Familial Chylomicronemia Syndrome
Dietary guidelines for all patients with FCS
1: Adopt 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 or
- individualizing fat restrictions to meet the daily essential fatty acid (EFA) needs of 2% to 4% daily caloric intake of a-linolenic acid (ALA) and linoleic acid (LA).
2: Meet EFA requirements of 2% to 4% of daily calories in the very-low-fat diet.
3: Medium-chain triglyceride (MCT) oil may be used as a source of fatty acids not transported via chylomicrons and therefore allow for adjustment of caloric intake and macronutrient composition of the diet.
4: Include fat-free or low-fat proteinrich foods with each meal and snack.
5: Choose complex carbohydrate foods, limit simple and refined carbohydrate foods, and avoid foods with added sugars.
Monitor levels of fat-soluble vitamins (A, D, E, K) and minerals regularly, supplementing as needed.
7: Encourage nutrient-dense foods as a component of the very-low-fat diet.
8: Distribute calorie and fat intake throughout the day while avoiding excess caloric intake.
9: Abstain from consuming alcohol.
10: Engage in at least 150 minutes of physical activity per week to improve overall health outcomes.
11: Drink sufficient water and stay adequately hydrated.
12: Develop an individualized, multidisciplinary, and collaborative management plan to address all aspects of FCS disease management.
Dietary guidelines by population
Assess the nutritional adequacy of the diet by monitoring developmental and cognitive milestones in pediatric patients with FCS.
Infants diagnosed with FCS require a special low-fat, low-LCT, high-MCT formula or skimmed and fortified expressed breast milk.
Transition children aged 12 to 24 months to skim milk and age-appropriate, low-fat solid foods.
School-aged children and adolescents
Provide families and caregivers with specific meals and/or guidelines for grams of fat per meal and snack. The recommended age-appropriate protein consumption should be encouraged.
Pregnancy and FCS
The goal for pregnant patients is to maintain fasting TG levels less than 500 mg/dL by following a very-low-fat diet, consume adequate calories to support pregnancy, and maintain electrolyte and fluid balance. Regular monitoring of TGs and diet adjustment during pregnancy is important.
Establish a multidisciplinary approach to develop an individualized management plan for FCS patients with diabetes.
Patients should be adequately hydrated after episodes of acute pancreatitis and should continue to follow the restricted very-low-fat diet.
Patients with FCS at risk or suffering from chronic pancreatitis should continue to follow the very-low-fat diet outlined previously.
In the event of diminished exocrine function and malabsorption, provision of pancreatic enzymes may be indicated with each meal and snack.
Familial Chylomicronemia Syndrome
July 1, 2018
Last Updated Month/Year
March 16, 2023
External Publication Status
Country of Publication
To report best practices and develop comprehensive dietary guidelines for nutrition therapy in patients with Familial chylomicronemia syndrome
Target Patient Population
Patients with familial chylomicronemia syndrome
Female, Male, Adolescent, Adult, Child, Infant, Older adult
Health Care Settings
Nurse, nurse practitioner, physician, physician assistant
D050171 - Dyslipidemias, D044623 - Nutrition Therapy, D002914 - Chylomicrons, D006950 - Hyperlipidemia, Familial Combined, D004032 - Diet, D004035 - Diet Therapy, D008072 - Hyperlipoproteinemia Type I, D006951 - Hyperlipoproteinemias
diet, dyslipidemia, nutritional care, Familial chylomicronemia, hyperlipoproteinemias