Case history
Case history #1
A 6-year-old girl presents to the ER with a history of vomiting for 2 days. Five days previously she had an episode of body ache, vomiting, and fever for which she received aspirin. Since then, the patient's vomiting has increased in frequency and severity. On examination, she seems weak, irritable, and confused. She has tachypnea and tachycardia. Laboratory investigations show hypoglycemia with elevation in AST, ALT, and ammonia.
Case history #2
A 7-month-old girl presents to the ER with a 1-day history of vomiting, irritability, and worsening lethargy. The family history is significant for one brother who died of sudden infant death syndrome (SIDS). Physical exam yields a dehydrated and somnolent infant with hepatomegaly. Laboratory investigations show marked hypoglycemia and acidosis with an elevated anion gap, but no ketones in the urine. There is also elevation in AST, ALT, and ammonia. In the ER, the patient develops seizures and becomes apneic, requiring intubation.
Other presentations
Case reports have shown that patients with Reye syndrome do not necessarily present with classic signs or symptoms, or have a precise order to their occurrence.[3] Therefore, in light of the decreased incidence of classic Reye syndrome, it is essential to maintain a low threshold for considering a Reye-like syndrome induced by an undiagnosed inborn error of metabolism. For instance, an atypical prodrome with sudden onset of symptoms, age <2 years, history of Reye-like syndrome, or siblings with a similar illness or sudden infant death syndrome (SIDS) could suggest a possible underlying metabolic disorder.[4]
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