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

Abstract

These patient-centered nutrition guidelines provide guidance to help patients adhere to the recommended diet and optimize nutritional needs.

Review of current evidence and clinical recommendations on the effects of low-carbohydrate and very-low-carbohydrate (including ketogenic) diets for the management of body weight and other cardiometabolic risk factors: A scientific statement from the National Lipid Association Nutrition and Lifestyle Task Force

“…In these patients, a VLCHF/KD could cause chylomicronemia and precipitate pancreatitis. Patients with hyperchylomicronemia must adhere to a very-low-fat diet (10-15% TDE or ,15-20 g fat/d); 105 thus, a VLCHF/ KD is contraindicated in these patients until the chylomicronemia is cleared, and then, only under close observation.…”

“…Because VLCHF/KDs are contraindicated in patients with a history of hypertriglyceridemia-associated acute pancreatitis, severe hypertriglyceridemia, or inherited severe hypercholesterolemia, they are not recommended for these patients. [101][102][103][104][105] III: Potential Harm…”

Management of hypertriglyceridemia

“…However, these general recommendations need to be modified in patients with extreme hypertriglyceridemia due to FCS who need restriction of dietary fat to below 10-15% of total energy intake (15-20 g/day). 126 Limited data also suggest a benefit of medium chain triglycerides in these patients, as they are absorbed and transported without being incorporated into chylomicrons. 127 128 Exercise Regular aerobic exercise not only promotes weight loss and physical fitness but has been shown to significantly reduce postprandial triglyceride response.…”

The Prevalence of Probable Familial Chylomicronemia Syndrome in a Southern California Population

“…FCS management: FCS is managed primarily by diet modification. Williams et al (25) provided recommendations for adopting a very-low-fat diet by either consuming less than 15 to 20 g of fat per day or restricting total dietary fat to less than 10% to 15% of total daily caloric intake individualizing fat restrictions to meet the daily essential fatty acid (EFA) needs of 2% to 4% daily caloric intake of α-linolenic acid (16) mRNA. This has shown efficacy in lowering total cholesterol and TG lipids in preclinical trials DOI:10.4158/EP-2020-0135 © 2020 AACE.…”