Department of Pediatric Gastroenterology, Hepatology and Nutrition, Intestinal Failure Rehabilitation Center, National Reference Center for Rare Digestive Diseases, Hospital Necker-Enfants Malades, Paris-Descartes Medical School at the University of Sorbonne-Paris-Cité, Paris, France.
Department of Pediatric Surgery, Division of Pediatric Gastroenterology and Nutrition, Xin Hua Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China.
JPEN J Parenter Enteral Nutr. 2020 Feb;44 Suppl 1:S55-S67. doi: 10.1002/jpen.1762.
The ability to deliver nutrients via parenteral nutrition (PN) has markedly improved the prognosis of infants and children with intestinal failure. Technical refinements and advances in knowledge have led to the development of highly sophisticated PN solutions that are tailored to meet the needs of pediatric patients. However, children who require long-term PN have an increased risk of complications such as catheter-related sepsis, liver disease, and bone disease. Although the pathogenesis of intestinal failure associated liver disease (IFALD) is multifactorial, studies have identified a possible link between the dose of lipid emulsions based on soybean oil and cholestasis, shown to occur with a significantly higher frequency in patients receiving >1 g lipids/kg/d. Potential contributing factors include oxidative stress, high ω-6 polyunsaturated fatty acid (PUFA) and phytosterol content, and relatively low α-tocopherol levels. Lipid emulsions containing fish oil offer potential advantages compared with traditional emulsions with a high soybean oil content, such as decreased ω-6 and increased ω-3 PUFA concentrations, high concentrations of α-tocopherol, and reduced phytosterol content. Studies in PN-dependent children at risk for IFALD have shown that lipid emulsions containing fish oil reduce the risk of cholestasis and improve biochemical measures of hepatobiliary function compared with pure soybean oil emulsions. This review summarizes evidence regarding the role of lipid emulsions in the management of pediatric patients with intestinal failure requiring long-term PN, with a particular focus on the prevention and treatment of IFALD.
肠外营养(PN)能够提供营养,显著改善了肠衰竭患儿的预后。技术的改进和知识的进步促进了高度复杂的 PN 解决方案的发展,这些解决方案能够满足儿科患者的需求。然而,需要长期 PN 的儿童发生并发症的风险增加,如导管相关败血症、肝病和骨病。尽管肠衰竭相关肝病(IFALD)的发病机制是多因素的,但研究已经确定了以大豆油为基础的脂肪乳剂剂量与胆汁淤积之间可能存在联系,在接受>1 g/kg/d 脂肪乳剂的患者中,胆汁淤积的发生率明显更高。潜在的促成因素包括氧化应激、高 ω-6 多不饱和脂肪酸(PUFA)和植物固醇含量以及相对较低的 α-生育酚水平。与含有高大豆油含量的传统乳剂相比,含有鱼油的脂肪乳剂具有潜在优势,例如 ω-6 降低和 ω-3 PUFA 增加、α-生育酚浓度高和植物固醇含量降低。在有 IFALD 风险的依赖 PN 的儿童中进行的研究表明,与纯大豆油乳剂相比,含有鱼油的脂肪乳剂可降低胆汁淤积的风险,并改善肝胆功能的生化指标。这篇综述总结了关于在需要长期 PN 的肠衰竭儿科患者管理中脂肪乳剂作用的证据,特别关注 IFALD 的预防和治疗。