A Comprehensive Update on the Chylomicronemia Syndrome.

Frontiers in Endocrinology
Ronald B Goldberg, Alan Chait

Abstract

The chylomicronemia syndrome is characterized by severe hypertriglyceridemia and fasting chylomicronemia and predisposes affected individuals to acute pancreatitis. When due to very rare monogenic mutations in the genes encoding the enzyme, lipoprotein lipase, or its regulators, APOC2, APOA5, GPIHBP1, and LMF1, it is referred to as the familial chylomicronemia syndrome. Much more frequently, the chylomicronemia syndrome results from a cluster of minor genetic variants causing polygenic hypertriglyceridemia, which is exacerbated by conditions or medications which increase triglyceride levels beyond the saturation point of triglyceride removal systems. This situation is termed the multifactorial chylomicronemia syndrome. These aggravating factors include common conditions such as uncontrolled diabetes, overweight and obesity, alcohol excess, chronic kidney disease and pregnancy and several medications, including diuretics, non-selective beta blockers, estrogenic compounds, corticosteroids, protease inhibitors, immunosuppressives, antipsychotics, antidepressants, retinoids, L-asparaginase, and propofol. A third uncommon cause of the chylomicronemia syndrome is familial forms of partial lipodystrophy. Development of pancreatitis is...Continue Reading

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Citations

Jan 23, 2021·Current Opinion in Endocrinology, Diabetes, and Obesity·Gissette Reyes-Soffer
Jun 20, 2021·Current Atherosclerosis Reports·Germán D CarrasquillaTuomas O Kilpeläinen
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Sep 18, 2021·The Canadian Journal of Cardiology·Kristen M KidsonJasmine Grewal

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Methods Mentioned

BETA
FCS
pharmacotherapy
Replacement
antisense oligonucleotide

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