A 5-year-old previously healthy girl presents with 24 hours of forceful nonbloody, nonbilious emesis, lethargy, and fevers. Physical examination reveals a nontoxic, interactive, well-developed, well-nourished girl with signs of mild dehydration, a benign abdominal examination, and a nonfocal neurologic examination. Pertinent laboratory findings include mild hyponatremia, an anion gap metabolic acidosis, and hypoglycemia. Urinalysis shows marked ketonuria and glucosuria following an intravenous dextrose bolus. Acetaminophen, salicylate, and urine toxicology screens are negative, as is her state newborn screen. Further social history reveals a well-adjusted adopted child with poor school performance and a mostly self-restricted vegan diet.

During her hospitalization, she develops visual hallucinations, gait abnormalities, and an episode of enuresis. Her emesis, progressive altered mental status, initial hypoglycemia, and prolonged ketonuria prompt abdominal imaging to evaluate for small bowel obstruction as well as a metabolic and endocrine evaluation to look for potential causes of ketotic hypoglycemia. Results of abdominal radiography;...

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