Medical Progress for Intestinal Failure Associated Liver Disease

A recent review (WS Lee, RJ Sokol. J Pediatr 2015; 167: 519-26) provides a good explanation of the role of various intralipids and intestinal microbial dysbiosis in the setting of intestinal failure-associated lipid disease (IFALD).

The review discusses criteria for IFALD (e.g. conjugated bilirubin ≥2 mg/dL & parenteral nutrition ≥14 days), the epidemiology, and the pathogenesis. Potential risk factors and level of evidence for these risk factors is noted in Table 1.

Table 2 describes the evidence supporting suggested strategies for the prevention of IFALD. The effectiveness and recommendation levels for these strategies are generally very low and weak based on reviews by ASPEN and the American Pediatric Surgical Association.  Among the strategies, reduction of lipid emulsion to ≤ 1 g/kg/day has some of the strongest support in this table but is still regarded as level III evidence and described as “probably effective.” Other strategies reviewed included ethanol locks, multidisciplinary team management, use of ursodeoxycholic acid/bile acid supplementation, cycling of parenteral nutrition, use of prokinetics, removal of manganese and copper from parenteral nutrition, and antibiotic use to prevent bacterial overgrowth.

With regard to alternative intravenous lipids (eg. fish oil, or SMOF mixture):

  • “Although a properly powered randomized controlled trial has not been conducted, current evidence suggests that the use of FO-ILE [fish oil -intravenous lipid emulsion] is effective in reversing the established cholestasis associated with IFALD, but there is insufficient evidence for a preventative effect in neonates.”
  • “Most studies have been retrospective, used a historical comparison group, used different doses of lipid, and were conducted in patients with quite advanced IFALD.”
  • Use of FO-ILE improves biochemical parameters, but has not been shown to “improve other important long-term clinical outcomes, such as severity of hepatic fibrosis.”
  • In addition, reduced ILE and FO-ILE may result in other sequelae such as cognitive developmental delay. “Recently, lower brain weight and alterations of brain PUFA content were demonstrated in newborn piglets receiving total PN with reduced dose SO-ILE or FO-ILE compared with normal dose SO-ILE.”

My take: This review underscores how little is known about the approaches often recommended for management of IFALD.

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Disclaimer: These blog posts are for educational purposes only. Specific dosing of medications/diets (along with potential adverse effects) should be confirmed by prescribing physician/nutritionist.  This content is not a substitute for medical advice, diagnosis or treatment provided by a qualified healthcare provider. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a condition.

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