Familial chylomicronemia syndrome is a rare inherited disease causing severe hypertriglyceridemia and pancreatitis, primarily treated with a specialized, very low-fat diet.
Familial chylomicronemia syndrome is a rare inherited disease. Recessive mutations in genes encoding lipoprotein lipase or modulator proteins result in loss of enzyme function. As a result, the removal of triglyceride-rich lipoproteins from plasma is impaired, severe hypertriglyceridemia develops, and the risk of acute pancreatitis sharply increases. The mainstay of treatment for patients with familial chylomicronemia syndrome is a specialized, very low-fat diet.
Pshenichnikova et al. (2023) studied this question.