Familial chylomicronemia syndrome (FCS) is a rare, life-threatening disease.
People with FCS have a genetic disorder that severely impairs the body’s ability to remove triglycerides (TGs) from the blood stream. This often causes people with FCS to have extremely high triglyceride levels, greater than 880 mg/dL or 10 mmol/L. Their blood may even appear fatty or milky in the lab after it is drawn due to this buildup of TGs.8
Even a little fat in food can make someone with FCS ill. The condition leads to the build-up of triglyceride-containing particles called chylomicrons in the blood, which can cause inflammation of the pancreas, called acute pancreatitis. People with FCS have very high risk for pancreatitis and must carefully restrict fats in their diet.38
Other names for FCS can include8:
Familial Hyperlipidemia, Familial Hypertriglyceridemia, Lipoprotein Lipase Deficiency (LPLD), Fredrickson Type 1 Lipoproteinemia.
