Internal medicine

Acute pancreatitis

Obesity

Lipoprotein lipase

Endocrinology

Viral load

Adipose tissue

Pancreatitis

Immunology

Hypertriglyceridemia

Cholesterol

Lipodystrophy

Triglyceride

Antiretroviral therapy

Metabolic syndrome

Abdominal pain

Human immunodeficiency virus (HIV)

Medicine

Annals of Internal Medicine

The chylomicronemia syndrome occurs when triglyceride levels are severely elevated (usually >16.95 mmol/L [1500 mg/dL]) and is characterized by such clinical features as abdominal pain, acute pancreatitis, eruptive xanthomas, and lipemia retinalis. It may result from 1 of 3 conditions: the presence of secondary forms of hypertriglyceridemia concurrent with genetic causes of hypertriglyceridemia, termed multifactorial chylomicronemia syndrome (MFCS); a deficiency in the enzyme lipoprotein lipase and some associated proteins, termed familial chylomicronemia syndrome (FCS); or familial partial lipodystrophy. Most chylomicronemia syndrome cases are the result of MFCS; FCS is very rare. In all these conditions, triglyceride-rich lipoproteins accumulate because of impaired plasma clearance. This review describes the 3 major causes of the chylomicronemia syndrome; their consequences; and the approaches to treatment, which differ considerably by group.

The Chylomicronemia Syndrome Is Most Often Multifactorial