A 4-year-old previously healthy girl presents to the emergency department with a 1-month history of vague abdominal pain, constipation, and a 5.5-lb (2.5 kg) weight loss. She then developed polydipsia, polyuria, and fatigue for 1 week. There were no infectious or neurologic symptoms. On examination she had dry mucous membranes and a nontender abdomen without hepatomegaly. Initial investigations were diagnostic for diabetic ketoacidosis (DKA) with a blood glucose of 901 mg/dL (50 mmol/L), serum pH of 7.28, and a bicarbonate of 11.9 mEq/L (mmol/L). However, it was noted that the blood specimen was profoundly lipemic. The patient was admitted to the hospital for management of DKA and investigation for lipemia.

Due to difficulty processing the sample, serum lipids levels were not accurately measured for 3 days, but the first available measurement showed a serum triglyceride of 5,938 mg/dL (67.1 mmol/L) and a total cholesterol of 1,227 mg/dL (13.87 mmol/L). There...

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