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肠衰竭相关肝病:病因、临床表现与治疗。

Intestinal failure-associated liver disease: causes, manifestations and therapies.

机构信息

Division of Gastroenterology and Hepatology, MSB 5533, 982000 The Nebraska Medical Center.

Department of Clinical Nutrition, 981200 Nebraska Medicine, Omaha, Nebraska, USA.

出版信息

Curr Opin Gastroenterol. 2019 Mar;35(2):126-133. doi: 10.1097/MOG.0000000000000503.

Abstract

PURPOSE OF REVIEW

The goal of this review is to provide updates on the causes, manifestations and therapies IFALD in adults with an emphasis on recent discoveries on pathways of pathogenesis and interventions to reduce the incidence of IFALD.

RECENT FINDINGS

IFALD is a multifactorial complication of long-term home parenteral therapy. Although exact pathways are unknown, altered bile acid metabolism, microbiome dysbiosis impact on the gut-liver axis and soybean-based lipid formulations are major drivers of IFALD development.

SUMMARY

IFALD contributes to morbidity and mortality in patients on parenteral nutrition. Proactive management by a multidisciplinary team has led to improved outcomes in at-risk patients. Attention to early treatment and prevention of sepsis, introduction of nonsoybean based lipid formulations, surgical procedures such as step enteroplasties and, potentially, microbiome dysbiosis are considerations in IFLAD management.

摘要

目的综述

本综述的目的是提供成人 IFALD(肝内胆汁淤积性肝病)病因、表现和治疗的最新信息,重点介绍发病机制途径的最新发现和减少 IFALD 发生率的干预措施。

最近的发现

IFALD 是长期家庭肠外营养治疗的一种多因素并发症。尽管确切的途径尚不清楚,但胆汁酸代谢改变、微生物组失调对肠道-肝脏轴的影响以及大豆基脂质制剂是 IFALD 发展的主要驱动因素。

总结

IFALD 会导致肠外营养患者的发病率和死亡率增加。多学科团队的积极管理可改善高危患者的预后。关注早期治疗和预防脓毒症、使用非大豆基脂质制剂、进行步骤式肠段成形术等外科手术,以及可能的微生物组失调,这些都是 IFALD 治疗的考虑因素。

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