Hyperchylomicronemia

This code represents a rare genetic disorder characterized by extremely high levels of chylomicrons in the blood, leading to severe hypertriglyceridemia. It results from a deficiency in lipoprotein lipase (LPL) or its cofactor, apolipoprotein C-II, impairing the body's ability to break down fats.

Use this code for patients diagnosed with familial chylomicronemia syndrome (FCS) or other primary causes of severe hyperchylomicronemia. Documentation should clearly indicate elevated chylomicron levels, often with triglyceride levels exceeding 1000 mg/dL, and a confirmed genetic or enzymatic defect.

• Differentiate from secondary hypertriglyceridemia caused by other conditions (e.g., diabetes, alcoholism).
• Ensure documentation specifies "hyperchylomicronemia" rather than just general hyperlipidemia.
• Consider sequencing with codes for associated complications like pancreatitis (e.g., 577.0).