Familial chylomicronemia syndrome: Bringing to life dietary recommendations throughout the life span
| Autor: | patients, Lori Alexander, Lori A. Welstead, Katherine S. Rhodes, Lindsey Sutton, Lauren Williams, Wahida Karmally |
|---|---|
| Rok vydání: | 2018 |
| Předmět: |
medicine.medical_specialty
Endocrinology Diabetes and Metabolism Longevity Guidelines as Topic 030204 cardiovascular system & hematology 03 medical and health sciences Lipoprotein lipase deficiency 0302 clinical medicine Internal Medicine Humans Medicine 030212 general & internal medicine Medical nutrition therapy Intensive care medicine Diet Fat-Restricted Life Style Nutrition and Dietetics Life span business.industry Hypertriglyceridemia Familial Chylomicronemia medicine.disease Pancreatitis High triglycerides Acute pancreatitis Hyperlipoproteinemia Type I lipids (amino acids peptides and proteins) Nutrition Therapy Energy Intake Cardiology and Cardiovascular Medicine business |
| Zdroj: | Journal of Clinical Lipidology. 12:908-919 |
| ISSN: | 1933-2874 |
| DOI: | 10.1016/j.jacl.2018.04.010 |
| Popis: | 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 to15 to 20 g per day (10%-15% of total daily energy intake); (2) meet recommendations for essential fatty acids: α-linolenic acid and linoleic acid; (3) choose complex carbohydrate foods while limiting simple and refined carbohydrate foods; (4) supplement with fat-soluble vitamins, minerals, and medium-chain triglyceride oil, as needed; (5) adjust calories for weight management. Recommended foods include vegetables, whole grains, legumes, lean protein foods, fruits in limited amounts, and fat-free milk products without added sugars. Foods to avoid include alcohol and products high in sugar.These patient-centered nutrition guidelines provide guidance to help patients adhere to the recommended diet and optimize nutritional needs. |
| Databáze: | OpenAIRE |
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