Severe hypertriglyceridemia, often linked to LPL deficiency, is managed with ultra-low-fat diets, lipid-lowering drugs, and novel therapies targeting lipoprotein assembly.
Severe hypertriglyceridemia management requires a combination of ultra-low-fat diet and pharmacotherapy, with novel agents targeting lipoprotein assembly emerging as potential options.
Severe hypertriglyceridemia is associated with acute pancreatitis and can be a manifestation of lipoprotein lipase (LPL) deficiency. It is associated with a spectrum of disorders, ranging from heterozygous LPL deficiency allied with environmental factors to rare severe cases of homozygous LPL deficiency. The genes associated with reduced LPL activity include LPL, its cofactor apoC-2, a controlling protein apoA-5 and the LPL receptor GPI-HBP1. The effects of mutations are exacerbated by environmental factors such as diet, pregnancy and insulin resistance. Treatment of clinical LPL deficiency is by ultra-low-fat diet along with the use of fibrates, omega-3 fatty acids, niacin, statins and insulin-sensitizing therapies, depending on the extent of residual LPL activity. Novel therapies that target lipoprotein particle assembly through the antisense oligonucleotides or by interference with triglyceride-loading microsomal transport protein inhibitors offer new potential options for treating hypertriglyceridemia.
Viljoen et al. (Thu,) conducted a review in Severe hypertriglyceridemia. Lipid-lowering therapies and diet was evaluated. Severe hypertriglyceridemia, often linked to LPL deficiency, is managed with ultra-low-fat diets, lipid-lowering drugs, and novel therapies targeting lipoprotein assembly.