Di Dato Fabiola, Iorio Raffaele, Spagnuolo Maria Immacolata
Department of Translational Medical Science, Section of Pediatrics, University of Naples Federico II, Naples, Italy.
Front Nutr. 2022 Jul 25;9:928371. doi: 10.3389/fnut.2022.928371. eCollection 2022.
Intestinal failure-associated liver disease (IFALD) is a progressive liver disease complicating intestinal failure (IF). It is a preventable and reversible condition, but at the same time, a potential cause of liver cirrhosis and an indication to combined or non-combined liver and small bowel transplantation. The diagnostic criteria are not yet standardized, so that its prevalence varies widely in the literature. Pathophysiology seems to be multifactorial, related to different aspects of intestinal failure and not only to the long-term parenteral nutrition treatment. The survival rates of children with IF have increased, so that the main problems today are preventing complications and ensuring a good quality of life. IFALD is one of the most important factors that limit long-term survival of patients with IF. For this reason, more and more interest is developing around it and the number of published articles is increasing rapidly. The purpose of this narrative review was to focus on the main aspects of the etiology, pathophysiology, management, prevention, and treatment of IFALD, based on what has been published mainly in the last 10 years. Controversies and current research gaps will be highlighted with the aim to pave the way for new project and high-quality clinical trials.
肠衰竭相关肝病（IFALD）是一种并发于肠衰竭（IF）的进行性肝病。它是一种可预防且可逆的病症，但同时也是肝硬化的潜在病因，以及联合或非联合肝小肠移植的指征。其诊断标准尚未标准化，因此在文献中其患病率差异很大。病理生理学似乎是多因素的，与肠衰竭的不同方面有关，而不仅仅与长期肠外营养治疗有关。IF患儿的生存率有所提高，因此如今的主要问题是预防并发症并确保良好的生活质量。IFALD是限制IF患者长期生存的最重要因素之一。因此，围绕它的关注度越来越高，发表的文章数量也在迅速增加。本叙述性综述的目的是基于过去10年主要发表的内容，聚焦于IFALD的病因、病理生理学、管理、预防和治疗的主要方面。将突出争议点和当前的研究空白，以期为新项目和高质量临床试验铺平道路。
