The Chylomicronemia Syndrome Is Most Often Multifactorial: A Narrative Review of Causes and Treatment.

Abstract

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.

• Authors: Alan Chait, Robert H Eckel
• Journal: Annals of internal medicine (Ann Intern Med) Vol. 170 Issue 9 Pg. 626-634 (05 07 2019) ISSN: 1539-3704 [Electronic] United States
• PMID: 31035285 (Publication Type: Journal Article, Review)

Chemical References

• Angiopoietins
• Apolipoproteins
• Chylomicrons
• Fatty Acids, Omega-3
• Fibric Acids
• GPIHBP1 protein, human
• Hypolipidemic Agents
• Oligonucleotides
• Receptors, Lipoprotein
• Lipoprotein Lipase

Topics

• Algorithms
• Angiopoietins (metabolism)
• Apolipoproteins (antagonists & inhibitors, metabolism)
• Cardiovascular Diseases (etiology, prevention & control)
• Chylomicrons (metabolism)
• Fatty Acids, Omega-3 (therapeutic use)
• Fibric Acids (therapeutic use)
• Genetic Predisposition to Disease
• Humans
• Hyperlipoproteinemia Type I (etiology, metabolism, therapy)
• Hypertriglyceridemia (etiology, therapy)
• Hypolipidemic Agents (therapeutic use)
• Lipodystrophy, Familial Partial (complications)
• Lipoprotein Lipase (metabolism)
• Mutation
• Oligonucleotides (therapeutic use)
• Pancreatitis (etiology, prevention & control)
• Receptors, Lipoprotein (genetics)
• Risk Factors