Risk Evaluation to Achieve Cardiovascular Health (REACH) Clinic, Cook Children's Endocrinology Clinic, Fort Worth, TX, USA.
Cardiovascular Medicine, Michigan Medicine, Ann Arbor, Michigan, USA.
J Clin Lipidol. 2018 Jul-Aug;12(4):908-919. doi: 10.1016/j.jacl.2018.04.010. Epub 2018 Apr 27.
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 <15 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.
家族性乳糜微粒血症综合征（FCS）是一种罕见的常染色体隐性遗传病，脂蛋白脂肪酶功能丧失突变导致甘油三酯升高和乳糜微粒在血浆中积聚，常导致胰腺炎。治疗的主要方法是采用专门的极低脂肪饮食。即使坚持这种饮食，一些患者仍可能出现高甘油三酯血症和胰腺炎。目前尚无综合饮食指南。
报告 FCS 患者营养治疗的最佳实践并制定综合饮食指南。
注册营养师召集会议，根据治疗 FCS 患者的经验以及对该主题现有文献的回顾，制定本报告。一位作者提供了患有 FCS 的患者的生活视角。
本报告提供了贯穿整个生命周期与 FCS 相关的营养治疗指南和基本原理。顶级全球指南包括：（1）将脂肪限制在每天<15 至 20g（<总每日能量摄入的 10%-15%）；（2）满足必需脂肪酸：α-亚麻酸和亚油酸的推荐量；（3）选择复合碳水化合物食物，同时限制简单和精制碳水化合物食物；（4）根据需要补充脂溶性维生素、矿物质和中链甘油三酯油；（5）根据体重管理调整卡路里。推荐的食物包括蔬菜、全谷物、豆类、瘦肉蛋白食物、适量的水果和无添加糖的脱脂奶制品。应避免的食物包括酒精和高糖产品。
这些以患者为中心的营养指南为帮助患者遵守推荐饮食和优化营养需求提供了指导。
