Skip to main content

Nutrition and Liver Disease

  • Chapter
  • First Online:
Book cover Pediatric Hepatology and Liver Transplantation
  • 1724 Accesses

Abstract

Protein-energy malnutrition is one of the most frequent complications in chronic liver diseases. Sarcopenia or muscle wasting has recently emerged as a central element. It is especially prevalent in young children with chronic cholestasis and fat malabsorption. It negatively impacts on prognosis, with and without liver transplantation. It should be prevented or treated with early nutritional treatment, oral or enteral, and the maintenance of physical activity. Vitamins and oligo-elements should be regularly checked and supplemented as needed.

Intestinal failure-associated liver disease is the most prevalent complication of long-term parenteral nutrition (PN). It is a multifactorial disease, where prematurity, anatomical factors, infections, and toxic effects of PN solutions interact. The prevention or correction of risk factors and the manipulation of PN, especially the reestablishment of enterohepatic circulation, control of infections, and use of fish oil-containing lipid emulsions, improve the evolution in most cases. Early shared care with an intestinal failure center is important to guide the management and timely refer for intestinal transplantation as needed.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 169.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Hardcover Book
USD 219.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Merli M, Riggio O, Dally L. Does malnutrition affect survival in cirrhosis ? PINC (Policentrica Italiana Nutrizione Cirrosi). Hepatology. 1996;23:1341–6.

    Article  Google Scholar 

  2. Chin SE, Shepherd RW, Thomas BJ, Cleghorn GJ, Patrick MK, Wilcox JA, et al. The nature of malnutrition in children with end-stage liver disease awaiting orthotopic liver transplantation. Am J Clin Nutr. 1992;56:164–8.

    Article  CAS  Google Scholar 

  3. Tandon P, Raman M, Mourtzakis M, Merli M. A practical approach to nutritional screening and assessment in cirrhosis. Hepatology. 2017;65:1044–57.

    Article  Google Scholar 

  4. Plauth M, Merli M, Kondrup J, Weimann A, Ferenci P, Müller MJ. ESPEN guidelines for nutrition in liver disease and transplantation. Clin Nutr. 1997;16:43–55.

    Article  CAS  Google Scholar 

  5. Ramaccioni V, Soriano HE, Arumugam R, Klish WJ. Nutritional aspects of chronic liver disease and liver transplantation in children. J Pediatr Gastroenterol Nutr. 2000;30:361–7.

    Article  CAS  Google Scholar 

  6. O’Brien A, Williams R. Nutrition in end-stage liver disease: principles and practice. Gastroenterology. 2008;134:1729–40.

    Article  Google Scholar 

  7. Toshikuni N, Arisawa T, Tsutsumi M. Nutrition and exercise in the management of liver cirrhosis. World J Gastroenterol. 2014;20:7286–97.

    Article  Google Scholar 

  8. Baker A, Stevenson R, Dhawan A, Goncalves I, Socha P, Sokal E. Guidelines for nutritional care for infants with cholestatic liver disease before liver transplantation. Pediatr Transplant. 2007;11:825–34.

    Article  CAS  Google Scholar 

  9. Dasarathy S, Merli M. Sarcopenia from mechanism to diagnosis and treatment in liver disease. J Hepatol. 2016;65:1232–44.

    Article  Google Scholar 

  10. Englesbe MJ, Patel SP, He K, Lynch RJ, Schaubel DE, Harbaugh C, et al. Sarcopenia and mortality after liver transplantation. J Am Coll Surg. 2010;211:271–8.

    Article  Google Scholar 

  11. Kim HY, Jang JW. Sarcopenia in the prognosis of cirrhosis: going beyond the MELD score. World J Gastroenterol. 2015;21:7637–47.

    Article  Google Scholar 

  12. Giannelli V, Di Gregorio V, Iebba V, Giusto M, Schippa S, Merli M, Thalheimer U. Microbiota and the gut-liver axis: bacterial translocation, inflammation and infection in cirrhosis. World J Gastroenterol. 2014;20:16795–810.

    Article  Google Scholar 

  13. Müller MJ, Lautz HU, Plogmann B, Bürger M, Körber J, Schmidt FW. Energy expenditure and substrate oxidation in patients with cirrhosis: the impact of cause, clinical staging and nutritional state. Hepatology. 1992;15:782–94.

    Article  Google Scholar 

  14. Greer R, Lehnert M, Lewindon P, Cleghorn GJ, Shepherd RW. Body composition and components of energy expenditure in children with end-stage liver disease. J Pediatr Gastroenterol Nutr. 2003;36:358–63.

    Article  Google Scholar 

  15. Holt RI, Baker AJ, Jones JS, Miell JP. The insulin-like growth factor and binding protein axis in children with end-stage liver disease before and after liver transplantation. Pediatr Transplant. 1998;2:76–84.

    CAS  PubMed  Google Scholar 

  16. Changani KK, Jalan R, Cox IJ, Ala-Korpela M, Bhakoo K, Taylor-Robinson SD, et al. Evidence for altered hepatic gluconeogenesis in patients with cirrhosis using in vivo 32-phosphorus magnetic resonance spectroscopy. Gut. 2001;49:557–64.

    Article  CAS  Google Scholar 

  17. Socha P, Koletzko B, Pawlowska J, Socha J. Essential fatty acid status in children with cholestasis, in relation to serum bilirubin concentration. J Pediatr. 1997;131:700–6.

    Article  CAS  Google Scholar 

  18. Tessari P. Protein metabolism in liver cirrhosis: from albumin to muscle myofibrils. Curr Opin Clin Nutr Metab Care. 2003;6:79–85.

    Article  CAS  Google Scholar 

  19. Kawaguchi T, Izumi N, Charlton MR, Sata M. Branched-chain amino acids as pharmacological nutrients in chronic liver disease. Hepatology. 2011;54:1063–70.

    Article  CAS  Google Scholar 

  20. World Health Organization, UNICEF. WHO child growth standards and the identification of severe acute malnutrition in infants and children. Geneva: World Health Organization; 2009.

    Google Scholar 

  21. Tajika M, Kato M, Mohri H, Miwa Y, Kato T, Ohnishi H, et al. Prognostic value of energy metabolism in patients with viral liver cirrhosis. Nutrition. 2002;18:229–34.

    Article  CAS  Google Scholar 

  22. Pirlich M, Schütz T, Spachos T, Ertl S, Weiss ML, Lochs H, et al. Bioelectric impedance analysis is a useful bedside technique to asssess malnutrition in cirrhotic patients with and without ascites. Hepatology. 2000;32:1208–15.

    Article  CAS  Google Scholar 

  23. Durand F, Buyse S, Francoz C, Laouénan C, Bruno O, Belghiti J, et al. Prognostic value of muscle atrophy in cirrhosis using psoas muscle thickness on computed tomography. J Hepatol. 2014;60:1151–7.

    Article  Google Scholar 

  24. Cowley AD, Cummins C, Beath SV, Lloyd C, van Mourik ID, McKiernan PJ, Kelly DA. Paediatric hepatology dependency score (PHD score): an audit tool. J Pediatr Gastroenterol Nutr. 2007;44:108–15.

    Article  Google Scholar 

  25. Verboeket-van de Venne WP, Westerterp KR, van Hoek B, Swart GR. Energy expenditure and substrate metabolism in patients with cirrhosis of the liver: effects of the pattern of food intake. Gut. 1995;36:110–6.

    Article  CAS  Google Scholar 

  26. Plauth M, Cabré E, Riggio O, Assis-Camilo M, Pirlich M, Kondrup J, et al. ESPEN guidelines on enteral nutrition: liver disease. Clin Nutr. 2006;25:285–94.

    Article  CAS  Google Scholar 

  27. Planck LD, Gane EJ, Peng S, Muthu C, Mathur S, Gillanders L, et al. Nocturnal nutritional supplementation improves total body protein status of patients with liver cirrhosis: a randomized 12-month trial. Hepatology. 2008;48:557–66.

    Article  Google Scholar 

  28. Grimber D, Michaud L, Ategbo S, Turck D, Gottrand F. Experience of parenteral nutrition for nutritional rescue in children with severe liver disease following failure of enteral nutrition. Pediatr Transplant. 1999;3:139–45.

    Article  Google Scholar 

  29. Thébault A, Nemeth A, Le Mouhaër J, Scheenstra R, Baumann U, Koot B, et al. Oral tocofersolan corrects or prevents vitamin E deficiency in children with chronic cholestasis. J Pediatr Gastroenterol Nutr. 2016;63:610–5.

    Article  Google Scholar 

  30. Marchesini G, Bianchi G, Merli M, Amodio P, Panella C, Loguercio C, et al. Nutritional supplementation with branched-chain amino acids in cirrhosis: a randomized double blind trial. Gastroenterology. 2003;124:1792–801.

    Article  CAS  Google Scholar 

  31. Chin SE, Shepherd RW, Thomas BJ, Cleghorn GJ, Patrick MK, Wilcox JA, et al. Nutritional support in children with end-stage liver disease: a randomized crossover trial of a branched-chain amino acid supplement. Am J Clin Nutr. 1992;56:158–63.

    Article  CAS  Google Scholar 

  32. Lemize D, Dharancy S, Wallaert B. Response to exercise in patients with liver cirrhosis: implications for liver transplantation. Dig Liver Dis. 2013;45:362–6.

    Article  Google Scholar 

  33. Lacaille F, Gupte G, Colomb V, D’Antiga L, Hartman C, Hojsak I, et al. Intestinal failure—associated liver disease. A position paper by the ESPGHAN Working Group of Intestinal failure and intestinal transplantation. J Pediatr Gastroenterol Nutr. 2015;60:272–83.

    Article  CAS  Google Scholar 

  34. D’Antiga L, Goulet O. Intestinal failure in children. The European view. J Pediatr Gastroenterol Nutr. 2012;56:118–26.

    Article  Google Scholar 

  35. Koletzko B, Goulet O, Hunt J, Krohn K, Shamir R, Parenteral Nutrition Guidelines Working Group; European Society for Clinical Nutrition and Metabolism; European Society of Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN); European Society of Paediatric Research (ESPR). Guidelines on Paediatric Parenteral Nutrition of the European Society of Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) and the European Society for Clinical Nutrition and Metabolism (ESPEN), Supported by the European Society of Paediatric Research (ESPR). J Pediatr Gastroenterol Nutr. 2005;41:S1–87.

    Article  Google Scholar 

  36. Kocoshis SA. Medical management of intestinal failure. Semin Pediatr Surg. 2010;19:20–6.

    Article  Google Scholar 

  37. Colomb V, Dabbas-Tyan M, Taupin P, Talbotec C, Révillon Y, Jan D, et al. Long term outcome of children receiving home parenteral nutrition: a 20 year single center experience in 302 patients. J Pediatr Gastroenterol Nutr. 2007;44:347–53.

    Article  Google Scholar 

  38. Pichler J, Horn V, Macdonald S, Hill S. Intestinal failure-associated liver disease in hospitalised children. Arch Dis Child. 2012;97:211–4.

    Article  Google Scholar 

  39. Ganousse-Mazeron S, Lacaille F, Colomb-Jung V, Talbotec C, Ruemmele F, Sauvat F, Chardot C, Canioni D, Jan D, Revillon Y, Goulet O. Assessment and outcome of children with intestinal failure referred for intestinal transplantation. Clin Nutr. 2015;34:428–35.

    Article  CAS  Google Scholar 

  40. Black DD, Suttle A, Whitington PF, Whitington GL, Korones SD. The effect of short-term parenteral nutrition on hepatic function in human neonate: a prospective randomized study demonstrating alteration of hepatic canalicular function. J Pediatr. 1981;99:445–9.

    Article  CAS  Google Scholar 

  41. Trauner M, Fuchs CD, Halibasic E, Paumgartner G. New therapeutic concepts in bile acid transport and signaling for management of cholestasis. Hepatology. 2017;65:1393–404.

    Article  Google Scholar 

  42. Goulet O, Olieman J, Ksiazyk J, Spolidoro J, Tibboe D, Köhler H, et al. Neonatal short bowel syndrome as a model of intestinal failure: physiological background for enteral feeding. Clin Nutr. 2013;32:162–71.

    Article  CAS  Google Scholar 

  43. Hermans D, Talbotec C, Lacaille F, Goulet O, Ricour C, Colomb V. Early central catheter infections may contribute to hepatic fibrosis in children receiving long-term parenteral nutrition. J Pediatr Gastroenterol Nutr. 2007;44:459–63.

    Article  Google Scholar 

  44. Goulet O, Lambe C. Intravenous lipid emulsions in pediatric patients with intestinal failure. Curr Opin Organ Transplant. 2017;22:142–8.

    Article  CAS  Google Scholar 

  45. Seida JC, Mager DR, Hartling L, Vandermeer B, Turner JM. Parenteral ω-3 fatty acid lipid emulsions for children with intestinal failure and other conditions: a systematic review. J Parenter Enter Nutr. 2013;37:44–55.

    Article  Google Scholar 

  46. Mercer DF, Hobson BD, Fischer RT, Talmon GA, Perry DA, Gerhardt BK, et al. Hepatic fibrosis persists and progresses despite biochemical improvement in children treated with intravenous fish oil emulsion. J Pediatr Gastroenterol Nutr. 2013;56:354–9.

    Article  Google Scholar 

  47. Belza C, Thompson R, Somers GR, de Silva N, Fitzgerald K, Steinberg K, et al. Persistence of hepatic fibrosis in pediatric intestinal failure patients treated with intravenous fish oil lipid emulsion. J Pediatr Surg. 2017;52:795–801.

    Article  Google Scholar 

  48. Colomb V, Jobert-Giraud A, Lacaille F, Goulet O, Fournet JC, Ricour C. Role of lipid emulsions in cholestasis associated with long-term parenteral nutrition in children. J Parenter Enter Nutr. 2000;24:345–50.

    Article  CAS  Google Scholar 

  49. Stout SM, Cober MP. Metabolic effects of cyclic parenteral nutrition infusion in adults and children. Nutr Clin Pract. 2010;25:277–81.

    Article  Google Scholar 

  50. Van Gossum A, Pironi L, Messing B, Moreno C, Colecchia A, D'Errico A, et al. Transient elastography (Fibroscan) is not correlated with fibrosis but to cholestasis in patients with long term home parenteral nutrition. J Parenter Enteral Nutr. 2015;39:719–24.

    Article  Google Scholar 

  51. Braegger C, Decsi T, Dias JA, Hartman C, Kolacek S, Koletzko B, et al. Practical approach to paediatric enteral nutrition: a comment by the ESPGHAN committee on nutrition. J Pediatr Gastroenterol Nutr. 2010;51:110–22.

    Article  Google Scholar 

  52. Jeppesen PB, Gilroy R, Pertkiewicz M, et al. Randomised placebo-controlled trial of teduglutide in reducing parenteral nutrition and/or intravenous fluid requirements in patients with short bowel syndrome. Gut. 2011;60:902–14.

    Article  CAS  Google Scholar 

  53. Carter BA, Cohran VC, Cole CR, Corkins MR, Dimmitt RA, Duggan C, et al. Outcomes from a 12-week, open-label, multicenter clinical trial of teduglutide in pediatric short bowel syndrome. J Pediatr. 2017;181:102–11.

    Article  CAS  Google Scholar 

  54. Frongia G, Kessler M, Weih S, Nickkholgh A, Mehrabi A, Holland-Cunz S. Comparison of LILT and STEP procedures in children with short bowel syndrome—a systematic review of the literature. J Pediatr Surg. 2013;48:1794–805.

    Article  Google Scholar 

  55. Intestinal Transplant Registry. http://www.intransplant/org.

  56. Abi Nader E, Lambe C, Talbotec C, Pigneur B, Lacaille F, Garnier-Lengliné H, et al. Outcome of home parenteral nutrition in 251 children over a 14-y period: report of a single center. Am J Clin Nutr. 2016;103:1327–36.

    Article  Google Scholar 

  57. Burghardt KM, Wales PW, de Silva N, Stephens D, Yap J, Grant D, Avitzur Y. Pediatric intestinal transplant listing criteria. A call for a change in the new era of intestinal failure outcomes. Am J Transplant. 2015;15:1674–81.

    Article  CAS  Google Scholar 

  58. Grant D, Abu-Elmagd K, Mazariegos G, Vianna R, Langnas A, Mangus R, et al. Intestinal transplant association. Intestinal transplant registry report: global activity and trends. Am J Transplant. 2015;15:210–9.

    Article  CAS  Google Scholar 

  59. Lacaille F, Irtan S, Dupic L, Talbotec C, Lesage F, Colomb V, et al. Twenty-eight years of intestinal transplantation in Paris: experience of the oldest European center. Transpl Int. 2017;30:178–86.

    Article  Google Scholar 

  60. Taha AMI, Sharif K, Johnson T, Clarke S, Murphy MS, Gupte GL. Long-term outcomes of isolated liver transplantation for short bowel syndrome and intestinal failure-associated liver disease. J Pediatr Gastroenterol Nutr. 2012;54:547–51.

    Article  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Florence Lacaille .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2019 Springer Nature Switzerland AG

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Lacaille, F. (2019). Nutrition and Liver Disease. In: D'Antiga, L. (eds) Pediatric Hepatology and Liver Transplantation. Springer, Cham. https://doi.org/10.1007/978-3-319-96400-3_23

Download citation

  • DOI: https://doi.org/10.1007/978-3-319-96400-3_23

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-96399-0

  • Online ISBN: 978-3-319-96400-3

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics