Abstract
Extreme hypertriglyceridemia is a metabolic disorder that is defined by plasma triglyceride levels exceeding 880 mg per deciliter (10 mmol per liter) caused by insufficient lipolytic clearance of triglyceride-rich lipoproteins. When this condition is caused by rare autosomal recessive mutations in lipoprotein lipase or in endogenous regulators of its activity, it is termed familial chylomicronemia syndrome (FCS), which is estimated to occur in approximately 1 to 10 persons per 1 million worldwide.1 A more prevalent form that affects approximately 1 in 600 persons is the multifactorial chylomicronemia syndrome (MCS), in which extreme elevations of plasma triglycerides are caused by rare .
Published Version
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