REYE’S SYNDROME BY: JENNIFER DELANEY

OBJECTIVES HISTORY HISTORY ETIOLOGY ETIOLOGY WHAT IT DOES WHAT IT DOES SIGN & SYMPTOMS SIGN & SYMPTOMS STAGES STAGES WHAT WE WILL BE CALLED FOR WHAT WE WILL BE CALLED FOR DIAGNOSIS DIAGNOSIS DIFFERNTIAL DIAGNOSIS DIFFERNTIAL DIAGNOSIS TREATMENT TREATMENT RECOVERY RATE RECOVERY RATE

HISTORY Was named after Dr. R. Douglas Reye. Was named after Dr. R. Douglas Reye. Also discovered with fellow Australians Dr. Graeme Morgan & Dr. Jim Baral. Also discovered with fellow Australians Dr. Graeme Morgan & Dr. Jim Baral. Study was first published in 1963 in the British Medical Journal. Study was first published in 1963 in the British Medical Journal. The first occurrence may have occurred prior in The first occurrence may have occurred prior in 1929.

HISTORY CONT. 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. 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. For this it is also know as Reye’s- Johnson Syndrome. For this it is also know as Reye’s- Johnson Syndrome.

ETIOLOGY Precise reason why it occurs is unknown. Precise reason why it occurs is unknown. It’s called a syndrome because the clinical features that are used to diagnosis it are very broad. It’s called a syndrome because the clinical features that are used to diagnosis it are very broad. Major presentation that occurs in the United States occurs after viral-flu like illness or after varicella. Major presentation that occurs in the United States occurs after viral-flu like illness or after varicella.

ETIOLOGY CONT. The involvement of salicylates and Reye’s was first suggested in 1962, which was before Reye’s was a clinical entity. The involvement of salicylates and Reye’s was first suggested in 1962, which was before Reye’s was a clinical entity. Reports showed that in 4 cases of infants that had varicella infections who had received ASA, 2 of the infants developed symptoms compatible with Reye’s Reports showed that in 4 cases of infants that had varicella infections who had received ASA, 2 of the infants developed symptoms compatible with Reye’s

ETIOLOGY CONT. Another study done in 1975 reviewed 56 patients with pathologically confirmed Reye’s. 53 of whom had some sort sacilcylate ingestion. Another study done in 1975 reviewed 56 patients with pathologically confirmed Reye’s. 53 of whom had some sort sacilcylate ingestion. In 1977 yet another study was done with 175 confirmed Reye’s syndrome which showed that 78% of the patients had received ASA. In 1977 yet another study was done with 175 confirmed Reye’s syndrome which showed that 78% of the patients had received ASA.

ETIOLOGY CONT. These plus many other reports have led to a great deal of talk about ASA and children. These plus many other reports have led to a great deal of talk about ASA and children. This lead to a significant decrease in the occurrence after This lead to a significant decrease in the occurrence after It is now not recommended to give ASA to kids 18 and under without doctor’s approval. It is now not recommended to give ASA to kids 18 and under without doctor’s approval. Progresses through 5 stages Progresses through 5 stages

WHAT IT DOES Disease causes fatty deposits to develop in the liver and the brain. Disease causes fatty deposits to develop in the liver and the brain.

SIGNS & SYMPTOMS Usually show up 3-4 days after a viral illness such as influenza B and varicella. Usually show up 3-4 days after a viral illness such as influenza B and varicella. Usually occurs during the Fall & Winter months. Usually occurs during the Fall & Winter months. Predominantly occurs in children. Predominantly occurs in children.

S/S CONT. Continuous N/V Continuous N/V Sleepless/lethargy Sleepless/lethargy Confusion Confusion Irritability Irritability Aggressive or irrational behavior Aggressive or irrational behavior Weakness or hemi- paresis Weakness or hemi- paresis Seizures Seizures Loss of consciousness Loss of consciousness Sluggish respiratory rate Sluggish respiratory rate Dilated pupils Dilated pupils Enlarged liver-with no jaundice present Enlarged liver-with no jaundice present Encephalitis Encephalitis Increased ICP Increased ICP Posturing- decerebrate or decorticate Posturing- decerebrate or decorticate

STAGES Stage I: Stage I: ~ Persistent, heavy N/V ~ Generalized lethargy ~ Confusion ~ Nightmares

STAGES CONT. Stage II: Stage II: ~ Stupor (caused by minor brain swelling) ~ Hyperventilation ~ Fatty liver (biopsy) ~ Hyperactive reflexes

STAGES CONT. Stage III: Stage III: ~ Continuation of stages I & II ~ Coma (possible) ~ Cerebral edema (possible) ~ Respiratory arrest (rarely)

STAGES CONT. Stage IV: Stage IV: ~ Deepening coma ~ Large pupils with minimal response ~ Hepatic dysfunction (minimal)

STAGES CONT. Stage V: (very rapid onset) Stage V: (very rapid onset) ~ Deep coma ~ Seizures ~ Respiratory failure ~ Flaccidity ~ Extremely high blood amonia ~ Death

911 CALL Seizure activity Seizure activity Loss of consciousness Loss of consciousness General malaise General malaise N/V N/V

DIAGNOSIS There is no test for Reye’s syndrome. There is no test for Reye’s syndrome. Testing for Reye’s usually starts with blood and urine tests. Testing for Reye’s usually starts with blood and urine tests. Spinal tap- rule out other diseases with similar S/S. Spinal tap- rule out other diseases with similar S/S. Liver biopsy- rule out other liver disorders. Liver biopsy- rule out other liver disorders. CT or MRI- rule out other causes of the behavioral changes. CT or MRI- rule out other causes of the behavioral changes.

DIFFERNTIAL DIAGNOSIS Inborn metabolic disorders Inborn metabolic disorders Viral encephalitis Viral encephalitis Drug overdose or poisoning Drug overdose or poisoning Head trauma Head trauma Hepatic failure Hepatic failure Meningitis Meningitis Renal failure Renal failure

TREATMANT Usually treated in the hospital setting. Usually treated in the hospital setting. Care is mainly supportive. Care is mainly supportive. IV fluids- glucose & electrolyte solution with sodium, potassium, and chloride. IV fluids- glucose & electrolyte solution with sodium, potassium, and chloride. Insulin- increase sugar metabolism Insulin- increase sugar metabolism Corticosteroids- to reduce brain swelling. Corticosteroids- to reduce brain swelling. Diuretics- increase fluid loss through urination. Diuretics- increase fluid loss through urination. Ventilator- to assist with breathing. Ventilator- to assist with breathing.

RECOVERY/PROGNOSIS Directly related to the duration of cerebral dysfunction. Directly related to the duration of cerebral dysfunction. Severity and rate of the progression of the coma. Severity and rate of the progression of the coma. Severity of increased ICP. Severity of increased ICP. Fatality rates average 21% percent but can range from 80% in patients in stage IV or V. Fatality rates average 21% percent but can range from 80% in patients in stage IV or V.

RECOVERY/PROGNOSIS If they recover the prognosis is good, and reoccurance is rare. If they recover the prognosis is good, and reoccurance is rare. However incidence of neurologic sequela, ie mental retardation, seizure disorders, is as high as 30% among those who developed seizures or decerebrate posturing during their illness. However incidence of neurologic sequela, ie mental retardation, seizure disorders, is as high as 30% among those who developed seizures or decerebrate posturing during their illness.

RESOURCES Merch Manual, 18 th edition Merch Manual, 18 th edition Mayo Clinic, syndrome Mayo Clinic, syndromewww.mayoclinic.com/print/reyes- syndromewww.mayoclinic.com/print/reyes- syndrome Wikipedia, Wikipedia, Kids Health, Reye’s Syndrome Kids Health, Reye’s Syndrome American Liver Foundation, American Liver Foundation, The Western Journal of Medicine, Reye’s Syndrome The Western Journal of Medicine, Reye’s Syndrome Taber’s Cyclopedic Medical Dictionary Taber’s Cyclopedic Medical Dictionary All sources where used throughout the presentation.