Predictive factors for survival in children receiving liver transplants for Wilson's disease: A Cohort Study Using European Liver Transplant Registry Data

Result code in IS VaVaI

<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00023001%3A_____%2F18%3A00077478" target="_blank" >RIV/00023001:_____/18:00077478 - isvavai.cz</a>

Result on the web

<a href="https://aasldpubs.onlinelibrary.wiley.com/doi/full/10.1002/lt.25308" target="_blank" >https://aasldpubs.onlinelibrary.wiley.com/doi/full/10.1002/lt.25308</a>

DOI - Digital Object Identifier

<a href="http://dx.doi.org/10.1002/lt.25308" target="_blank" >10.1002/lt.25308</a>

Result language

angličtina

Original language name

Predictive factors for survival in children receiving liver transplants for Wilson's disease: A Cohort Study Using European Liver Transplant Registry Data

Original language description

Liver transplantation (LT) is a rescue therapy for life-threatening complications of Wilson&apos;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 (&lt;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 &lt; 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.

Czech name

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Czech description

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Type

J_imp - Article in a specialist periodical, which is included in the Web of Science database

CEP classification

—

OECD FORD branch

30213 - Transplantation

Project

—

Continuities

N - Vyzkumna aktivita podporovana z neverejnych zdroju

Publication year

2018

Confidentiality

S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Name of the periodical

Liver transplantation

ISSN

1527-6465

e-ISSN

—

Volume of the periodical

24

Issue of the periodical within the volume

9

Country of publishing house

US - UNITED STATES

Number of pages

13

Pages from-to

1186-1198

UT code for WoS article

000452293300005

EID of the result in the Scopus database

2-s2.0-85053827594