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Chapter 9 - Medical and Nutritional Management of Cholestasis in Infants and Children

from Section II - Cholestatic Liver Disease

Published online by Cambridge University Press:  19 January 2021

Frederick J. Suchy
Affiliation:
University of Colorado, Children’s Hospital Colorado, Aurora
Ronald J. Sokol
Affiliation:
University of Colorado, Children’s Hospital Colorado, Aurora
William F. Balistreri
Affiliation:
Cincinnati Children’s Hospital Medical Center, Cincinnati
Jorge A. Bezerra
Affiliation:
Cincinnati Children’s Hospital Medical Center, Cincinnati
Cara L. Mack
Affiliation:
University of Colorado, Children’s Hospital Colorado, Aurora
Benjamin L. Shneider
Affiliation:
Texas Children’s Hospital, Houston
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Summary

When first encountering an infant or child with cholestatic liver disease, it is essential that diagnostic evaluation be conducted promptly in order to recognize disorders amenable either to specific medical therapy (e.g., galactosemia, tyrosinemia, hypothyroidism, urinary tract infection) or to early surgical intervention (e.g., biliary atresia, choledochal cyst); institute treatment directed toward enhancing bile flow; and prevent and treat the varied medical, nutritional, and emotional consequences of chronic liver disease.

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Publisher: Cambridge University Press
Print publication year: 2021

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References

Shneider, BL, Moore, J, Kerkar, N, Magee, JC, Ye, W, Karpen, SJ, Kamath, BM, et al. Initial assessment of the infant with neonatal cholestasis-Is this biliary atresia? PLoS One 2017;12:e0176275.Google Scholar
Feldman, AG, Sokol, RJ. Neonatal cholestasis: emerging molecular diagnostics and potential novel therapeutics. Nat Rev Gastroenterol Hepatol 2019;16:346–60.Google Scholar
Lane, E, Murray, KF. cholestasis, Neonatal. Pediatr Clin North Am 2017;64:621–39.Google Scholar
Squires, JE, McKiernan, P. Molecular mechanisms in pediatric cholestasis. Gastroenterol Clin North Am 2018;47:921–37.Google Scholar
Sticova, E, Jirsa, M, Pawlowska, J. New insights in genetic cholestasis: from molecular mechanisms to clinical implications. Can J Gastroenterol Hepatol 2018;2018:2313675.Google Scholar
Li, Y, Lu, LG. Therapeutic roles of bile acid signaling in chronic liver diseases. J Clin Transl Hepatol 2018;6:425–30.Google Scholar
Jones, H, Alpini, G, Francis, H. Bile acid signaling and biliary functions. Acta Pharm Sin B 2015;5:123–8.CrossRefGoogle ScholarPubMed
Lazaridis, KN, LaRusso, NF. Primary sclerosing cholangitis. N Engl J Med 2016;375:2501–2.Google Scholar
Deneau, M, Perito, E, Ricciuto, A, Gupta, N, Kamath, BM, Palle, S, Vitola, B, et al. Ursodeoxycholic acid therapy in pediatric primary sclerosing cholangitis: predictors of gamma glutamyltransferase normalization and favorable clinical course. J Pediatr 2019;209:92–6.Google Scholar
Cheng, K, Ashby, D, Smyth, RL. Ursodeoxycholic acid for cystic fibrosis-related liver disease. Cochrane Database Syst Rev 2017;9:CD000222.Google Scholar
Boelle, PY, Debray, D, Guillot, L, Clement, A, Corvol, H, French CF Modifier Gene Study Investigators. Cystic fibrosis liver disease: outcomes and risk factors in a large cohort of French patients. Hepatology 2019;69:1648–56.CrossRefGoogle Scholar
Balistreri, WF. Bile acid therapy in pediatric hepatobiliary disease: the role of ursodeoxycholic acid. J Pediatr Gastroenterol Nutr 1997;24:573–89.Google Scholar
Jacquemin, E, Hermans, D, Myara, A, Habes, D, Debray, D, Hadchouel, M, Sokal, EM, et al. Ursodeoxycholic acid therapy in pediatric patients with progressive familial intrahepatic cholestasis. Hepatology 1997;25:519–23.CrossRefGoogle ScholarPubMed
Heubi, JE, Setchell, KD, Jha, P, Buckley, D, Zhang, W, Rosenthal, P, Potter, C, et al. Treatment of bile acid amidation defects with glycocholic acid. Hepatology 2015;61:268–74.CrossRefGoogle ScholarPubMed
Ruutu, T, Eriksson, B, Remes, K, Juvonen, E, Volin, L, Remberger, M, Parkkali, T, et al. Ursodeoxycholic acid for the prevention of hepatic complications in allogeneic stem cell transplantation. Blood 2002;100:1977–83.CrossRefGoogle ScholarPubMed
Bezerra, JA, Spino, C, Magee, JC, Shneider, BL, Rosenthal, P, Wang, KS, Erlichman, J, et al. Use of corticosteroids after hepatoportoenterostomy for bile drainage in infants with biliary atresia: the START randomized clinical trial. JAMA 2014;311:1750–9.Google Scholar
Chiang, JYL, Ferrell, JM. Bile acids as metabolic regulators and nutrient sensors. Annu Rev Nutr 2019;39:175200.Google Scholar
Gitto, S, Guarneri, V, Sartini, A, Andreone, P. The use of obeticholic acid for the management of non-viral liver disease: current clinical practice and future perspectives. Expert Rev Gastroenterol Hepatol 2018;12:165–71.Google Scholar
Koyama, Y, Brenner, DA. Liver inflammation and fibrosis. J Clin Invest 2017;127:5564.CrossRefGoogle ScholarPubMed
Koyama, Y, Xu, J, Liu, X, Brenner, DA. New developments on the treatment of liver fibrosis. Dig Dis 2016;34:589–96.CrossRefGoogle ScholarPubMed
Bergasa, NV. The itch of liver disease. Semin Cutan Med Surg 2011;30:93–8.CrossRefGoogle ScholarPubMed
Thebaut, A, Debray, D, Gonzales, E. An update on the physiopathology and therapeutic management of cholestatic pruritus in children. Clin Res Hepatol Gastroenterol 2018;42:103–9.CrossRefGoogle ScholarPubMed
Hegade, VS, Bolier, R, Oude Elferink, RP, Beuers, U, Kendrick, S, Jones, DE. A systematic approach to the management of cholestatic pruritus in primary biliary cirrhosis. Frontline Gastroenterol 2016;7:158–66.Google Scholar
Kremer, AE, van Dijk, R, Leckie, P, Schaap, FG, Kuiper, EM, Mettang, T, Reiners, KS, et al. Serum autotaxin is increased in pruritus of cholestasis, but not of other origin, and responds to therapeutic interventions. Hepatology 2012;56:13911400.Google Scholar
Meixiong, J, Vasavda, C, Green, D, Zheng, Q, Qi, L, Kwatra, SG, Hamilton, JP, et al. Identification of a bilirubin receptor that may mediate a component of cholestatic itch. Elife 2019;8:e44116.Google Scholar
De Vloo, C, Nevens, F. Cholestatic pruritus: an update. Acta Gastroenterol Belg 2019;82:7582.Google Scholar
Yerushalmi, BSR, Narkewicz, MR, Smith, D, Karrer, FM. Use of rifampin for severe pruritus in children with chronic cholestasis. J Pediatr Gastroenterol Nutr 1999;29(4):442–7.Google Scholar
Wolfhagen, FH, Sternieri, E, Hop, WC, Vitale, G, Bertolotti, M, Van Buuren, HR. Oral naltrexone treatment for cholestatic pruritus: a double-blind, placebo-controlled study. Gastroenterology 1997;113:1264–9.Google Scholar
Shneider, BL, Spino, C, Kamath, BM, Magee, JC, Bass, LM, Setchell, KD, Miethke, A, et al. Placebo-controlled randomized trial of an intestinal bile salt transport inhibitor for pruritus in Alagille syndrome. Hepatol Commun 2018;2:1184–98.Google Scholar
Hegade, VS, Kendrick, SF, Dobbins, RL, Miller, SR, Thompson, D, Richards, D, Storey, J, et al. Effect of ileal bile acid transporter inhibitor GSK2330672 on pruritus in primary biliary cholangitis: a double-blind, randomised, placebo-controlled, crossover, phase 2a study. Lancet 2017;389:1114–23.Google Scholar
Bull, LN, Pawlikowska, L, Strautnieks, S, Jankowska, I, Czubkowski, P, Dodge, JL, Emerick, K, et al. Outcomes of surgical management of familial intrahepatic cholestasis 1 and bile salt export protein deficiencies. Hepatol Commun 2018;2:515–28.Google Scholar
Hegade, VS, Krawczyk, M, Kremer, AE, Kuczka, J, Gaouar, F, Kuiper, EM, van Buuren, HR, et al. The safety and efficacy of nasobiliary drainage in the treatment of refractory cholestatic pruritus: a multicentre European study. Aliment Pharmacol Ther 2016;43:294302.CrossRefGoogle ScholarPubMed
Austin, PW, Gerber, L, Karrar, AK. Fatigue in chronic liver disease: exploring the role of the autonomic nervous system. Liver Int 2015;35:1489–91.Google Scholar
Dyson, JK, Elsharkawy, AM, Lamb, CA, Al-Rifai, A, Newton, JL, Jones, DE, Hudson, M. Fatigue in primary sclerosing cholangitis is associated with sympathetic over-activity and increased cardiac output. Liver Int 2015;35:1633–41.Google Scholar
Black, DD. Chronic cholestasis and dyslipidemia: what is the cardiovascular risk? J Pediatr 2005;146:306–7.Google Scholar
Longo, M, Crosignani, A, Podda, M. Hyperlipidemia in chronic cholestatic liver disease. Curr Treat Options Gastroenterol 2001;4:111–14.Google Scholar
Yu, RWY, Xiao, Y, Mo, L, Liu, A, Li, D, Ge, T, Yu, G, Zhang, T. Prevalence of malnutrition and risk of undernutrition in hospitalised children with liver disease. J Nutr Sci 2017;6:e55:15.Google Scholar
Chin, SE, Shepherd, RW, Thomas, BJ, Cleghorn, GJ, Patrick, MK, Wilcox, JA, Ong, TH, 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.Google Scholar
Bucuvalas, JC, Cutfield, W, Horn, J, Sperling, MA, Heubi, JE, Campaigne, B, Chernausek, SD. Resistance to the growth-promoting and metabolic effects of growth hormone in children with chronic liver disease. J Pediatr 1990;117:397402.Google Scholar
Sokol, RJ, Stall, C. Anthropometric evaluation of children with chronic liver disease. Am J Clin Nutr 1990;52:203–8.CrossRefGoogle ScholarPubMed
Hogler, W, Baumann, U, Kelly, D. Growth and bone health in chronic liver disease and following liver transplantation in children. Pediatr Endocrinol Rev 2010;7:266–74.Google Scholar
Loomes, KM, Spino, C, Goodrich, NP, Hangartner, TN, Marker, AE, Heubi, JE, Kamath, BM, et al. Bone density in children with chronic liver disease correlates with growth and cholestasis. Hepatology 2019;69:245–57.CrossRefGoogle ScholarPubMed
Mandato, C, Di Nuzzi, A, Vajro, P. Nutrition and liver disease. Nutrients 2017;10(1):9.Google Scholar
DeRusso, P, Ye, W, Shepherd, R, Haber, BA, Shneider, BL, Whitington, PF, Schwarz, KB, Bezerra, JA, et al. Growth failure and outcomes in infants with biliary atresia: a report from the Biliary Atresia Research Consortium. Hepatology 2007;46:1632–8.Google Scholar
Sullivan, JS, Sundaram, SS, Pan, Z, Sokol, RJ. Parenteral nutrition supplementation in biliary atresia patients listed for liver transplantation. Liver Transpl 2012;18:120–8.Google Scholar
Nightingale, S, Ng, VL. Optimizing nutritional management in children with chronic liver disease. Pediatr Clin North Am 2009;56:1161–83.Google Scholar
Yang, CH, Perumpail, BJ, Yoo, ER, Ahmed, A, Kerner, JA Jr. Nutritional needs and support for children with chronic liver disease. Nutrients 2017;9(10):1127.Google Scholar
Enguita, M, Razquin, N, Pamplona, R, Quiroga, J, Prieto, J, Fortes, P. The cirrhotic liver is depleted of docosahexaenoic acid (DHA), a key modulator of NF-kappaB and TGFbeta pathways in hepatic stellate cells. Cell Death Dis 2019;10:14.Google Scholar
Sokol, RJ. Fat-soluble vitamins and their importance in patients with cholestatic liver diseases. Gastroenterol Clin North Am 1994;23:673705.Google Scholar
Shen, YM, Wu, JF, Hsu, HY, Ni, YH, Chang, MH, Liu, YW, Lai, HS, et al. Oral absorbable fat-soluble vitamin formulation in pediatric patients with cholestasis. J Pediatr Gastroenterol Nutr 2012;55:587–91.Google Scholar
Shneider, BL, Magee, JC, Bezerra, JA, Haber, B, Karpen, SJ, Raghunathan, T, Rosenthal, P, et al. Efficacy of fat-soluble vitamin supplementation in infants with biliary atresia. Pediatrics 2012;130:e607–14.Google Scholar
Feranchak, AP, Gralla, J, King, R, Ramirez, RO, Corkill, M, Narkewicz, MR, Sokol, RJ. Comparison of indices of vitamin A status in children with chronic liver disease. Hepatology 2005;42:782–92.Google Scholar
Sathe, MN, Patel, AS. Update in pediatrics: focus on fat-soluble vitamins. Nutr Clin Pract 2010;25:340–6.Google Scholar
Sokol, RJ. Assessing vitamin E status in childhood cholestasis. J Pediatr Gastroenterol Nutr 1987;6:1013.Google Scholar
Sokol, RJ, Heubi, JE, Butler-Simon, N, McClung, HJ, Lilly, JR, Silverman, A. Treatment of vitamin E deficiency during chronic childhood cholestasis with oral d-alpha-tocopheryl polyethylene glycol-1000 succinate. Gastroenterology 1987;93:975–85.Google Scholar
Sokol, RJ. A new old treatment for vitamin E deficiency in cholestasis. J Pediatr Gastroenterol Nutr 2016;63:577–8.Google Scholar
Sokol, RJ, Butler-Simon, N, Conner, C, Heubi, JE, Sinatra, FR, Suchy, FJ, Heyman, MB, et al. Multicenter trial of d-alpha-tocopheryl polyethylene glycol 1000 succinate for treatment of vitamin E deficiency in children with chronic cholestasis. Gastroenterology 1993;104:1727–35.CrossRefGoogle ScholarPubMed
Haney, S, Harper, J, Truemper, E. Progressive familial intrahepatic cholestasis presenting with an intracranial bleed and mimicking abusive head trauma. WMJ 2019;118:47–8.Google Scholar
Yanofsky, RA, Jackson, VG, Lilly, JR, Stellin, G, Klingensmith, WC, 3rd, Hathaway, WE. The multiple coagulopathies of biliary atresia. Am J Hematol 1984;16:171–80.CrossRefGoogle ScholarPubMed
Heubi, JE, Higgins, JV, Argao, EA, Sierra, RI, Specker, BL. 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.Google Scholar
Mattar, RH, Azevedo, RA, Speridiao, PG, Fagundes Neto, U, Morais, MB. Nutritional status and intestinal iron absorption in children with chronic hepatic disease with and without cholestasis. J Pediatr 2005;81:317–24.Google Scholar
Gold, A, Rogers, A, Cruchley, E, Rankin, S, Parmar, A, Kamath, BM, Avitzur, Y, et al. Assessment of school readiness in chronic cholestatic liver disease: a pilot study examining children with and without liver transplantation. Can J Gastroenterol Hepatol 2017;2017:9873945.Google Scholar
Ng, VL, Sorensen, LG, Alonso, EM, Fredericks, EM, Ye, W, Moore, J, Karpen, SJ, et al. Neurodevelopmental outcome of young children with biliary atresia and native liver: results from the ChiLDReN Study. J Pediatr 2018;196:139–47 e133.Google Scholar
Feldman, AG, Kempe, A, Beaty, BL, Sundaram, SS. Studies of Pediatric Liver Transplantation Research Group. Immunization practices among pediatric transplant hepatologists. Pediatr Transplant 2016;20:1038–44.Google Scholar

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