by Starko, et al. [[5]]pointed to the use of aspirin during an upper respiratory tract infection and chicken pox as a potential indicator for the syndrome. Aspirin use in children and adolescents decreased sharply in the mid-1980's in North America, the UK, Europe and Australia. A decline in the incidence of Reye's syndrome was almost immediately observed. The decrease in the number of cases of Reye's syndrome was once thought to directly correspond to a decrease in aspirin use [[6]] but that surmise has since come under some scrutiny. The decrease in the number of cases may also reflect a concurrent increased accuracy in diagnosis and the redesignation of misdiagnosed cases since the mid-1980's -- i.e., initially there were thought to be some 47 cases of Reye's Syndrome in the UK in 1980/1981 but after a reassessment of susbsequent rediagnoses, that figure was revised downward to just 7 cases. How many reported cases prior to 1980 might also be removed from the tallies, resulting in a decrease, if reassesments were conducted where possible? It is worth noting that a decrease in the number of cases has also been observed in countries where children's aspirin is still in use. A dramatic worldwide decrease since 1996, regardless of salicylate use, has not been satisfactorily accounted for. Further case studies, primarily in the UK and Australia, have revealed 19 viruses singly and in conjunction with salicylates, pesticides and aflatoxin as potential factors contributing to the disease.

Presentation

Symptoms and signs

Reye's syndrome progresses through five stages, explained below:

  • Stage I
  • Stage II
  • Stage III
    • Possible coma
    • Possible cerebral edema
    • Rarely, respiratory arrest
    • Also, symptoms above continue
  • Stage IV
    • The ever-deepening coma
    • Large pupils with minimal response to light
    • Minimal but still present hepatic dysfunction
  • Stage V
    • Very rapid onset after happening of stage IV
    • Death is imminent
    • Deep coma
    • Strong seizures
    • Respiratory failure
    • Flaccidity
    • Extremely high blood ammonia (above 300mg per 100mL of blood)

Features

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Early diagnosis is vital, otherwise death or severe brain damage may follow.

The disease causes hepatic steatosis with minimal inflammation and severe encephalopathy (with swelling of the brain). Jaundice is NOT usually present. The liver may become slightly enlarged and firm, and there is a change in the appearance of the kidneys. (Suchy, Frederick, Ed: J Liver Disease in Children. Mosby, St. Louis.1994. Chapter 36.)

Prognosis

In adults who survive the acute illness the recovery is generally complete, with liver and brain function returning to normal within two weeks of the illness. In children however, permanent brain damage is possible, especially in infants, and ranges from mild to severe.

Differential diagnosis

Causes for similar symptoms include

Epidemiology

While Reye's Syndrome is considered a children's disease, an ever larger percentage of the cases now appear in teenagers and adults, where it is often confused with excessive alcohol or other drug consumption. Factors other than a viral infection and salicylates are suspected for the disease, while over 90% of patients admitted to hospitals in the United States with Reye's Syndrome have taken aspirin to treat the symptoms of a viral infection, less than 0.1% of children who have had their viral infections treated with aspirin develop the syndrome. The syndrome also seems to have a familial factor, with a greatly increased odds of developing this disease when a family member has had it.

The incidence of death in patients admitted with Reye's syndrome in the United States fell in the 1980's from over 40% to between 20-30%. However, the incidence of death increased again in the $1990s$ (52% in 1996). The CDC speculates that this may be due to decreased interest in the disease.

References

  • Arrowsmith JB, Kennedy DL, Kuritsky JN, Faich GA. National patterns of aspirin use and Reye syndrome reporting, United States, 1980 to 1985. Pediatrics. 1987;79:858-63. PMID 3588140.
  • Johnson GM, Scurletis TD, Carroll NB. A study of sixteen fatal cases of encephalitis-like disease in North Carolina children. N C Med J 1963;24:464-73. PMID 14053538.
  • Reye RDK, Morgan G, Basal J. Encephalopathy and fatty degeneration of the viscera. A disease entity in childhood. Lancet 1963;2:749-52. PMID 14055046.
  • Starko KM, Ray CG, Dominguez LB, Stromberg WL, Woodall DF. Reye's syndrome and salicylate use. Pediatrics 1980;66:859-64. PMID 7454476.
 

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