Reye Syndrome
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Basics
Description
- Reversible clinicopathologic syndrome of unknown etiology
- Primary mitochondrial injury
- Decreased enzyme activity:
- Krebs cycle
- Gluconeogenesis
- Urea biosynthesis
- Fatty infiltration:
- Liver:
- Hyperammonemia due to decreased conversion from ammonia to urea
- Hepatorenal syndrome may be the end result
- Rapid recovery of liver function in survivors
- Brain:
- Encephalopathy of unclear etiology
- Cytotoxic edema
- Deteriorating level of consciousness reflects increasing intracranial pressure (ICP)
- Herniation is the most common cause of death
- Normal recovery of neurologic function in survivors
- Skeletal and myocardial muscle
- Fatty infiltration and distorted mitochondria
- Liver:
- <10% of cases occur before the age of 1 yr:
- Average age is 7 yr
- Peak age is 4–11 yr
- Extremely rare in age >18 yr
- Regional differences:
- Highest incidence in the Midwestern states
- Lower incidence in the states of the Southeast and far West
- More common in whites than in blacks
- Peak incidence in winter and early spring
- Reye-like syndrome:
- Describes conditions resulting in defects in urea and fatty acid metabolism, toxicologic injury, and impaired gluconeogenesis
Etiology
- Not known with certainty
- Multifactorial causes have been epidemiologically implicated:
- Antecedent viral syndrome
- Influenza A or B
- Varicella
- Diarrhea illness
- Genetic predisposition
- Exposure to salicylates
- Other undefined factors
-- To view the remaining sections of this topic, please log in or purchase a subscription --
Basics
Description
- Reversible clinicopathologic syndrome of unknown etiology
- Primary mitochondrial injury
- Decreased enzyme activity:
- Krebs cycle
- Gluconeogenesis
- Urea biosynthesis
- Fatty infiltration:
- Liver:
- Hyperammonemia due to decreased conversion from ammonia to urea
- Hepatorenal syndrome may be the end result
- Rapid recovery of liver function in survivors
- Brain:
- Encephalopathy of unclear etiology
- Cytotoxic edema
- Deteriorating level of consciousness reflects increasing intracranial pressure (ICP)
- Herniation is the most common cause of death
- Normal recovery of neurologic function in survivors
- Skeletal and myocardial muscle
- Fatty infiltration and distorted mitochondria
- Liver:
- <10% of cases occur before the age of 1 yr:
- Average age is 7 yr
- Peak age is 4–11 yr
- Extremely rare in age >18 yr
- Regional differences:
- Highest incidence in the Midwestern states
- Lower incidence in the states of the Southeast and far West
- More common in whites than in blacks
- Peak incidence in winter and early spring
- Reye-like syndrome:
- Describes conditions resulting in defects in urea and fatty acid metabolism, toxicologic injury, and impaired gluconeogenesis
Etiology
- Not known with certainty
- Multifactorial causes have been epidemiologically implicated:
- Antecedent viral syndrome
- Influenza A or B
- Varicella
- Diarrhea illness
- Genetic predisposition
- Exposure to salicylates
- Other undefined factors
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