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Prevention and treatment of intestinal failure-associated liver disease in children
Bram P Raphael1, Christopher Duggan
1Division of Gastroenterology and Nutrition, Boston Children's Hospital, Harvard Medical School, Boston, MA 02115, USA.
Insights
Intestinal failure-associated liver disease (IFALD) is a serious complication of long-term parenteral nutrition (PN). While risk factors and PN components are implicated, effective management strategies are still under investigation.
Area of Science:
- Hepatology
- Pediatric Gastroenterology
- Clinical Nutrition
Background:
- Intestinal failure-associated liver disease (IFALD) is a severe complication in patients requiring long-term parenteral nutrition (PN).
- It manifests with a spectrum of liver damage, from cholestasis and steatosis to advanced fibrosis and cirrhosis.
- Known risk factors include prematurity, low birth weight, short bowel syndrome, and recurrent sepsis.
Purpose of the Study:
- To review the pathophysiology and current management strategies for IFALD.
- To highlight the potential role of parenteral nutrition components, particularly intravenous lipid emulsions (ILEs), in IFALD development.
- To discuss emerging and established treatments for IFALD.
Main Methods:
- Literature review of studies on IFALD pathophysiology and treatment.
- Analysis of the role of host factors and PN components in IFALD.
- Evaluation of current and potential therapeutic interventions.
Main Results:
- IFALD is multifactorial, involving host susceptibility and PN components like ILEs.
- ILEs may contribute to inflammation and impaired bile excretion.
- Potential treatments include ILE modification (e.g., omega-3 fatty acids), PN cycling, ursodeoxycholic acid, and sepsis prevention.
Conclusions:
- Management and prevention of IFALD remain significant clinical challenges.
- Further randomized controlled trials are needed to validate novel therapeutic approaches, such as specific ILE formulations.
- A comprehensive approach addressing PN composition, host factors, and supportive therapies is crucial for IFALD care.
Abstract:
Intestinal failure-associated liver disease (IFALD), a serious complication occurring in infants, children, and adults exposed to long-term parenteral nutrition (PN), causes a wide-spectrum of disease, ranging from cholestasis and steatosis to fibrosis and eventually cirrhosis. Known host risk factors for IFALD include low birth weight, prematurity, short bowel syndrome, and recurrent sepsis. The literature suggests that components of PN may also play a part of the multifactorial pathophysiology. Because some intravenous lipid emulsions (ILEs) may contribute to inflammation and interfere with bile excretion, treatment with ILE minimization and/or ILEs composed primarily of omega-3 fatty acids can be helpful, but requires careful monitoring for growth failure and essential fatty acid deficiency (EFAD). Data from randomized controlled trials are awaited to support widespread use of these approaches. Other IFALD treatments include cycling PN, ursodeoxycholic acid, sepsis prevention, photoprotection, and polyvinylchloride-free tubing. Management and prevention of IFALD remains a clinical challenge.
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