Current strategies for managing intestinal failure-associated liver disease

ABSTRACT

Introduction: Intestinal failure-associated liver disease (IFALD) refers to hepatic dysfunction that results from prolonged parenteral nutrition (PN) use. IFALD is multifactorial in origin and remains a major cause of morbidity and mortality. Prior to 2004, IFALD was associated with mortality as high as 90% in infants who remained on PN greater than 1 year. The advent of new strategies for intravenous lipid emulsion (ILE) administration and improved catheter care now allow many patients to remain on PN and recover from this once fatal condition. Several additional treatment modalities are often used to further improve outcomes for IFALD patients and they are reviewed here.

Areas covered: The etiology of IFALD is presented, as well as the rationale behind the use of ILEs that contain fish oil. Other management strategies are addressed, including the effects of several pharmacologic and nutritional interventions.

Expert opinion: Like its etiology, the management of IFALD is multifactorial. Prompt recognition of patients at risk, avoiding macronutrient excess, and preventing central line associated bloodstream infections will improve outcomes. In patients who develop IFALD, the use of fish oil monotherapy seems to be efficacious. The most effective intervention, however, continues to be discontinuation of PN and achieving full enteral feedings.

KEYWORDS:

• Intestinal failure-associated liver disease
• intravenous lipid emulsion
• fish oil
• ursodiol
• metronidazole
• phenobarbital

Article highlights

• Intestinal failure-associated liver disease (IFALD) refers to hepatic injury associated with intestinal failure that is more frequent in neonates and young children; older children and adults mainly develop steatosis.

• IFALD is multifactorial in etiology. Prolonged courses of PN, lack of enteral feedings, recurrent episodes of sepsis, and short bowel syndrome play a role in predisposing patients to this complication.

• IFALD is a diagnosis of exclusion, based on biochemical, clinical, and histologic parameters. Although there is no one definition, many consider a direct bilirubin ≥ 2mg/dL after 2 weeks of PN in neonates and more than 6 months in adults to be diagnostic of IFALD.

• Treatment focuses on advancement of enteral nutrition and reduction in PN exposure. When that is not possible, avoiding macronutrient excess and optimizing lipid provision is essential.

• In patients who develop IFALD, stopping all soybean oil intravenous lipid emulsions and replacing with fish oil monotherapy appears to be an effective means of reversing this potentially fatal condition.

• Preventing sepsis through catheter management bundles, patient/caregiver education, and the use of ethanol locks improves outcomes in patients at risk for developing IFALD.

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Declaration of interest

K Gura is a consultant for Pronova/BASF, Northsea Therapeutics, Xellia Pharmaceuticals, Pfizer Pediatric Center of Excellence, Baxter, and has received research support from Northsea Therapeutics, Otsuka Pharmaceutical Company, Alcresta, and Fresenius Kabi. M Puder is a consultant for Pronova/BASF, Northsea Therapeutics, and has received research support from Northsea Therapeutics, Otsuka Pharmaceutical Company, Alcresta, and Fresenius Kabi; Patent/Royalties for Omegaven are forthcoming. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

This paper was not funded.