Harvard Medical School, Vascular Biology Program, Boston Children's Hospital, Boston, MA, USA.
Department of Surgery, Harvard Medical School, Boston Children's Hospital, Boston, MA, USA.
Expert Opin Drug Saf. 2021 Mar;20(3):307-320. doi: 10.1080/14740338.2021.1867099. Epub 2020 Dec 30.
: Intestinal failure-associated liver disease (IFALD) refers to hepatic dysfunction that results from prolonged parenteral nutrition (PN) use. IFALD is multifactorial in origin and remains a major cause of morbidity and mortality. Prior to 2004, IFALD was associated with mortality as high as 90% in infants who remained on PN greater than 1 year. The advent of new strategies for intravenous lipid emulsion (ILE) administration and improved catheter care now allow many patients to remain on PN and recover from this once fatal condition. Several additional treatment modalities are often used to further improve outcomes for IFALD patients and they are reviewed here.: The etiology of IFALD is presented, as well as the rationale behind the use of ILEs that contain fish oil. Other management strategies are addressed, including the effects of several pharmacologic and nutritional interventions.: Like its etiology, the management of IFALD is multifactorial. Prompt recognition of patients at risk, avoiding macronutrient excess, and preventing central line associated bloodstream infections will improve outcomes. In patients who develop IFALD, the use of fish oil monotherapy seems to be efficacious. The most effective intervention, however, continues to be discontinuation of PN and achieving full enteral feedings.
肠衰竭相关肝病(IFALD)是指由于长期使用肠外营养(PN)而导致的肝功能障碍。IFALD 是多因素引起的,仍然是发病率和死亡率的主要原因。在 2004 年之前,对于接受 PN 治疗超过 1 年的婴儿,IFALD 与高达 90%的死亡率相关。静脉内脂肪乳剂(ILE)给药新策略的出现和改进的导管护理现在允许许多患者继续接受 PN 治疗并从这种曾经致命的疾病中恢复过来。这里还介绍了其他几种治疗方法,这些方法通常用于进一步改善 IFALD 患者的预后。IFALD 的病因,以及使用含鱼油的 ILE 的基本原理。还讨论了其他管理策略,包括几种药物和营养干预的影响。与病因一样,IFALD 的治疗也是多因素的。及时识别有风险的患者,避免大量营养素过剩,并预防中心静脉相关血流感染将改善预后。对于发生 IFALD 的患者,鱼油单药治疗似乎有效。然而,最有效的干预措施仍然是停止 PN 并实现完全肠内喂养。