Abstract
The liver is the major organ for maintaining normal homeostasis of both macronutrients (proteins, carbohydrates, and fat including essential fatty acids) and micronutrients (vitamins and minerals). Therefore, children with significant liver disease, especially those with chronic cholestasis, cirrhosis, or end-stage liver disease, often demonstrate impaired nutrient metabolism as well as possible malabsorption or maldigestion of nutrients. Routine assessments of nutritional status, appropriate supplementations, and nutritional intervention or rehabilitation (if needed) are crucial in managing these children. The roles of various diets in children who suffer from end-stage liver disease and complications from hepatic dysfunction such as hepatic encephalopathy or ascites have been widely studied. During the past two decades, many children with chronic end-stage or life-threatening liver disease have undergone liver transplantation as a therapeutic option. Nutritional support also plays an essential role in managing these children both before and after the transplantation.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Gabay C, Kushner I. Acute-phase proteins and other systemic responses to inflammation. N Engl J Med. 1999;340:448–54.
Chrousos GP. The hypothalamic-pituitary-adrenal axis and immune-mediated inflammation. N Engl J Med. 1995;332:1351–62.
Karalis K, Sano H, Redwine J, Listwak S, Wilder RL, Chrousos GP. Autocrine or paracrine inflammatory actions of corticotropin-releasing hormone in vivo. Science. 1991;254:421–3.
Sarraf P, Frederich RC, Turner EM, et al. Multiple cytokines and acute inflammation raise mouse leptin levels: potential role in inflammatory anorexia. J Exp Med. 1997;185:171–5.
Muller MJ, Pirlich M, Balks HJ, Selberg O. Glucose intolerance in liver cirrhosis: role of hepatic and non-hepatic influences. Eur J Clin Chem Clin Biochem. 1994;32:749–58.
Petrides AS, DeFronzo RA. Glucose and insulin metabolism in cirrhosis. J Hepatol. 1989;8:107–14.
Muller MJ, Willman O, Rieger A, et al. Mechanism of insulin resistance associated with liver cirrhosis. Gastroenterology. 1992;102:2033–41.
Selberg O, Burchert W, van der Hoff J, et al. Insulin resistance in cirrhosis. Positron emission tomography scan analysis of skeletal muscle glucose metabolism. J Clin Invest. 1993;91:1897–902.
Petrides AS, Luzi L, Reuben A, Riely C, De Fronzo RA. Effect of insulin and plasma amino acid concentration on leucine metabolism in cirrhosis. Hepatology. 1991;14:432–41.
Freeman Jr RB, Wisener RH, Harper A, et al. The new liver allocation system: moving toward evidence-based transplantation policy. Liver Transpl. 2002;8:851–8.
Owen OE, Trapp VE, Reichard JA, et al. Nature and quantity of fuels consumed in patients with alcoholic cirrhosis. J Clin Invest. 1983;72:1821–32.
Merli M, Erickson SL, Hagenfeldt H, Wahren J. Splanchnic and peripheral exchange of FFA in patients with liver cirrhosis. J Hepatol. 1986;3:348–55.
Shneider BL, Magee JC, Bezerra JA, Haber B, Karpen SJ, Raghunathan T, et al. Efficacy of fat-soluble vitamin supplementation in infants with biliary atresia. Pediatrics. 2012;130:e607–14.
Ryckman F, Fisher R, Pedersen S, Dittrich V, et al. Improved survival in biliary atresia patients in the present era of liver transplantation. J Pediatr Surg. 1993;28:382–5.
Florez DA, Aranda-Michel J. Nutritional management of acute and chronic liver disease. Semin Gastrointest Dis. 2002;13:169–78.
Dowling RH. Compensatory changes in intestinal absorption. Br Med Bull. 1967;23:275–8.
Bahr MJ, Manns MP. Function of the immune system in liver cirrhosis. Gastroenterology. 1993;39:601–7.
Badley BWD, Murphy GM, Bouchier IAD, et al. Diminished micellar phase lipid in patients with chronic nonalcoholic liver disease and steatorrhea. Gastroenterology. 1970;58:781–9.
Sanders T. Essential fatty acids. In: Macrae R, Robinson RK, Sadler MJ, editors. Encyclopaedia of food science, food technology, and nutrition. London: Academic; 1993. p. 1651–4.
Hansen AE, Haggard ME, Boelsche AN, et al. Essential fatty acids in infant nutrition III. Clinical manifestations of linoleic acid deficiency. J Nutr. 1958;60:565–76.
Holman RT, Johnson SB, Hatch F. A case of human linolenic acid deficiency involving neurological abnormalities. Am J Clin Nutr. 1982;35:617–23.
Wene JD, Connor WE, Den Besten L. The development of EFA deficiency in healthy men fed fat free diets intravenously and orally. J Clin Invest. 1975;56:127–34.
Clandinin MT, Chappell JE, Heim T, et al. Fatty acid utilization in perinatal de novo synthesis of tissues. Early Hum Dev. 1981;5:355–66.
Pettei MJ, Daftary S, Levine JJ. EFA deficiency associated with the use of a MCT infant formula in pediatric hepatobiliary disease. Am J Clin Nutr. 1991;53:1217–21.
Neuringer M, Connor WE, Lin DS, Barstad L, Luck SJ. Biochemical and functional effects of prenatal and postnatal omega-3 fatty acid deficiency on retina and brain in rhesus monkeys. Proc Natl Acad Sci U S A. 1986;83:185–294.
Birch DG, Birch EE, Hoffman DR, Uauy RD. Retinal development in very low birth weight infants fed diets differing in omega-3 fatty acids. Invest Ophthalmol Vis Sci. 1992;33:2365–76.
Carlson SE, Werkman SH, Tolley EA. Effect of long-chain N-3 fatty acid supplementation on visual acuity and growth of preterm infants with and without bronchopulmonary dysplasia. Am J Clin Nutr. 1996;63(5):687–97.
Chase HP, Long MA, Lavin MH. Cystic fibrosis and malnutrition. J Pediatr. 1979;95:337–47.
Bender DA, Bender AE. Nutrition, a reference handbook. Oxford: Oxford University Press; 1997. p. 131–3.
Duerksen DR, Nehra V, Palombo JD, Ahmad A, Bristrian BR. Essential fatty acid deficiencies in patients with chronic liver disease are not reversed by short-term intravenous lipid supplementation. Dig Dis Sci. 1999;44:1342–8.
Burke PA, Ling PR, Forse RA, Bristrian BR. Conditionally essential fatty acid deficiencies in end-stage liver disease. Nutrition. 1999;15:302–4.
Kaufman SS, Scrivner DJ, Murray ND, et al. Influence of portagen and pregestimil on essential fatty acid status in infantile liver disease. Pediatrics. 1992;89:151–4.
Feranchak AP, Gralla J, King R, et al. Comparison of indices of vitamin A status in children with chronic liver disease. Hepatology. 2005;42:782–92.
Phillips JR, Angulo P, Petterson T, et al. Fat-soluble vitamin levels in patients with primary biliary cirrhosis. Am J Gastroenterol. 2001;96:2745–50.
Sherwin JC, Reacher MH, Dean WH, et al. Epidemiology of vitamin A deficiency and xerophthalmia in at-risk populations. Trans R Soc Trop Med Hyg. 2012;106:205–14.
Lippe B, Hensen L, Mendoza G, et al. Chronic vitamin A intoxication. Am J Dis Child. 1981;135:634–6.
Institute of Medicine, Food and Nutrition Board. Dietary reference intakes: vitamin A, vitamin K, arsenic, boron, chromium, copper, iodine, iron, manganese, molybdenum, nickel, silicon, vanadium, and zinc. Washington, DC: National Academy Press; 2001.
Penniston KL, Tanumihardjo SA. The acute and chronic toxic effects of vitamin A. Am J Clin Nutr. 2006;83:191–201.
Institute of Medicine (US) Committee to Review Dietary Reference Intakes for Vitamin D and Calcium, Ross AC, Taylor CL, Yaktine AL, Del Valle HB, editors. Dietary reference intakes for calcium and vitamin D. Washington, DC: National Academies Press; 2011.
Holick MF, Binkley NC, Bischoff-Ferrari HA, et al. Endocrine Society. Evaluation, treatment, and prevention of vitamin D deficiency: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab. 2011;96:1911–30.
Heubi JE, Hollis BW, Specker B, et al. Bone disease in chronic childhood cholestasis. Vitamin D adsorption and metabolism. Hepatology. 1989;9:258–64.
Bucuvalas JC, Heubi JE, Specker BL, et al. Calcium absorption in bone disease associated with liver cholestasis during childhood. Hepatology. 1990;12:1200–5.
Heubi JE, Higgins JV, Argao EA, et al. The role of magnesium in the pathogenesis of bone disease in childhood cholestatic liver disease: a preliminary report. J Pediatr Gastroenterol Nutr. 1997;25:301–6.
Argao EA, Balistreri WF, Hollis BW, et al. Effect of orthotopic liver transplantation on bone mineral content and serum vitamin D metabolites in infants and children with chronic cholestasis. Hepatology. 1994;20:598–603.
Institute of Medicine, Food and Nutrition Board. Dietary reference intakes for calcium and vitamin D. Washington, DC: National Academy Press; 2010.
Sokol RJ, Heubi JE, Iannaccone S, et al. Mechanisms causing vitamin E deficiency during chronic childhood cholestasis. Gastroenterology. 1983;85:1172–82.
Sokol RJ, Heubi JE, Iannaccone ST, et al. Vitamin E deficiency with normal serum vitamin E concentration in children with chronic cholestasis. N Engl J Med. 1984;310:1209–12.
Gordon HH, Nitowsky HM, Cornblath M. Studies of tocopherol deficiency in infants and children. 1. Hemolysis of erythrocytes in hydrogen peroxide. Am J Dis Child. 1955;90:669–81.
Cynamon HA, Isenberg JN, Nguyen CH. Erythrocyte malondialdehyde release in vitro: a functional measure of vitamin E status. Clin Chim Acta. 1985;151:169–76.
Refat M, Moore TJ, Kazui M, et al. Utility of breathe ethane as a noninvasive biomarker of vitamin E status in children. Pediatr Res. 1991;30:396–403.
Sokol RJ, Guggenheim MA, Iannaccone ST, et al. Improved neurologic function flowing correction of vitamin E deficiency in children with chronic cholestasis. N Engl J Med. 1985;313:1580–6.
Olson RE. The function and metabolism of vitamin K. Annu Rev Nutr. 1984;4:281–337.
Bancroft J, Cohen MB. Intracranial hemorrhage due to vitamin K deficiency in breast fed infants with cholestasis. J Pediatr Gastroenterol Nutr. 1993;16:78–80.
Yanofsky RA, Jackson VG, Lilly JR, et al. The multiple coagulopathies of biliary atresia. Am J Hematol. 1984;16:171–80.
Hambridge KM, Casey CE, Krebs NF. Zinc. In: Mertz W, editor. Trace elements in human and animal nutrition, vol. 2. 5th ed. Florida: Academic; 1986. p. 12–4.
Mohammad MK, Zhou Z, Cave M, Barve A, McClain CJ. Zinc and liver disease. Nutr Clin Pract. 2012;27:8–20.
Sazawal S, Black RE, Bhan MK, et al. Zinc supplementation in young children with acute diarrhea in India. N Engl J Med. 1995;333:839–44.
Roy SK, Tomkins AM, Akramuzzaman SM, et al. Randomised controlled trial of zinc supplementation in malnourished Bangladeshi children with acute diarrhoea. Arch Dis Child. 1997;77:196–200.
Ferenci P, Steindl-Munda P, Vogel W, et al. Diagnostic value of quantitative hepatic copper determination in patients with Wilson’s Disease. Clin Gastroenterol Hepatol. 2005;3:811–8.
Grunebaum M, Horodniceanu C, Steinherz R. The radiographic manifestations of bone changes in copper deficiency. Pediatr Radiol. 1980;9:101–4.
Jiser SR, Winston GP. Copper deficiency myelopathy. J Neurol. 2010;2100:869–81.
Keshan Disease Research Group. Epidemiologic studies on the etiologic relationship of selenium and Keshan disease. Chin Med J (Engl). 1979;92:477–82.
Rotruck JT, et al. Selenium: biochemical role as a component of glutathione peroxidase. Science. 1973;179:588–90.
Fleming CR, Lie JT, McCall JT, et al. Selenium deficiency and fatal cardiomyopathy in a patient on home parenteral nutrition. Gastroenterology. 1982;83:689–93.
van Rij A, Thomson CD, McKenzie JM, Robinson MF. Selenium deficiency in total parenteral nutrition. Am J Clin Nutr. 1979;32:2076–85.
Thuluvath PJ, Triger DR. Selenium in chronic liver disease. J Hepatol. 1992;14:176–82.
Chin SE, Shepherd RW, Thomas BJ, 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.
Levander OA. Considerations on the assessment of selenium status. Fed Proc. 1985;44:2579–83.
Mertz W. Chromium occurrence and function in biological systems. Physiol Rev. 1969;49:163–239.
Freund H, Atamiam S, Fischer JE. Chromium deficiency during total parenteral nutrition. JAMA. 1979;241:496–8.
Jeejeebhoy KN, Chu RC, Marliss EB, Greenberg GR, Bruce-Robertson A. Chromium deficiency, glucose intolerance, and neuropathy reversed by chromium supplementation in a patient receiving long-term total parenteral nutrition. Am J Clin Nutr. 1977;30:531–8.
Bavdekar A, Bhave S, Pandit A. Symposium on hospital-based nutrition: nutrition management in chronic liver disease. J Trauma Inj Infect Crit Care. 2004;56:37–44.
Eriksson LS, Persson A, Wahren J. Branched-chain amino acids in the treatment of chronic hepatic encephalopathy. Gut. 1982;23:801–6.
Wahren J, Denis J, Desurmont P, et al. Is intravenous administration of branched chain amino acids effective in the treatment of hepatic encephalopathy? A multicenter study. Hepatology. 1983;3:475–80.
Horst D, Grace ND, Conn HO, et al. Comparison of dietary protein with an oral, branched chain-enriched amino acid supplement in chronic portal-systemic encephalopathy: a randomized controlled trial. Hepatology. 1984;4:279–87.
Cascino A, Cangioano C, Calcaterra V, et al. Plasma amino acids imbalance in patients with liver disease. Am J Dig Dis. 1978;23:591–8.
Rossi-Francelli F, Freund H, Krause R, et al. Induction of coma in normal dogs by the infusion of aromatic amino acids and its prevention by the addition of branched-chain amino acids. Gastroenterology. 1982;83:664–71.
Fisher JE, Baldessarini RJ. False neurotransmitters and hepatic failure. Lancet. 1971;ii:75–80.
Rossi-Francelli F, Riggio O, Cangiano C, et al. Branched-chain amino acids vs lactulose in the treatment of hepatic coma: a controlled study. Dig Dis Sci. 1982;27:929–35.
McGhee A, Henderson JM, Millikan Jr WJ, et al. Comparison of the effects of hepatic-aid and casein modular diet on encephalopathy, plasma amino acids, and nitrogen balance in cirrhotic patients. Ann Surg. 1983;197:288–93.
Fabbri A, Magrini N, Bianchi G, et al. Overview of randomized clinical trials of oral branched-chain fatty acid treatment in chronic hepatic encephalopathy. JPEN J Parenter Enteral Nutr. 1996;20:159–64.
Charlton CP, Buchanan E, Holden CE, et al. Intensive enteral feeding in advanced cirrhosis: reversal of malnutrition without precipitation of hepatic encephalopathy. Arch Dis Child. 1992;67:603–7.
Helton WS. Nutritional issues in hepatobiliary surgery. Semin Liver Dis. 1994;14:140–57.
Fan ST, Lo CM, Lai ECS, Chu KM, Liu CL, Wong J. Perioperative nutritional support in patients undergoing hepatectomy for hepatocellular carcinoma. N Engl J Med. 1994;331:1547–52.
Garrison RN, Cryer HM, Howard DA, Polk HC. Clarification of risk factors for abdominal operations in patients with hepatic cirrhosis. Ann Surg. 1984;199:648–55.
Bonkovsky HL, Fiellin DA, Smith GS, et al. A randomized controlled trial of treatment of alcoholic hepatitis with parenteral nutrition and oxandrolone. 1. Short-term effects on liver function. Am J Gastroenterol. 1991;86:1200–8.
Kearns PJ, Young H, Garcia G, et al. Accelerated improvement of alcoholic liver disease with enteral nutrition. Gastroenterology. 1992;102:200–5.
Cabre E, Gonzalez-Huix F, Abad-Lacruz A, et al. Effect of total enteral nutrition on the short-term outcome of severely malnourished cirrhotics. A randomized controlled trial. Gastroenterology. 1990;98:715–20.
Moukarzel AA, Najm I, Vargas J, et al. Effect of nutritional status on outcome of orthotopic liver transplantation in pediatric patients. Transplant Proc. 1900;22:1560–3.
Pikul J, Sharpe MD, Lowndes R, Ghent CN. Degree of preoperative malnutrition is predictive of postoperative morbidity and mortality in liver transplant recipients. Transplantation. 1994;57:469–72.
Prijatmoko D, Strauss BJG, Lambert JR, et al. Early detection of protein depletion in alcoholic cirrhosis: role of body composition analysis. Gastroenterology. 1993;105:1839–45.
Heymsfield SB, Artega C, McManus C, Smith J, Moffitt S. Measurement of muscle mass in humans: validity of the 24-hour urinary creatinine method. Am J Clin Nutr. 1983;37:478–94.
Mendenhall CL, Moritz TE, Roselle GA, et al. A study of oral nutritional support with oxandrolone in malnourished patients with alcoholic hepatitis: results of a department of veterans affairs cooperative study. Hepatology. 1993;17:564–76.
Thomas S, Wolfe SE, Murphy KD, Chinkes DL, Herndon DN. The long-term effect of oxandrolone on hepatic acute phase proteins in severely burned children. J Trauma Inj Infect Crit Care. 2004;56:32–44.
Verboeket-van de Venne WPGH, Westerp 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.
Chin SE, Shepherd RW, Thomas BJ, 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.
deLedinghen V, Beau P, Mannant PR, et al. Early feeding or enteral nutrition in patients with cirrhosis after bleeding from esophageal varices? A randomized controlled study. Dig Dis Sci. 1997;42:536–41.
Gura KM, Duggan CP, Collier SB, et al. Reversal of parenteral nutrition-associated liver disease in two infants with short bowel syndrome using parenteral fish oil: implications for future management. Pediatrics. 2006;118:e197–201.
Le HD, deMeijer VE, Robinson EM, et al. Parenteral fish-oil-based lipid emulsion improves fatty acid profiles and lipids in parenteral nutrition-0 dependent children. Am J Clin Nutr. 2011;94:749–58.
Diamond IR, Sterescu A, Pencharz PB, et al. Changing the paradigm: omegaven for the treatment of liver failure in pediatric short bowel syndrome. J Pediatr Gastroenterol Nutr. 2009;48:209–15.
Xu Z, Li Y, Wang J, et al. Effect of omega-3 polyunsaturated fatty acids to reverse biopsy-proven parenteral nutrition-associated liver disease in adults. Clin Nutr. 2012;31:217–23.
Michel H, Bories P, Aubin JP, et al. Treatment of acute hepatic encephalopathy in cirrhotics with a branched-chain amino acids enriched versus a conventional amino acid mixture: a controlled study in 70 patients. Liver. 1985;5:282–9.
Vilstrup H, Gluud C, Hardt F, et al. Branched-chain enriched amino acid versus glucose treatment of hepatic encephalopathy. A double-blind study of 65 patients with cirrhosis. J Hepatol. 1990;10:291–6.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2014 Springer Science+Business Media New York
About this chapter
Cite this chapter
Hardy, S.C., Tanpowpong, P., Kleinman, R.E. (2014). Nutrition in Children with Liver Disease: Evaluation and Management. In: Murray, K., Horslen, S. (eds) Diseases of the Liver in Children. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-9005-0_27
Download citation
DOI: https://doi.org/10.1007/978-1-4614-9005-0_27
Published:
Publisher Name: Springer, New York, NY
Print ISBN: 978-1-4614-9004-3
Online ISBN: 978-1-4614-9005-0
eBook Packages: MedicineMedicine (R0)