IFALD in children: What's new? A narrative review
Fabiola Di Dato1, Raffaele Iorio1, Maria Immacolata Spagnuolo1
1Department of Translational Medical Science, Section of Pediatrics, University of Naples Federico II, Naples, Italy.
Insights
Intestinal failure-associated liver disease (IFALD) is a serious complication of intestinal failure (IF). Early prevention and management are key to improving patient survival and quality of life.
Area of Science:
- Hepatology
- Pediatric Gastroenterology
- Intestinal Failure Management
Background:
- Intestinal failure-associated liver disease (IFALD) is a progressive liver condition in patients with intestinal failure (IF).
- IFALD can lead to liver cirrhosis and necessitate liver transplantation.
- Standardized diagnostic criteria for IFALD are lacking, leading to varied prevalence estimates.
Purpose of the Study:
- To review the etiology, pathophysiology, management, prevention, and treatment of IFALD.
- To highlight current research gaps and controversies in IFALD.
- To guide future research and clinical trials for IFALD.
Main Methods:
- Narrative review of published literature, focusing on the last 10 years.
- Synthesis of information on IFALD from various sources.
- Identification of key challenges and future directions in IFALD research.
Main Results:
- IFALD pathophysiology is multifactorial, involving aspects of IF beyond parenteral nutrition.
- IFALD significantly impacts long-term survival in IF patients.
- Increasing research interest and publications on IFALD.
Conclusions:
- IFALD is a critical factor limiting long-term survival in IF.
- Further research is needed to standardize diagnosis and optimize management strategies.
- High-quality clinical trials are essential to address current knowledge gaps in IFALD.
Abstract:
Intestinal failure-associated liver disease (IFALD) is a progressive liver disease complicating intestinal failure (IF). It is a preventable and reversible condition, but at the same time, a potential cause of liver cirrhosis and an indication to combined or non-combined liver and small bowel transplantation. The diagnostic criteria are not yet standardized, so that its prevalence varies widely in the literature. Pathophysiology seems to be multifactorial, related to different aspects of intestinal failure and not only to the long-term parenteral nutrition treatment. The survival rates of children with IF have increased, so that the main problems today are preventing complications and ensuring a good quality of life. IFALD is one of the most important factors that limit long-term survival of patients with IF. For this reason, more and more interest is developing around it and the number of published articles is increasing rapidly. The purpose of this narrative review was to focus on the main aspects of the etiology, pathophysiology, management, prevention, and treatment of IFALD, based on what has been published mainly in the last 10 years. Controversies and current research gaps will be highlighted with the aim to pave the way for new project and high-quality clinical trials.
