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Published on: September 20, 2019
Novel lipid-based approaches to pediatric intestinal failure-associated liver disease
Ivan R Diamond1, Paul B Pencharz, Brian M Feldman
1Child Health Evaluative Sciences Program, The Hospital for Sick Children, Toronto, Ontario, Canada.
Insights
New strategies for preventing intestinal failure-associated liver disease (IFALD) in children show promise but lack definitive evidence. Current data is insufficient to support novel lipid therapies as standard care for early-stage IFALD.
Area of Science:
- Pediatric Gastroenterology
- Hepatology
- Nutritional Support
Background:
- Intestinal failure-associated liver disease (IFALD) is a major cause of morbidity in children with intestinal failure.
- Intravenous lipid emulsions are implicated in IFALD development, prompting research into alternatives.
- Current management focuses on addressing multifactorial causes of IFALD.
Purpose of the Study:
- To examine the rationale and current evidence for alternative intravenous lipid strategies in children with IFALD.
- To assess the efficacy and safety of lipid minimization and alternative lipid emulsions (including ω-3 fatty acids).
- To determine if novel therapies are suitable for standard care in preventing IFALD progression.
Main Methods:
- Review of existing literature on lipid minimization and alternative intravenous lipid emulsions.
- Analysis of studies focusing on children with intestinal failure and IFALD.
- Evaluation of evidence from uncontrolled settings, primarily in advanced IFALD cases.
Main Results:
- Alternative approaches like lipid minimization and ω-3 fatty acid emulsions show potential.
- Studies are predominantly uncontrolled and focus on children with advanced IFALD.
- Lack of definitive evidence regarding the efficacy of these novel therapies.
Conclusions:
- Insufficient evidence exists to support novel lipid therapies as standard of care for preventing IFALD progression in children with no or early IFALD.
- Further research is needed to evaluate the efficacy and safety, including effects on growth and development.
- Current data does not support widespread adoption of these therapies beyond specific clinical contexts.
Abstract:
Historically, intestinal failure-associated liver disease (IFALD) has been the greatest contributor to the morbidity experienced by children with intestinal failure. Although the cause of IFALD is multifactorial, recently much attention has been devoted to the critical role that intravenous lipid emulsions play in the development of IFALD. This attention has prompted an interest in alternate approaches to the provision of intravenous lipid in children with IFALD. The 2 approaches that have been advanced are that of lipid minimization and alternate intravenous lipid emulsions, including those containing ω-3 fatty acids. This article examines the rationale and current evidence for these approaches in children with intestinal failure. Our overall finding is that although these alternate approaches show significant promise, they have primarily been studied in uncontrolled settings, mainly in children with advanced IFALD. As such, we believe that there remains a lack of definitive evidence for their efficacy. Furthermore, important safety parameters remain to be evaluated, including the effect of these therapies on growth and development. Therefore, there is currently insufficient evidence to support the use of these novel therapies as standard of care in children with no or early IFALD with the goal of preventing the progression of liver disease.
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