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Intestinal failure-associated liver disease: management and treatment strategies past, present, and future
1Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Baylor College of Medicine, Houston, Texas 77030, USA.
Insights
Intestinal failure-associated liver disease (IFALD) affects 40-60% of infants on long-term parenteral nutrition (PN). This review explores novel strategies for IFALD management, including advanced PN and surgical interventions.
Area of Science:
- Pediatric Gastroenterology
- Hepatology
- Surgical Innovation
Background:
- Intestinal failure-associated liver disease (IFALD) is a serious complication affecting 40-60% of infants requiring long-term parenteral nutrition (PN).
- Multifactorial causes include prematurity, sepsis, infusate issues, and lack of enteral stimulation.
- Current treatments focus on choleretics, bowel decontamination, surgical procedures, and PN adjustments.
Purpose of the Study:
- To review current and novel treatment and management strategies for IFALD.
- To highlight emerging therapies and advanced care practices for IFALD.
Main Methods:
- Literature review of current and proposed IFALD treatments.
- Discussion of advanced central line care, novel surgical techniques (e.g., serial transverse enteroplasty), liver transplantation, probiotics, glutamine, hormonal therapies, and new PN formulations.
Main Results:
- The review synthesizes information on a range of IFALD interventions.
- Emerging strategies show promise in addressing the complexities of IFALD.
Conclusions:
- A comprehensive approach combining optimized PN, advanced surgical techniques, and novel therapeutic agents is crucial for managing IFALD.
- Continued research into new PN formulations and gut adaptation strategies is warranted.
Abstract:
Liver disease is estimated to develop in 40% to 60% of infants on long-term parenteral nutrition (PN) secondary to intestinal failure. The etiology of intestinal failure-associated liver disease (IFALD) is multifactorial with primary contributors including prematurity, sepsis, deficiencies or hepatotoxicities of infusates, and lack of enterally stimulated bile flow. IFALD treatment strategies have historically emphasized the following: choleretics such as ursodeoxycholic acid, bowel decontamination for small bowel bacterial overgrowth, bowel tapering and lengthening procedures, and manipulations of PN prescriptions (e.g., cycling). This review highlights current and proposed novel treatment and management strategies for IFALD. These include a discussion of state-of-the art central line care practices, novel bowel-lengthening procedures such as serial transverse enteroplasty, isolated liver transplant for IFALD, probiotics and glutamine for bowel decontamination, hormonal therapies for achieving bowel adaptation, and a discussion of new PN formulations that may have emerging roles in IFALD.
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