Intestinal failure-associated liver disease: what do we know today?

Area of Science:

• Hepatology
• Gastroenterology
• Pediatrics

Background:

• Intestinal failure-associated liver disease (IFALD) is a significant complication in patients requiring long-term parenteral nutrition (PN).
• IFALD prevalence ranges from 40-60% in infants and 15-40% in adults, with a spectrum of liver issues including steatosis, cholestasis, and fibrosis.
• While often reversible, IFALD can progress to cirrhosis, portal hypertension, and liver failure, particularly in neonates.

Purpose of the Study:

• To review the pathogenesis, clinical spectrum, and management strategies for intestinal failure-associated liver disease.
• To highlight the multifactorial causes of IFALD in both pediatric and adult populations.
• To discuss current and potential therapeutic interventions, including nutritional support and transplantation.

Main Methods:

• Literature review and synthesis of existing data on IFALD.
• Analysis of etiological factors in infants (prematurity, low birth weight, duration of PN, sepsis) and adults (age, PN duration, caloric/lipid/glucose load).
• Evaluation of the role of specific nutrients (choline, taurine, cysteine) and management strategies (enteral feeding, PN optimization, ursodeoxycholic acid).

Main Results:

• IFALD pathogenesis is multifactorial, involving lack of enteral feeding, reduced bile flow, biliary stasis, and nutrient deficiencies or excesses.
• Specific risk factors differ between infants and adults, with prematurity and sepsis being key in neonates, and PN composition and duration more critical in adults.
• Nutritional interventions (choline, taurine, cysteine) and improved PN management show promise in preventing and managing IFALD.

Conclusions:

• Early enteral feeding, optimized PN, and aseptic techniques are crucial for IFALD prevention.
• Addressing nutrient deficiencies and toxicities, alongside optimizing PN delivery, can mitigate liver injury.
• Liver transplantation remains an option for irreversible IFALD, with a 5-year survival rate of approximately 50%.