Outcomes of 5-Year Survivors of Pediatric Liver Transplantation: Report on 461 Children From a North American Multicenter Registry

Abstract

Children who are 5-year survivors of liver transplantation have good graft function, but chronic medical conditions and posttransplantation complications affect extrahepatic organs. A comprehensive approach to the management of these patients' multiple unique needs requires the expertise and commitment of health care providers both beyond and within transplant centers to further optimize long-term outcomes for pediatric liver transplant recipients.

Selection and work-up for liver transplantation

“…17 Ng et al reported second and third transplantation rates of 12% and 2%, respectively, 5% chronic rejection, and 6% post-transplant lymphoproliferative disease in their paediatric LT cohort. 18 Although tests of graft function were preserved in 90% of 5-year survivors in the cohort, one-third of children did not have complete normalisation of liver enzymes, suggesting ongoing graft inflammation. 18 Most long-term survivors of paediatric LT retain good graft function, but may have chronic medical conditions and post-transplantation complications.…”

“…18 Although tests of graft function were preserved in 90% of 5-year survivors in the cohort, one-third of children did not have complete normalisation of liver enzymes, suggesting ongoing graft inflammation. 18 Most long-term survivors of paediatric LT retain good graft function, but may have chronic medical conditions and post-transplantation complications. LT success is determined by more than graft survival rates; ongoing management requires the involvement and commitment of healthcare providers within and beyond transplantation teams.…”

Paediatric lver transplantation for children treated at public health facilities in South Africa: Time for change

“…Children with metabolic liver disease (such as α1-antitrypsin deficiency, tyrosinaemia and Wilson disease) and other cholestatic disorders (such as Alagille syndrome and progressive intrahepatic cholestatic syndrome) are likely to account for the remainder of the referrals for liver transplantation. Although acute liver failure accounts for about a tenth of paediatric liver transplantation performed in developed countries, [2] our experience at CHBAH suggests that the dire lack of paediatric intensive care facilities will severely restrict the number of children with acute liver failure who can be referred for transplantation.…”

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“…22 School performance assessment questionnaire The questionnaire which comprises 5 questions was developed by the researchers through a literature review to assess school performance. [11][12][13] The questions are as follows: class repetition (yes/no, if yes, which year), being held back (yes/no, if yes, how many years), adversely affected school life (yes/no), an increase in absence (yes/no), and the number of days absent per year.…”

“…In several studies, it has been indicated that recipients' school performance are adversely affected because of an increase in absenteeism, grade repetition, hyperactivity, and learning disorders after undergoing a liver transplant. [11][12][13] In studies conducted to investigate pediatric patients having undergone liver transplant in Turkey, the most common indications observed in these patients are biliary atresia, Wilson's disease, cryptogenic liver disease, and familial intrahepatic cholestasis. It also has been reported that most of these children (80%-85%) underwent a living-donor liver transplant, and the most common complications they had were bile complications (eg, biliary leak, biliary atresia), infection, portal vein thrombosis, and hepatic artery thrombosis.…”