Pediatric Gastroenterologist and the Infant and Child Before Liver and Small Bowel Transplantation

  • Sana Mansoor
  • Katryn N. Furuya
Reference work entry
Part of the Organ and Tissue Transplantation book series (OTT)


Pediatric liver transplantation is the standard of care for children with acute liver failure or end-stage liver disease and has excellent outcomes with 5-year survival rates greater than 85%. The most common cause for liver transplantation in children is biliary atresia unlike in adults where end-stage liver disease is most commonly due to nonalcoholic steatohepatitis (NASH). Similarly, intestinal transplantation is offered to those children with intestinal failure who were unable to achieve intestinal rehabilitation or who were born with congenital/inherited diseases such as microvillus inclusion disease. Three-year survival rates with pediatric intestinal transplantation are greater than 65%. The role of the pediatric gastroenterologist/hepatologist is in the early recognition of children who meet the criteria for transplantation and refer them to an appropriate transplantation center for assessment and evaluation. Pediatric gastroenterologists/hepatologists work in a team setting with pediatric surgeons/transplant surgeons, nutritionists, social work, other pediatric providers and nursing to provide children who are awaiting transplantation optimization of their clinical status and to prevent or treat complications associated with end-stage liver disease or intestinal failure which play a critical role in posttransplant outcomes. This chapter focuses on these topics in detail.


Liver transplantation Small intestinal transplantation Cirrhosis Portal hypertension Intestinal rehabilitation Varices Hepatopulmonary syndrome Portopulmonary syndrome Hepatorenal syndrome Hepatic encephalopathy Ascites Coagulopathy Nutrition PELD MELD 


  1. American Gastroenterology Association (2003) American Gastroenterological Association medical position statement: short bowel syndrome and intestinal transplantation. Gastroenterology 124(4):1105–1110CrossRefGoogle Scholar
  2. Arnon R, Kerkar N, Davis MK et al (2010) Liver transplantation in children with metabolic diseases: the studies of pediatric liver transplantation experience. Pediatr Transplant 14(6):796–806CrossRefPubMedGoogle Scholar
  3. Boluda ER (2015) Pediatric small bowel transplantation. Curr Opin Organ Transplant 20(5):550–556CrossRefPubMedGoogle Scholar
  4. Bucuvalas JC, Horn JA, Chernausek SD (1997) Resistance to growth hormone in children with chronic liver disease. Pediatr Transplant 1(1):73–79PubMedGoogle Scholar
  5. Cole CR, Hansen NI, Higgins RD et al (2008) Very low birth weight preterm infants with surgical short bowel syndrome: incidence, morbidity and mortality, and growth outcomes at 18 to 22 months. Pediatrics 122(3):e573–e582CrossRefPubMedPubMedCentralGoogle Scholar
  6. Freeman RB Jr, Wiesner RH, Roberts JP et al (2004) Improving liver allocation: MELD and PELD. Am J Transplant Suppl 9:114–131CrossRefGoogle Scholar
  7. Garcia-Tsao G, Sanyal AJ, Grace ND et al (2007) Prevention and management of gastroesophageal varices and variceal hemorrhage in cirrhosis. Hepatology 46(3):922–938CrossRefPubMedGoogle Scholar
  8. Graham DY, Smith JL (1981) The course of patients after variceal hemorrhage. Gastroenterology 80(4):800–809PubMedGoogle Scholar
  9. Grant D, Abu-Elmagd K, Mazariegos G et al (2015) Intestinal transplant registry report: global activity and trends. Am J Transplant 15(1):210–219CrossRefPubMedGoogle Scholar
  10. Gutierrez IM, Kang KH, Jaksic T (2011) Neonatal short bowel syndrome. Semin Fetal Neonatal Med 16(3):157–163CrossRefPubMedGoogle Scholar
  11. Hartley JL, Davenport M, Kelly DA (2009) Biliary atresia. Lancet 374(9702):1704–1713CrossRefPubMedGoogle Scholar
  12. Javid PJ, Malone FR, Reyes J et al (2010) The experience of a regional pediatric intestinal failure program: successful outcomes from intestinal rehabilitation. Am J Surg 199(5):676–679CrossRefPubMedGoogle Scholar
  13. Kamath BM, Schwarz KB, Hadzic N (2010) Alagille syndrome and liver transplantation. J Pediatr Gastroenterol Nutr 50(1):11–15CrossRefPubMedGoogle Scholar
  14. Kobelska-Dubiel N, Klincewicz B, Cichy W (2014) Liver disease in cystic fibrosis. Prz Gastroenterol 9(3):136–141PubMedPubMedCentralGoogle Scholar
  15. Kulkarni S, Perez C, Pichardo C et al (2015) Use of Pediatric Health Information System database to study the trends in the incidence, management, etiology, and outcomes due to pediatric acute liver failure in the United States from 2008–2013. Pediatr Transplant 19(8):888–895CrossRefPubMedGoogle Scholar
  16. Malone FR, Horslen SP (2007) Medical and surgical management of the pediatric patient with intestinal failure. Curr Treat Options Gastroenterol 10(5):379–390CrossRefPubMedGoogle Scholar
  17. McDiarmid SV, Millis MJ, Olthoff KM et al (1998) Indications for pediatric liver transplantation. Pediatr Transplant 2(2):106–116PubMedGoogle Scholar
  18. McDiarmid SV, Anand R, Lindblad AS et al (2002) Development of a pediatric end-stage liver disease score to predict poor outcome in children awaiting liver transplantation. Transplantation 74(2):173–181CrossRefPubMedGoogle Scholar
  19. Moore SW, Davidson A, Hadley GP et al (2008) Malignant liver tumors in South African children: a national audit. World J Surg 32(7):1389–1395CrossRefPubMedGoogle Scholar
  20. Ng VL, Fecteau A, Shepherd R et al (2008) Outcomes of 5-year survivors of pediatric liver transplantation: report on 461 children from a North American multicenter registry. Pediatrics 122(6):e1128–e1135CrossRefPubMedGoogle Scholar
  21. Otte JB, Pritchard J, Aronson DC et al (2004) Liver transplantation for hepatoblastoma: results from the International Society of Pediatric Oncology (SIOP) study of SIOPEL-1 and review of the world experience. Pediatr Blood Cancer 42(1):74–83CrossRefPubMedGoogle Scholar
  22. Protheroe SM (1998) Feeding the child with chronic liver disease. Nutrition 14(10):796–800CrossRefPubMedGoogle Scholar
  23. Shneider BL, Brown MB, Haber B et al (2006) A multicenter study of the outcome of biliary atresia in the United States, 1997–2000. J Pediatr 148(4):467–474CrossRefPubMedGoogle Scholar
  24. Smith JM, Skeans MA, Horslen SP et al (2017) OPTN/SRTR 2015 Annual Data Report: intestine. Am J Transplant 17(Suppl 1):252–285CrossRefPubMedGoogle Scholar
  25. Sokol RJ, Shepherd RW, Superina R et al (2007) Screening and outcomes in biliary atresia: summary of a National Institutes of Health workshop. Hepatology 46(2):566–581CrossRefPubMedPubMedCentralGoogle Scholar
  26. Squires RH, Shneider BL, Bucuvalas J et al (2006) Acute liver failiure in children: the first 348 patients in the pediatric acute liver failure study group. J Pediatr 148(5):652–658CrossRefPubMedPubMedCentralGoogle Scholar
  27. Squires RH, Ng V, al RR (2014) Evaluation of the pediatric patient for liver transplantation: 2014 practice guideline by the American Association for the Study of Liver Diseases, American Society of Transplantation and the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition. J Pediatr Gastroenterol Nutr 59(1):112–131CrossRefPubMedGoogle Scholar
  28. Starzl TE, Rowe MI, Todo S et al (1989) Transplantation of multiple abdominal viscera. JAMA 261(10):1449–1457CrossRefPubMedPubMedCentralGoogle Scholar
  29. Wieman RA, Balistreri WF (2007) Nutritioinal support in children with liver disease. In: Baker RD, Baker SS, Davis AM (eds) Pediatric nutritioni support. Jones and Bartlett Publishers, Sudbury, pp 459–476Google Scholar
  30. Zargar SA, Javid G, Khan BA et al (2002) Endoscopic ligation compared with sclerotherapy for bleeding esophageal varices in children with extrahepatic portal venous obstruction. Hepatology 36(3):666–672CrossRefPubMedGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Division of Pediatric GastroenterologyNemours Alfred I. duPont Hospital for ChildrenWilmingtonUSA
  2. 2.Division of Pediatric GastroenterologyMayo ClinicRochesterUSA

Section editors and affiliations

  • J Jeffrey Malatack
    • 1
  1. 1.Diagnostic Referral DivisionNemours/Alfred I. duPont Hospital for ChildrenWilmingtonUSA

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