An unusual cause for ketoacidosis

A 22-year-old male developed a severe degree of metabolic acidosis (plasma pH 7.20, bicarbonate 8 mmol/l), with a large increase in the plasma anion gap (26 mEq/l). Ketoacidosis was suspected because of the odour of acetone on his breath and a positive qualitative test for acetone in plasma (to a 1:4 dilution). Later, his plasma beta-hydroxybutyrate concentration was found to be 4.5 mmol/l. After receiving an infusion of 1 l of half-isotonic saline and 1 l of 5% dextrose in water over 24 h, as well as curtailing his large oral intake of sweetened beverages, all blood tests became normal. Diabetic ketoacidosis, alcoholic ketoacidosis, starvation ketosis and hypoglycaemic ketoacidosis were all ruled out, and his toxin screen was negative for salicylates. Finding another possible cause for ketoacidosis became the focus of this case.