Prognosis of Children Undergoing Liver Transplantation: A 30-Year European Study

TY - JOUR

T1 - Prognosis of Children Undergoing Liver Transplantation: A 30-Year European Study

AU - Baumann, Ulrich

AU - Karam, Vincent

AU - Adam, René

AU - Fondevila, Constantino

AU - Dhawan, Anil

AU - Sokal, Etienne

AU - Jacquemin, Emmanuel

AU - Kelly, Deirdre A

AU - Grabhorn, Enke

AU - Pawlowska, Joanna

AU - D'Antiga, Lorenzo

AU - Jara Vega, Paloma

AU - Debray, Dominique

AU - Polak, Wojciech G

AU - de Ville de Goyet, Jean

AU - Verkade, Henkjan J

AU - European Liver and Intestine Transplant Association (ELITA) and all ELTR contributing centers

N1 - Copyright © 2022 by the American Academy of Pediatrics.

PY - 2022/10/1

Y1 - 2022/10/1

N2 - OBJECTIVES: The European Liver Transplant Registry has been collecting data on virtually all pediatric liver transplant (PLT) procedures in Europe since 1968. We analyzed patient outcome over time and identified parameters associated with long-term patient outcome.METHODS: Participating centers and European organ-sharing organizations provided retrospective data to the European Liver Transplant Registry. To identify trends, data were grouped into consecutive time spans: era A: before 2000, era B: 2000 to 2009, and the current era, era C: since 2010.RESULTS: From June 1968 until December 2017, 16 641 PLT were performed on 14 515 children by 133 centers. The children <7 years of age represented 58% in era A, and 66% in the current era (P <.01). The main indications for PLT were congenital biliary diseases (44%) and metabolic diseases (18%). Patient survival at 5 years is currently 86% overall and 97% in children who survive the first year after PLT. The survival rate has improved from 74% in era A to 83% in era B and 85% in era C (P <.0001). Low-volume centers (<5 PLT/year) represented 75% of centers but performed only 19% of PLT and were associated with a decreased survival rate. In the current era, however, survival rates has become irrespective of volume. Infection is the leading cause of death (4.1%), followed by primary nonfunction of the graft (1.4%).CONCLUSIONS: PLT has become a highly successful medical treatment that should be considered for all children with end-stage liver disease. The main challenge for further improving the prognosis remains the early postoperative period.

AB - OBJECTIVES: The European Liver Transplant Registry has been collecting data on virtually all pediatric liver transplant (PLT) procedures in Europe since 1968. We analyzed patient outcome over time and identified parameters associated with long-term patient outcome.METHODS: Participating centers and European organ-sharing organizations provided retrospective data to the European Liver Transplant Registry. To identify trends, data were grouped into consecutive time spans: era A: before 2000, era B: 2000 to 2009, and the current era, era C: since 2010.RESULTS: From June 1968 until December 2017, 16 641 PLT were performed on 14 515 children by 133 centers. The children <7 years of age represented 58% in era A, and 66% in the current era (P <.01). The main indications for PLT were congenital biliary diseases (44%) and metabolic diseases (18%). Patient survival at 5 years is currently 86% overall and 97% in children who survive the first year after PLT. The survival rate has improved from 74% in era A to 83% in era B and 85% in era C (P <.0001). Low-volume centers (<5 PLT/year) represented 75% of centers but performed only 19% of PLT and were associated with a decreased survival rate. In the current era, however, survival rates has become irrespective of volume. Infection is the leading cause of death (4.1%), followed by primary nonfunction of the graft (1.4%).CONCLUSIONS: PLT has become a highly successful medical treatment that should be considered for all children with end-stage liver disease. The main challenge for further improving the prognosis remains the early postoperative period.

KW - Child

KW - End Stage Liver Disease/mortality

KW - Humans

KW - Liver Transplantation

KW - Prognosis

KW - Retrospective Studies

KW - Survival Rate

KW - Survivorship

U2 - 10.1542/peds.2022-057424

DO - 10.1542/peds.2022-057424

M3 - SCORING: Journal article

C2 - 36111446

VL - 150

JO - PEDIATRICS

JF - PEDIATRICS

SN - 0031-4005

IS - 4

M1 - e2022057424

ER -