Department of Gastroenterology and Hepatology, School of Health Sciences, University Hospital of Ioannina, Faculty of Medicine, University of Ioannina, Ioannina, Greece.
Saudi J Gastroenterol. 2021 Jan-Feb;27(1):3-12. doi: 10.4103/sjg.sjg_551_20.
Intestinal failure-associated liver disease (IFALD) remains one of the most common and serious complications of parenteral nutrition (PN), causing a wide spectrum of hepatic manifestations from steatosis and mild cholestasis to portal hypertension and end-stage liver failure. The prevalence of IFALD depends on the diagnostic criteria and ranges from 4.3% to 65%. Moreover, many factors are shown to contribute to its development, including nutrient deficiencies, toxicity of PN, infections, and alterations of bile acid metabolism and gut microbiota. Prevention and management of IFALD aim at ameliorating or eliminating the risk factors associated with IFALD. The use of PN formulations with a lower ratio omega-6-to-omega-3 polyunsaturated fatty acids, cycle PN, optimization of enteral stimulation and prevention and early treatment of infections constitute the main therapeutic targets. However, failure of improvement and severe IFALD with end-stage liver failure should be considered as the indications of intestinal transplantation. The aim of this review is to provide an update of the epidemiology, pathophysiology, and diagnosis of IFALD in the adult population as well as to present a clinical approach of the therapeutic strategies of IFALD and present novel therapeutic targets.
肠衰竭相关肝病(IFALD)仍然是肠外营养(PN)最常见和最严重的并发症之一,可引起从脂肪变性和轻度胆汁淤积到门静脉高压和终末期肝衰竭等广泛的肝脏表现。IFALD 的患病率取决于诊断标准,范围为 4.3%至 65%。此外,许多因素被证明与 IFALD 的发展有关,包括营养缺乏、PN 的毒性、感染以及胆汁酸代谢和肠道微生物群的改变。IFALD 的预防和管理旨在改善或消除与 IFALD 相关的危险因素。使用ω-6 与 ω-3 多不饱和脂肪酸比例较低的 PN 配方、循环 PN、优化肠内刺激以及预防和早期治疗感染构成了主要的治疗目标。然而,对于改善失败和伴有终末期肝衰竭的严重 IFALD,应考虑作为肠移植的适应证。本文旨在提供成人 IFALD 的流行病学、病理生理学和诊断的最新信息,并介绍 IFALD 的治疗策略的临床方法以及新的治疗靶点。