Department of Gastroenterology, St James University Hospital, Leeds, United Kingdom.
Nutr Clin Pract. 2024 Oct;39(5):1003-1025. doi: 10.1002/ncp.11116. Epub 2024 Jan 21.
Intestinal failure-associated liver disease (IFALD) is a serious life-limiting complication that can occur throughout the clinical course of intestinal failure and its management by parenteral nutrition (PN). Despite this, there is a lack of a standardized definition for IFALD, which makes this insidious condition increasingly difficult to screen and diagnose in clinical practice. Attenuating the progression of liver disease before the onset of liver failure is key to improving morbidity and mortality in these patients. This requires timely detection and promptly addressing reversible factors. Although there are various noninvasive tools available to the clinician to detect early fibrosis or cirrhosis in various chronic liver disease states, these have not been validated in the patient population with IFALD. Such tools include biochemical composite scoring systems for fibrosis, transient elastography, and dynamic liver function tests. This review article aims to highlight the existing real need for an accurate, reproducible method to detect IFALD in its early stages. In addition, we also explore the role PN plays in the pathogenesis of this complex multifactorial condition. Various aspects of PN administration have been implicated in the etiology of IFALD, including the composition of the lipid component, nutrient excess and deficiency, and infusion timing. We aim to highlight the clinical relevance of these PN-associated factors in the development of IFALD and how these can be managed to mitigate the progression of IFALD.
肠衰竭相关肝病(IFALD)是一种严重的、限制生命的并发症,可发生在肠衰竭的整个临床过程中,并由肠外营养(PN)进行管理。尽管如此,IFALD 缺乏标准化的定义,这使得这种隐匿性疾病在临床实践中越来越难以筛查和诊断。在肝衰竭发生之前减缓肝病的进展是改善这些患者发病率和死亡率的关键。这需要及时发现并迅速解决可逆转的因素。尽管临床医生有各种非侵入性工具可用于检测各种慢性肝病患者的早期纤维化或肝硬化,但这些工具尚未在 IFALD 患者群体中得到验证。这些工具包括用于纤维化的生化综合评分系统、瞬时弹性成像和动态肝功能测试。本文旨在强调在早期阶段准确、可重复地检测 IFALD 的迫切需求。此外,我们还探讨了 PN 在这种复杂多因素疾病发病机制中的作用。PN 管理的各个方面都与 IFALD 的病因有关,包括脂质成分的组成、营养过剩和缺乏以及输注时间。我们旨在强调这些与 PN 相关的因素在 IFALD 发展中的临床相关性,以及如何管理这些因素以减轻 IFALD 的进展。