REYE’S SYNDROME BY: JENNIFER DELANEY. OBJECTIVES HISTORY HISTORY ETIOLOGY ETIOLOGY WHAT IT DOES WHAT IT DOES SIGN & SYMPTOMS SIGN & SYMPTOMS STAGES STAGES.

REYE’S SYNDROMEREYE’S SYNDROME

BY: JENNIFER DELANEYBY: JENNIFER DELANEY

OBJECTIVESOBJECTIVES HISTORYHISTORY ETIOLOGYETIOLOGY WHAT IT DOESWHAT IT DOES SIGN & SYMPTOMSSIGN & SYMPTOMS STAGESSTAGES WHAT WE WILL BE CALLED FORWHAT WE WILL BE CALLED FOR DIAGNOSISDIAGNOSIS DIFFERNTIAL DIAGNOSISDIFFERNTIAL DIAGNOSIS TREATMENTTREATMENT RECOVERY RATERECOVERY RATE

HISTORYHISTORY

Was named after Dr. R. Douglas Reye.Was named after Dr. R. Douglas Reye. Also discovered with fellow Australians Also discovered with fellow Australians

Dr. Graeme Morgan & Dr. Jim Baral.Dr. Graeme Morgan & Dr. Jim Baral. Study was first published in 1963 in Study was first published in 1963 in

the British Medical Journal.the British Medical Journal. The first occurrence may have The first occurrence may have

occurred prior in 1929.occurred prior in 1929.

HISTORY CONT.HISTORY CONT.

In 1963 Dr. George Johnson with In 1963 Dr. George Johnson with colleagues published an investigation colleagues published an investigation of an outbreak in 16 children with of an outbreak in 16 children with influenza B that developed influenza B that developed neurological problems after the virus. neurological problems after the virus. 4 of the children had symptoms that 4 of the children had symptoms that were very similar to those of Reye’s.were very similar to those of Reye’s.

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

ETIOLOGYETIOLOGY

Precise reason why it occurs is Precise reason why it occurs is unknown.unknown.

It’s called a syndrome because the It’s called a syndrome because the clinical features that are used to clinical features that are used to diagnosis it are very broad.diagnosis it are very broad.

Major presentation that occurs in the Major presentation that occurs in the United States occurs after viral-flu United States occurs after viral-flu like illness or after varicella. like illness or after varicella.

ETIOLOGY CONT.ETIOLOGY CONT.

The involvement of salicylates and The involvement of salicylates and Reye’s was first suggested in 1962, Reye’s was first suggested in 1962, which was before Reye’s was a which was before Reye’s was a clinical entity.clinical entity.

Reports showed that in 4 cases of Reports showed that in 4 cases of infants that had varicella infections infants that had varicella infections who had received ASA, 2 of the who had received ASA, 2 of the infants developed symptoms infants developed symptoms compatible with Reye’scompatible with Reye’s

ETIOLOGY CONT.ETIOLOGY CONT.

Another study done in 1975 reviewed Another study done in 1975 reviewed 56 patients with pathologically 56 patients with pathologically confirmed Reye’s. 53 of whom had confirmed Reye’s. 53 of whom had some sort sacilcylate ingestion.some sort sacilcylate ingestion.

In 1977 yet another study was done In 1977 yet another study was done with 175 confirmed Reye’s syndrome with 175 confirmed Reye’s syndrome which showed that 78% of the which showed that 78% of the patients had received ASA.patients had received ASA.

ETIOLOGY CONT.ETIOLOGY CONT.

These plus many other reports have These plus many other reports have led to a great deal of talk about ASA led to a great deal of talk about ASA and children. and children.

This lead to a significant decrease in This lead to a significant decrease in the occurrence after 1970.the occurrence after 1970.

It is now not recommended to give It is now not recommended to give ASA to kids 18 and under without ASA to kids 18 and under without doctor’s approval.doctor’s approval.

Progresses through 5 stagesProgresses through 5 stages

WHAT IT DOESWHAT IT DOES

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

SIGNS & SYMPTOMSSIGNS & SYMPTOMS

Usually show up 3-4 days after a viral Usually show up 3-4 days after a viral illness such as influenza B and illness such as influenza B and varicella.varicella.

Usually occurs during the Fall & Usually occurs during the Fall & Winter months.Winter months.

Predominantly occurs in children.Predominantly occurs in children.

S/S CONT.S/S CONT. Continuous N/VContinuous N/V Sleepless/lethargySleepless/lethargy ConfusionConfusion IrritabilityIrritability Aggressive or Aggressive or

irrational behaviorirrational behavior Weakness or hemi-Weakness or hemi-

paresisparesis SeizuresSeizures Loss of consciousnessLoss of consciousness

Sluggish respiratory Sluggish respiratory raterate

Dilated pupilsDilated pupils Enlarged liver-with Enlarged liver-with

no jaundice presentno jaundice present EncephalitisEncephalitis Increased ICPIncreased ICP Posturing-Posturing-

decerebrate or decerebrate or decorticatedecorticate

STAGESSTAGES

Stage I:Stage I:

~ Persistent, heavy N/V~ Persistent, heavy N/V

~ Generalized lethargy~ Generalized lethargy

~ Confusion~ Confusion

~ Nightmares~ Nightmares

STAGES CONT.STAGES CONT.

Stage II:Stage II:

~ Stupor (caused by minor brain ~ Stupor (caused by minor brain swelling)swelling)

~ Hyperventilation~ Hyperventilation

~ Fatty liver (biopsy)~ Fatty liver (biopsy)

~ Hyperactive reflexes~ Hyperactive reflexes

STAGES CONT.STAGES CONT.

Stage III:Stage III:

~ Continuation of stages I & II~ Continuation of stages I & II

~ Coma (possible)~ Coma (possible)

~ Cerebral edema (possible)~ Cerebral edema (possible)

~ Respiratory arrest (rarely)~ Respiratory arrest (rarely)

STAGES CONT.STAGES CONT.

Stage IV:Stage IV:

~ Deepening coma~ Deepening coma

~ Large pupils with minimal ~ Large pupils with minimal responseresponse

~ Hepatic dysfunction (minimal) ~ Hepatic dysfunction (minimal)

STAGES CONT.STAGES CONT.

Stage V: (very rapid onset)Stage V: (very rapid onset)

~ Deep coma~ Deep coma

~ Seizures~ Seizures

~ Respiratory failure~ Respiratory failure

~ Flaccidity~ Flaccidity

~ Extremely high blood amonia~ Extremely high blood amonia

~ Death~ Death

911 CALL911 CALL

Seizure activitySeizure activity Loss of consciousnessLoss of consciousness General malaiseGeneral malaise N/VN/V

DIAGNOSISDIAGNOSIS

There is no test for Reye’s syndrome.There is no test for Reye’s syndrome. Testing for Reye’s usually starts with Testing for Reye’s usually starts with

blood and urine tests.blood and urine tests. Spinal tap- rule out other diseases Spinal tap- rule out other diseases

with similar S/S.with similar S/S. Liver biopsy- rule out other liver Liver biopsy- rule out other liver

disorders.disorders. CT or MRI- rule out other causes of CT or MRI- rule out other causes of

the behavioral changes.the behavioral changes.

DIFFERNTIAL DIAGNOSISDIFFERNTIAL DIAGNOSIS

Inborn metabolic disordersInborn metabolic disorders Viral encephalitisViral encephalitis Drug overdose or poisoningDrug overdose or poisoning Head traumaHead trauma Hepatic failureHepatic failure MeningitisMeningitis Renal failureRenal failure

TREATMANTTREATMANT

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 IV fluids- glucose & electrolyte solution

with sodium, potassium, and chloride.with sodium, potassium, and chloride. Insulin- increase sugar metabolismInsulin- increase sugar metabolism Corticosteroids- to reduce brain swelling.Corticosteroids- to reduce brain swelling. Diuretics- increase fluid loss through Diuretics- increase fluid loss through

urination.urination. Ventilator- to assist with breathing.Ventilator- to assist with breathing.

RECOVERY/PROGNOSIS RECOVERY/PROGNOSIS

Directly related to the duration of Directly related to the duration of cerebral dysfunction.cerebral dysfunction.

Severity and rate of the progression of Severity and rate of the progression of the coma.the coma.

Severity of increased ICP.Severity of increased ICP. Fatality rates average 21% percent Fatality rates average 21% percent

but can range from <2% in patients in but can range from <2% in patients in stage I, to >80% in patients in stage stage I, to >80% in patients in stage IV or V.IV or V.

RECOVERY/PROGNOSISRECOVERY/PROGNOSIS

If they recover the prognosis is good, If they recover the prognosis is good, and reoccurance is rare.and reoccurance is rare.

However incidence of neurologic However incidence of neurologic sequela, ie mental retardation, sequela, ie mental retardation, seizure disorders, is as high as 30% seizure disorders, is as high as 30% among those who developed seizures among those who developed seizures or decerebrate posturing during their or decerebrate posturing during their illness.illness.

RESOURCESRESOURCES Merch Manual, 18Merch Manual, 18thth edition edition Mayo Clinic, Mayo Clinic,

www.mayoclinic.com/print/reyes-syndrome Wikipedia, Wikipedia,

www.wikipedia.org/wiki/Reye’s_syndrome Kids Health, Reye’s SyndromeKids Health, Reye’s Syndrome American Liver Foundation, American Liver Foundation,

www.liverfoundation.org/education/info/reye The Western Journal of Medicine, Reye’s The Western Journal of Medicine, Reye’s

SyndromeSyndrome Taber’s Cyclopedic Medical DictionaryTaber’s Cyclopedic Medical Dictionary

All sources where used throughout the presentation.All sources where used throughout the presentation.