History and exam

Key diagnostic factors

common

vomiting

Patients present with persistent and profuse vomiting. May or may not have volume depletion.

altered mental status

May initially start as lethargy and proceed to irritability, delirium, combativeness, disorientation, and coma.

Other diagnostic factors

common

hyperventilation

More commonly present in younger patients.

hepatomegaly

Present in 50% of patients at the time of diagnosis.

abnormal pupillary response

May be helpful in assessing disease severity (stage): dilated/sluggish in stage 2, fixed/dilated in stage 4, unresponsive in stage 5.[2]

hyperreflexia/areflexia

Helpful in assessing disease stage. In stage 2, patients may show hyperreflexive response. Areflexia is seen in stage 5.[2]

diminished pain response

In early stages (1-2), patients are responsive to pain.[2] If unresponsive, patients are considered to be at stage 3 or higher.

seizures

May occur in late stages of disease.

uncommon

absence of, or minimal, jaundice and scleral icterus

Jaundice is typically not a feature of Reye syndrome.

Risk factors

strong

age 5-14 years

In the US, the peak ages of incidence are 5 to 14 years old, with a median of 6 years old, and mean of 7 years old.[5] Reye syndrome rarely occurs in children <1 year old or >18 years old, and rarely occurs in adults.[5][10]

white ethnicity

CDC surveillance data for 1980 to 1997 showed that 93% of patients were white, 5% were black, 2% were Asian, and 0.3% were Native American or Native Alaskan.[5]

recent viral infection

Antecedent viral illness during the three week period prior to onset of Reye syndrome was reported in 93% of patients (n=1080) for whom data was available (n=1160).[5]

Of these, 73% had influenza-like illness, 21% had varicella-zoster infection, 14% had viral gastroenteritis, and 5% had viral exanthema.[5]

weak

aspirin (acetylsalicylic acid) and other drug exposure

Aspirin is the drug classically associated with Reye syndrome and has been studied the most.[5] However, studies have not clearly supported or refuted a cause-effect relationship between aspirin intake and Reye syndrome in children.[8]

Other drugs include antiemetics, valproic acid, outdated tetracyclines, zidovudine, didanosine, and acetaminophen.

genetically predisposed individual

Data suggest that an exogenous factor contributes to the mitochondrial dysfunction in certain genetically predisposed individuals with Reye syndrome.[7]

toxin exposure

Aflatoxin, margosa oil, hypoglycin (found in unripe ackee fruit), various pesticides, polyethylene, and various insecticides have been implicated.

winter/spring presentation

In the US, an increase in the number of cases of Reye syndrome was found during the months of December through April, which correlated with the peak occurrences of most viral respiratory infections.[5] Since the 1990s, this seasonal association has diminished. Globally, a seasonal predominance has not been found.[5]

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