Pediatric intestinal failure-associated liver disease.
Cathleen M Courtney1, Brad W Warner
1aDivision of Pediatric Surgery, St. Louis Children's Hospital bDepartment of Surgery, Washington University School of Medicine, St. Louis, Missouri, USA.
Current Opinion in Pediatrics
|March 24, 2017
Summary
Intestinal failure-associated liver disease (IFALD) is a major cause of mortality in pediatric patients. Novel lipid emulsions show promise in preventing and treating IFALD by reducing inflammation and improving bile flow.
Area of Science:
- Pediatric Gastroenterology
- Hepatology
- Parenteral Nutrition
Background:
- Intestinal failure-associated liver disease (IFALD) is a significant complication in pediatric patients requiring parenteral nutrition.
- The multifactorial nature of IFALD pathogenesis involves gut microbiome alterations, sepsis, and lack of enteral intake.
Purpose of the Study:
- To provide updated information on the definition, pathophysiology, treatment, and prevention of IFALD in pediatric patients.
- To highlight recent advancements in understanding IFALD molecular pathways and therapeutic strategies.
Main Methods:
- Literature review focusing on current research regarding IFALD.
- Analysis of molecular pathways, including Toll-like receptor 4 and farnesoid X receptor.
- Evaluation of lipid composition in parenteral nutrition and its impact on IFALD.
Main Results:
- IFALD pathogenesis is complex, involving proinflammatory cytokines and altered bile acid synthesis.
- Lipid emulsions with a reduced omega-6-to-omega-3 polyunsaturated fatty acid ratio may improve bile flow and decrease hepatic inflammation.
- Long-term effects of alternative lipid emulsions require further investigation.
Conclusions:
- IFALD remains a leading cause of mortality in pediatric intestinal failure.
- Novel lipid formulations offer promising alternatives to traditional soy-based formulas for reducing cholestasis.
- Addressing risk factors like gut dysbiosis and sepsis is crucial for IFALD management.
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