A 6-year-old boy is brought to the emergency department due to abrupt-onset vomiting, lethargy, and altered mental status. For 4 days, the patient has had a fever, cough, and rhinorrhea, which were treated symptomatically with aspirin. He has no prior medical conditions, and his family history is unremarkable. Vital signs are normal. The patient is stuporous and does not respond to verbal commands. Hepatomegaly is present. There is no jaundice. Laboratory results are as follows: Which of the following is the most likely cause of this patient's presentation?

Question

A 6-year-old boy is brought to the emergency department due to abrupt-onset vomiting, lethargy, and altered mental status.  For 4 days, the patient has had a fever, cough, and rhinorrhea, which were treated symptomatically with aspirin.  He has no prior medical conditions, and his family history is unremarkable.  Vital signs are normal.  The patient is stuporous and does not respond to verbal commands.  Hepatomegaly is present.  There is no jaundice.  Laboratory results are as follows:  Which of the following is the most likely cause of this patient's presentation?

Quizplus · Accepted Answer

11ec160b_3ae7_48d4_9249_716574e8bed8_MD0001_00 This patient likely has Reye syndrome, a rapidly progressive disorder characterized by acute liver failure (eg, hepatomegaly, elevated transaminases, coagulopathy) and encephalopathy. Reye syndrome most commonly occurs in children who are given salicylates (eg, aspirin) during a viral infection (eg, influenza). In susceptible patients, salicylates can damage mitochondria within hepatocytes and inhibit enzymes involved in fatty acid beta-oxidation, the primary metabolic pathway hepatocytes depend on to maintain normal cellular function. Salicylate-induced hepatic injury prevents the liver from keeping up with metabolic demands, which often increase in the setting of a viral infection and predisposes patients to the development of acute liver failure. Patients with Reye syndrome typically have vomiting and encephalopathy (eg, lethargy, altered mental status) related to the buildup of ammonia from liver failure. Seizures and coma may also occur. In addition to hyperammonemia, laboratory analysis typically shows markedly elevated aminotransferases, as seen in this patient. In addition, PT and PTT are often prolonged due to impaired hepatic synthetic function, but bilirubin is generally normal to slightly elevated. There is no specific treatment for Reye syndrome other than supportive care. (Choice A) Alpha-1 antitrypsin (A1A) deficiency causes abnormal A1A protein folding and accumulation within hepatocytes. Although it can cause chronic liver disease in childhood, this patient's acute hepatic dysfunction and encephalopathy after salicylate exposure is more characteristic of Reye syndrome. (Choice B) Viral hepatitis induces a robust CD8⁺ T-cell response against infected hepatocytes, which typically leads to elevations in aminotransferases. Although viral hepatitis may cause acute liver failure, it would be associated with increased bilirubin and jaundice (especially with marked AST/ALT elevation). (Choice C) Acetaminophen overdose can cause acute liver failure through the depletion of intracellular glutathione, leading to an overabundance of hepatotoxic metabolites (eg, N-acetyl-p-benzoquinone imine). Salicylates do not deplete intracellular glutathione like acetaminophen does. (Choice D) Reperfusion after ischemic insult to the liver can create hepatotoxic reactive oxygen species, such as superoxide. Reperfusion injury occurs in the setting of profound hypotension (eg, septic shock), which is not present in this patient. Educational objective: Reye syndrome is characterized by acute liver failure (eg, hepatomegaly, elevated aminotransferases, coagulopathy) and rapidly progressive encephalopathy related to hyperammonemia. It usually develops in susceptible children during a viral infection after administration of salicylates (eg, aspirin), which cause damage to the mitochondria and impair fatty acid beta-oxidation within hepatocytes. __________ References: A case of Reye syndrome caused by influenza A virus. (http://www.ncbi.nlm.nih.gov/pubmed/30559633)

A 6-Year-Old Boy Is Brought to the Emergency Department Due