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Copyright ©The Author(s) 2021. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Jun 28, 2021; 27(24): 3440-3465
Published online Jun 28, 2021. doi: 10.3748/wjg.v27.i24.3440
Chronic intestinal failure and short bowel syndrome in Crohn’s disease
Aysegül Aksan, Karima Farrag, Irina Blumenstein, Oliver Schröder, Axel U Dignass, Jürgen Stein
Aysegül Aksan, Institute of Nutritional Sciences, Justus-Liebig-Universität, Giessen 35392, Germany
Aysegül Aksan, Karima Farrag, Oliver Schröder, Jürgen Stein, Department of Clinical Research, Interdisziplinäres Crohn Colitis Centrum Rhein-Main, Frankfurt am Main 60594, Germany
Karima Farrag, Oliver Schröder, Jürgen Stein, Department of Gastroenterology and Clinical Nutrition, DGD Kliniken Sachsenhausen, Teaching Hospital of the JW Goethe University, Frankfurt am Main 60594, Germany
Irina Blumenstein, Department of Gastroenterology, Hepatology and Clinical Nutrition, First Medical Clinic, JW Goethe University Hospital, Frankfurt am Main 60529, Germany
Axel U Dignass, Department of Medicine I, Agaplesion Markus Hospital, Goethe-University, Frankfurt am Main 60431, Germany
Jürgen Stein, Institute of Pharmaceutical Chemistry, JW Goethe University, 60438 Frankfurt am Main, Germany
Author contributions: Aksan A, Farrag K, Blumenstein I, Schröder O, Dignass A, and Stein J designed and performed the literature search, interpreted the data, and drafted the manuscript; All authors have read and approved the final manuscript.
Conflict-of-interest statement: Aksan A reports congress expenses, consultancy fees and research funding from Vifor Pharma and Immundiagnostik AG. Farrag K reports speakers’ honoraria and congress expenses from Immundiagnostik AG, Janssen Cilag GmbH; consultancy honoraria from Vifor Pharma. Blumenstein I reports consultancy honoraria from AbbVie Deutschland, Amgen GmbH, Bristol Myers Squibb, Janssen Cilag GmbH, Fresenius Kabi Deutschland, Pharmacosmos GmbH, Pfizer GmbH, Shire Deutschland GmbH, Takeda Pharma GmbH; honoraria from AbbVie Deutschland, Biogen GmbH, DGVS, Dr. Falk Pharma GmbH, Endoakademie, Ferring Arzneimittel, Fraunhofer, Fresenius Kabi Deutschland, Kompetenznetz Darmerkrankungen, MSD Sharp Dome, Mylan GmbH, Pfizer GmbH, Janssen Cilag GmbH, Shire Deutschland GmbH, Takeda Pharma GmbH. Schroeder O reports congress expenses from Abbvie, Falk, Janssen, Takeda; speakers’ honoraria from Abbvie, Falk, Janssen, Takeda, Norgine, Pfizer. Dignass A reports consultancy honoraria from Abbott, Pharmacosmos, MSD, Ferring, Janssen, Pfizer, Otsuka, Roche/Genentech, Takeda, Falk; grants from Institut für Gemeinwohl & Stiftung Leben mit Krebs; Lecturing: Falk, Ferring, MSD, Abbott, Janssen, Pfizer, Otsuka, Vifor, Stiftung Leben mit Krebs, Kompetenznetz CED, Takeda, Pharmacosmos; Manuscript preparation: Falk. Education presentations: Abbott, Pharmacosmos, Falk, Ferring. Stein J reports personal fees from Abbvie, personal fees from Dr Schär, personal fees from Falk, personal fees from Ferring, personal fees from Fresenius Kabi, personal fees from Immundiagnostik AG, personal fees from Janssen, personal fees from Medice, personal fees from MSD, personal fees from Pfizer, personal fees from Pharmacosmos, personal fees from Shire, personal fees from Shield, personal fees from Takeda, personal fees from ThermoFisher, personal fees from Vifor Pharma, from NPS, outside the submitted work.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Corresponding author: Jürgen Stein, MD, PhD, Chief Physician, Full Professor, Department of Gastroenterology and Clinical Nutrition, DGD Kliniken Sachsenhausen, Teaching Hospital of the JW Goethe University, Schulstr. 31, Frankfurt am Main 60594, Germany. j.stein@em.uni-frankfurt.de
Received: December 25, 2020
Peer-review started: December 25, 2020
First decision: January 9, 2021
Revised: January 24, 2021
Accepted: March 7, 2021
Article in press: March 7, 2021
Published online: June 28, 2021
Processing time: 181 Days and 20.8 Hours
Abstract

Chronic intestinal failure (CIF) is a rare but feared complication of Crohn’s disease. Depending on the remaining length of the small intestine, the affected intestinal segment, and the residual bowel function, CIF can result in a wide spectrum of symptoms, from single micronutrient malabsorption to complete intestinal failure. Management of CIF has improved significantly in recent years. Advances in home-based parenteral nutrition, in particular, have translated into increased survival and improved quality of life. Nevertheless, 60% of patients are permanently reliant on parenteral nutrition. Encouraging results with new drugs such as teduglutide have added a new dimension to CIF therapy. The outcomes of patients with CIF could be greatly improved by more effective prevention, understanding, and treatment. In complex cases, the care of patients with CIF requires a multidisciplinary approach involving not only physicians but also dietitians and nurses to provide optimal intestinal rehabilitation, nutritional support, and an improved quality of life. Here, we summarize current literature on CIF and short bowel syndrome, encompassing epidemiology, pathophysiology, and advances in surgical and medical management, and elucidate advances in the understanding and therapy of CIF-related complications such as catheter-related bloodstream infections and intestinal failure-associated liver disease.

Keywords: Chronic intestinal failure; Short bowel syndrome; Crohn's disease; Inflammatory bowel disease; Parenteral nutrition; Intestinal failure-associated liver disease

Core Tip: Chronic intestinal failure (CIF) is a rare but feared severe complication of Crohn’s disease, with 60% of patients permanently dependent on parenteral nutrition. This review aims to summarize the knowledge available in the current literature describing recent advances in the management and treatment of adult patients with CIF, with emphasis on patients with Crohn’s disease. Moreover, it aims to further understanding of modern approaches to CIF complications such as catheter-related bloodstream infections and intestinal failure-associated liver disease.