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Predictive Factors for Survival in Children Receiving Liver Transplants for Wilson's Disease: A Cohort Study Using European Liver Transplant Registry Data
ED. Pfister, A. Karch, R. Adam, WG. Polak, V. Karam, D. Mirza, J. O'Grady, J. Klempnauer, R. Reding, P. Kalicinski, A. Coker, P. Trunecka, I. Astarcioglu, E. Jacquemin, J. Pratschke, A. Paul, I. Popescu, S. Schneeberger, O. Boillot, L. Fischer,...
Jazyk angličtina Země Spojené státy americké
Typ dokumentu časopisecké články, multicentrická studie
PubMed
30021057
DOI
10.1002/lt.25308
Knihovny.cz E-zdroje
- MeSH
- časové faktory MeSH
- disparity zdravotní péče MeSH
- dítě MeSH
- hepatolentikulární degenerace diagnóza mortalita chirurgie MeSH
- konečné stadium selhání jater diagnóza mortalita chirurgie MeSH
- lidé MeSH
- mladiství MeSH
- příčina smrti MeSH
- registrace MeSH
- retrospektivní studie MeSH
- rizikové faktory MeSH
- transplantace jater * škodlivé účinky mortalita MeSH
- věkové faktory MeSH
- výsledek terapie MeSH
- Check Tag
- dítě MeSH
- lidé MeSH
- mladiství MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- multicentrická studie MeSH
- Geografické názvy
- Evropa MeSH
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.
AP HP Hôpital Paul Brousse Université Paris Sud Center Hépato Biliaire INSERM U 935 Villejuif France
Cliniques Universitaires Saint Luc Brussels Belgium
Department of Gastroenterology Bozyaka Training and Research Hospital Izmir Turkey
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 Pediatric and Transplant Surgery Children's Memorial Health Institute Warsaw Poland
Department of Surgery Charite Berlin Germany
Erasmus University Rotterdam Rotterdam the Netherlands
General Visceral and Transplantation Surgery Hannover Medical School Hannover Germany
Institute of Liver Studies King's College Hospital London United Kingdom
Liver Transplant Unit Edouard Herriot Hospital Lyon France
Transplant Center Institute for Clinical and Experimental Medicine Prague Czech Republic
Citace poskytuje Crossref.org
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