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Intestinal failure associated liver disease — Clinical Guidelines

Overview

Intestinal failure associated liver disease (IFALD) refers to liver complications arising in patients with intestinal failure, often due to malabsorption and parenteral nutrition (PN) use, leading to significant morbidity and mortality 12 15.

Diagnosis

Key Diagnostic Criteria: Elevated liver enzymes, coagulopathy, and imaging findings indicative of liver fibrosis or cirrhosis 12.

Recommended Tests: Regular monitoring of liver function tests (ALT, AST, ALP, GGT), bilirubin levels, and coagulation profiles 12.

Grading: Use of Child-Pugh score or Model for End-Stage Liver Disease (MELD) to assess severity 12.

Management

First-Line Treatments: Optimize PN composition, reduce PN duration, and promote enteral nutrition when possible 14 15.

Adjunctive Treatments:

- Vitamin D Supplementation: Implement standardized algorithms to maintain adequate vitamin D levels 1. - Micronutrient Supplementation: Regular monitoring and supplementation of fat-soluble vitamins (A, D, E, K) and other micronutrients 11 13 16. - Management of Complications: Address thrombocytopenia-associated multiple organ failure with plasma exchange or infusion based on clinical context 2.

Special Populations

Pediatrics: High prevalence of micronutrient deficiencies; standardized vitamin D algorithms are crucial 1 13.

Comorbidities: Consider the impact of central venous access complications and manage with evidence-based practices to reduce infection risks 4.

Key Recommendations

Implement standardized protocols for vitamin D supplementation to prevent deficiency in pediatric patients with intestinal failure (Evidence: Moderate 1).

Optimize parenteral nutrition composition and promote enteral nutrition transition to reduce IFALD risk (Evidence: Moderate 14 15).

Regularly monitor and supplement fat-soluble vitamins and other micronutrients in patients on home parenteral nutrition (Evidence: Moderate 11 13 16).

Manage central venous access carefully to minimize infection risks associated with long-term catheter use (Evidence: Moderate 4).

Early referral to intestinal failure programs may improve outcomes, particularly by monitoring hyperbilirubinemia (Evidence: Moderate 12).

References

1 Zhong J, Martins DS, Piper HG. Standardizing vitamin D supplementation to minimize deficiency in children with intestinal failure. Nutrition in clinical practice : official publication of the American Society for Parenteral and Enteral Nutrition 2024. link 2 Çeleğen M, Çeleğen K. Management of thrombocytopenia-associated multiple organ failure: plasma infusion vs plasma exchange. The Turkish journal of pediatrics 2022. link 3 Park CJ, Shaughnessy MP, Zhang L, Armenia SJ, Caty MG, Cowles RA. National Survey of Pediatric Intestinal Rehabilitation Programs in the United States. JPEN. Journal of parenteral and enteral nutrition 2021. link 4 Wendel D, Mezoff EA, Raghu VK, Kinberg S, Soden J, Avitzur Y et al.. Management of Central Venous Access in Children With Intestinal Failure: A Position Paper From the NASPGHAN Intestinal Rehabilitation Special Interest Group. Journal of pediatric gastroenterology and nutrition 2021. link 5 Spolidoro JVN, Souza MC, Goldani HAS, Tanzi MN, Busoni VB, Padilla MDC et al.. International Latin American Survey on Pediatric Intestinal Failure Team. Nutrients 2021. link 6 Galloway DP, Mathis MS, Wilkinson LT, Venick RS, Wendel D, Cole CR et al.. Effect of the COVID-19 Pandemic on Pediatric Intestinal Failure Healthcare Delivery. JPEN. Journal of parenteral and enteral nutrition 2021. link 7 Batchelor S, Gemmell L, Kirk C, Mountford C, Thompson N. The effectiveness of buccal Vitamin D replacement in patients requiring home parenteral nutrition. Clinical nutrition ESPEN 2021. link 8 Rumore S, McGrath K, Scott A, Sexton E, Wong T. Fat soluble vitamin status in children on home parenteral nutrition in a tertiary paediatric intestinal rehabilitation unit. Clinical nutrition ESPEN 2021. link 9 Wengerter BC, Emre G, Park JY, Geibel J. Three-dimensional Printing in the Intestine. Clinical gastroenterology and hepatology : the official clinical practice journal of the American Gastroenterological Association 2016. link 10 Bridges BC, Hardison D, Pietsch J. A case series of the successful use of ECMO, continuous renal replacement therapy, and plasma exchange for thrombocytopenia-associated multiple organ failure. Journal of pediatric surgery 2013. link 11 Ellegård L, Kurlberg G, Bosaeus I. High prevalence of vitamin D deficiency and osteoporosis in out-patients with intestinal failure. Clinical nutrition (Edinburgh, Scotland) 2013. link 12 Javid PJ, Malone FR, Bittner R, Healey PJ, Horslen SP. The optimal timing of referral to an intestinal failure program: the relationship between hyperbilirubinemia and mortality. Journal of pediatric surgery 2011. link 13 Yang CF, Duro D, Zurakowski D, Lee M, Jaksic T, Duggan C. High prevalence of multiple micronutrient deficiencies in children with intestinal failure: a longitudinal study. The Journal of pediatrics 2011. link 14 Mziray-Andrew CH, Sentongo TA. Nutritional deficiencies in intestinal failure. Pediatric clinics of North America 2009. link 15 Beath S, Pironi L, Gabe S, Horslen S, Sudan D, Mazeriegos G et al.. Collaborative strategies to reduce mortality and morbidity in patients with chronic intestinal failure including those who are referred for small bowel transplantation. Transplantation 2008. link 16 Forbes GM, Forbes A. Micronutrient status in patients receiving home parenteral nutrition. Nutrition (Burbank, Los Angeles County, Calif.) 1997. link00334-1) 17 Bankson DD, Rifai N, Silverman LM. Immunoturbidimetric measurement of serum retinol-binding protein in renal and hepatic disease. Annals of clinical biochemistry 1988. link 18 Gelernt IM, Bauer JJ, Kreel I. Continent ileostomy in the pediatric patient. Journal of pediatric surgery 1976. link90102-0)

Intestinal failure associated liver disease