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Liver Transpl. 2018 Sep;24(9):1186-1198. doi: 10.1002/lt.25308.

Predictive Factors for Survival in Children Receiving Liver Transplants for Wilson's Disease: A Cohort Study Using European Liver Transplant Registry Data.

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Division of Pediatric Gastroenterology and Hepatology, Departments of Pediatric Kidney, Liver, and Metabolic Diseases, Hannover, Germany.
Research Group Epidemiological and Statistical Methods, Helmholtz Center for Infection Research, Braunschweig, Germany.
German Center for Infection Research, Hannover-Braunschweig, Germany.
European Liver Transplant Registry.
AP-HP Hôpital Paul Brousse, Université Paris-Sud, Center Hépato-Biliaire, INSERM U 935, Villejuif, France.
Erasmus University Rotterdam, Rotterdam, the Netherlands.
Liver Unit, Elizabeth Hospital Birmingham, University Hospitals Birmingham National Health Service Foundation Trust, Birmingham, United Kingdom.
Institute of Liver Studies, King's College Hospital, London, United Kingdom.
General, Visceral and Transplantation Surgery, Hannover Medical School, Hannover, Germany.
Cliniques Universitaires Saint-Luc, Brussels, Belgium.
Department of Pediatric and Transplant Surgery, Children's Memorial Health Institute, Warsaw, Poland.
Department of Gastroenterology, Bozyaka Training and Research Hospital, Izmir, Turkey.
Transplant Center, Institute for Clinical and Experimental Medicine, Prague, Czech Republic.
HPB Surgery and Liver Transplantation Unit, Department of General Surgery, School of Medicine, Dokuz Eylul University, Inciralti, İzmir, Turkey.
Department of Surgery, Charite, Berlin, Germany.
Department of General and Transplant Surgery, University Hospital Essen, Essen, Germany.
Department of General Surgery and Liver Transplantation, Fundeni Clinical Institute, Bucharest, Romania.
Department of Visceral, Transplant and Thoracic Surgery, Center of Operative Medicine, Innsbruck Medical University, Innsbruck, Austria.
Liver Transplant Unit, Edouard Herriot Hospital, Lyon, France.
Department of Hepatobiliary Surgery and Visceral Transplantation, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
Department of Hepatology and Liver Transplant Unit, AP-HP Henri Mondor Hospital, Paris Est University, Créteil, France.


Liver transplantation (LT) is a rescue therapy for life-threatening complications of Wilson's disease (WD). However, data on the outcome of WD patients after LT are scarce. The aim of our study was to analyze a large pediatric WD cohort with the aim of investigating the longterm outcome of pediatric WD patients after LT and to identify predictive factors for patient and transplant survival. This is a retrospective cohort study using data of all children (<18 years) transplanted for WD enrolled in the European Liver Transplant Registry from January 1968 until December 2013. In total, 338 patients (57.6% female) transplanted at 80 different European centers (1-26 patients per center) were included in this study. The median age at transplantation was 14.0 years (interquartile range [IQR], 11.2-16.1 years); patients were followed up for a median of 5.4 years (IQR, 1.0-10.9 years) after LT. Overall patient survival rates were high with 87% (1-year survival), 84% (5-year survival), and 81% (10-year survival); survival rates increased considerably with the calendar year (P < 0.001). Early age at LT, living donation, and histidine tryptophan ketoglutarate preservation liquid were identified as risk factors for poor patient survival in the multivariate analysis. LT is an excellent treatment option for pediatric patients with WD and associated end-stage liver disease. Longterm outcome in these patients is similar to other pediatric causes for LT. Overall patient and graft survival rates improved considerably over the last decades. To improve future research in the field, the vast variability of allocation strategies should be harmonized and a generally accepted definition or discrimination of acute versus chronic WD needs to be found.

[Indexed for MEDLINE]

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