Prevention and Treatment of Intestinal Failure-Associated Liver Disease in Children

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 (ILE) 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.

Lipid management in pediatric intestinal failure

“…Among these complications, PNALD is the most dreaded, as it can lead to progressive steatosis, cirrhosis, and liver failure [15,16]. PNALD is first evident as cholestasis and is generally accepted to occur when the direct bilirubin rises above 2 mg/dl after other causes of direct hyperbilirubinemia have been excluded [17][18][19][20]. In neonates, this biochemical evidence of liver damage may be evident as early as 2 weeks after initiating therapy and can be seen in nearly 100% of neonates on parenteral nutrition for longer than 8 weeks [21].…”

Monitoring of long‐term parenteral nutrition in children with intestinal failure

“…Pathogenesis of IFALD is multifactorial . The nutrition‐related factors include absence of enteral feeding, overfeeding, excess of lipid or glucose intake, imbalanced amino acids composition, continuous PN (non‐cyclical), lipid emulsions containing high w‐6/w‐3 ratio and high phytosterols with low α‐tocopherol levels, and micronutrient toxicity (copper, manganese, iron, aluminum).…”

“…The contamination of aluminum in PN solutions has been a concern as aluminum toxicity can cause bone disease, neurological impairment, liver disease, and anemia even years after initial exposure . Although aluminum content in PN has significantly decreased in the last years, high aluminum levels are still reported in children receiving PN .…”

The Principles and Practice of Nutritional Support

“…7. TPN-induced cholestasis [202] (a) Ursodeoxycholic acid for restoring the bile salt pool balance [203]. (b) Different lipid formulations (if available) containing medium-chain triglycerides or n-3 fi sh oils.…”