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

Author:

Baumann Ulrich123456,Karam Vincent45,Adam René45,Fondevila Constantino47,Dhawan Anil8,Sokal Etienne9,Jacquemin Emmanuel10,Kelly Deirdre A.23,Grabhorn Enke611,Pawlowska Joanna612,D’Antiga Lorenzo613,Jara Vega Paloma614,Debray Dominique615,Polak Wojciech G.516,de Ville de Goyet Jean17,Verkade Henkjan J.518,

Affiliation:

1. aHannover Medical School, Divisions of Paediatric Gastroenterology and Hepatology, Department for Paediatric Kidney, Liver, and Metabolic Diseases, Hannover, Germany

2. bInstitute of Immunology and Immunotherapy, University of Birmingham, Birmingham, United Kingdom

3. cLiver Unit, Birmingham Women’s and Children’s Hospital, Birmingham, United Kingdom

4. dEuropean Liver Transplant Registry, AP-HP Hôpital Paul Brousse, Research Unit “Chronotherapy, cancers and transplantation,” University Paris-Saclay, Villejuif, France

5. eEuropean Liver and Intestine Transplant Association, Padova, Italy

6. fEuropean Reference Network TransplantChild, La Paz University Hospital, Madrid, Spain

7. gDepartment of General and Digestive Surgery, Hospital Universitario La Paz, IDIPAZ, CIBERehd, Madrid, Spain

8. hKing’s College Hospital, London, United Kingdom

9. iCliniques Universitaires Saint Luc, Catholic University of Louvain, Brussels, Belgium

10. jPediatric Hepatology and Liver Transplantation Unit, National Reference Centre for Biliary Atresia and Genetic Cholestasis, FILFOIE, ERN RARE LIVER, Bicêtre Hospital, Assistance Publique: Hôpitaux de Paris, University Paris-Saclay, Le Kremlin-Bicêtre; Inserm U1193, Hepatinov, University Paris-Saclay, Orsay, France

11. kChildreńs Hospital, University Medical Center Hamburg-Eppendorf, Hamburg, Germany

12. lDepartment of Gastroenterology, Hepatology, Nutritional Disorders and Pediatrics, The Children’s Memorial Health Institute, Warsaw, Poland

13. mPaediatric Hepatology, Gastroenterology, and Transplantation, Hospital Papa Giovanni XXIII, Bergamo, Italy

14. nPaediatric Hepatology Service, Coordinator ERN TransplantChild, Hospital Infantil Universitario La Paz, Madrid, Spain

15. oPediatric Liver Unit and Reference Center for Biliary Atresia and Genetic Cholestasis, APHP-Hôpital Necker, Université de Paris, Paris, France

16. pDepartment of Surgery, Division of Hepatopancreatobiliary and Transplant Surgery, Erasmus MC Transplant Institute, University Medical Center Rotterdam, Rotterdam, the Netherlands

17. qDepartment for the Treatment and Study of Pediatric Abdominal Diseases and Abdominal Transplantation, ISMETT, Palermo, Italy

18. rDept. of Pediatrics, Beatrix Children’s Hospital/University Medical Center Groningen, University of Groningen, ERN RareLiver, Groningen, The Netherlands

Abstract

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.

Publisher

American Academy of Pediatrics (AAP)

Subject

Pediatrics, Perinatology and Child Health

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