MEDizzy - A 5-year-old girl presents with severe vomiting that

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Gastrointestinal System

A 5-year-old girl presents with severe vomiting that developed suddenly 5 days after she had a viral infection. Upon questioning, her parents indicate that she was given aspirin for several days to treat a fever that occurred with the viral illness. She is hospitalized and quickly develops signs of cerebral edema. Microscopic examination of a liver biopsy from this young girl reveals marked microvesicular steatosis. What is the best diagnosis?

ExplanationReye syndrome (RS) is an acute postviral illness that is seen mainly in children who are given acetylsalicylic acid (aspirin) for their fever. RS is characterized by encephalopathy, microvesicular fatty change of the liver, and widespread mitochondrial injury. Electron microscopy (EM) reveals large budding or branching mitochondria. The mitochondrial injury results in decreased activity of the citric acid cycle and urea cycle and defective β-oxidation of fats, which then leads to the accumulation of serum fatty acids. The typical patient presents several days after a viral illness with vomiting. RS is associated with hyperammonemia, elevated serum free fatty acids, and salicylate ingestion.