Familial chylomicronemia syndrome: Bringing to life dietary recommendations throughout the life span

Familial chylomicronemia syndrome (FCS) is a rare autosomal recessive disorder with loss of function mutations of lipoprotein lipase resulting in hypertriglyceridemia and accumulation of chylomicrons in plasma, often leading to acute pancreatitis. The mainstay of treatment is a specialized very-low-...

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Bibliographic Details
Published in:Journal of clinical lipidology 2018-07, Vol.12 (4), p.908-919
Main Authors: Williams, Lauren, Rhodes, Katherine S., Karmally, Wahida, Welstead, Lori A., Alexander, Lori, Sutton, Lindsey
Format: Article
Language:English
Subjects:
Chylomicronemia
Dietary recommendations
Familial chylomicronemia syndrome (FCS)
Hyperlipoproteinemia
Hypertriglyceridemia
Lipoprotein lipase deficiency
Low-fat diet
Pancreatitis
Pediatrics
Type 1 hyperlipoproteinemia
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Summary:Familial chylomicronemia syndrome (FCS) is a rare autosomal recessive disorder with loss of function mutations of lipoprotein lipase resulting in hypertriglyceridemia and accumulation of chylomicrons in plasma, often leading to acute pancreatitis. The mainstay of treatment is a specialized very-low-fat diet. Even adhering to the diet, some patients may experience high triglycerides and pancreatitis. There currently are no comprehensive dietary guidelines. To report best practices and develop comprehensive dietary guidelines for nutrition therapy in patients with FCS. Registered dietitian nutritionists (RDNs) convened to develop this report based on experience treating patients with FCS and a review of current literature on the topic. One author provided a patient perspective of living with FCS. This report provides guidelines and rationales for nutrition therapy associated with FCS across the life span. The top global guidelines are to (1) limit fat to
ISSN:1933-2874
1876-4789
DOI:10.1016/j.jacl.2018.04.010