Premature infants and children with intestinal failure (IF) or short bowel syndrome are susceptible to intestinal failure-associated liver disease (IFALD, previously referred to as parenteral nutrition-associated liver disease, or PNALD). IFALD in children is characterized by progressive cholestasis and biliary fibrosis, and steatohepatitis in adults, and is seen in individuals dependent upon prolonged administration of PN. Many factors have been proposed as contributing to the pathogenesis of IFALD. In recent years, the focus has been on the potential synergistic roles of the intestinal microbiome, increased intestinal permeability, activation of hepatic innate immune pathways, and the use of intravenous soybean-oil-based intravenous lipid emulsions (SO-ILE). In vitro and in vivo studies have identified stigmasterol, a component of the plant sterols present in SO-ILE, as playing an important role. Although various strategies have been adopted to prevent or reverse IFALD, most suffer from a lack of strong evidence supported by well-designed, prospective clinical trials with clearly defined endpoints. Reduction in the amount of SO-ILEs or replacement with non-SO-ILEs has been shown to reverse IFALD although safety and long-term effectiveness have not been studied. Medical and surgical modalities to increase intestinal adaptation, advance enteral feedings, and prevent central line bloodstream infections are also important preventative strategies. There is a continued need to conduct high-quality, prospective trials with clearly define outcome measures to ascertain the potential benefits of these strategies.

中文翻译：

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肠衰竭相关肝病（IFALD）：深入了解发病机制和治疗进展。


患有肠衰竭（IF）或短肠综合征的早产儿和儿童容易患肠衰竭相关肝病（IFALD，以前称为肠外营养相关肝病，或 PNALD）。儿童 IFALD 的特征是进行性胆汁淤积和胆道纤维化，以及成人脂肪性肝炎，并且可见于依赖于长期给予 PN 的个体。已提出许多因素导致 IFALD 的发病机制。近年来，人们关注的焦点是肠道微生物组的潜在协同作用、增加肠道通透性、激活肝脏先天免疫途径以及静脉注射大豆油基静脉脂质乳剂（SO-ILE）的使用。体外和体内研究已确定豆甾醇（SO-ILE 中植物甾醇的一种成分）发挥着重要作用。尽管已经采取了各种策略来预防或逆转 IFALD，但大多数策略都缺乏精心设计、具有明确终点的前瞻性临床试验支持的有力证据。尽管安全性和长期有效性尚未研究，但减少 SO-ILE 的量或用非 SO-ILE 替代已被证明可以逆转 IFALD。增加肠道适应、促进肠内喂养和预防中心静脉血流感染的医疗和手术方式也是重要的预防策略。仍然需要进行高质量的前瞻性试验，并明确定义结果衡量标准，以确定这些策略的潜在益处。