A 6-year-old female presents with emesis and fatigue. The patient’s mother reports her daughter has a history of Maple Syrup Urine Disease. The day before presentation, on routine urine check, the patient had 2+ ketones in her urine. The patient consumed a protein restricted diet, which initially cleared the ketones. However, on the morning of presentation, the patient began vomiting, appeared more lethargic and had 4+ ketones in her urine. On arrival, she was tachycardic and tachypneic. Her exam was significant for a tired appearing female with dry mucous membranes and poor skin turgor.

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