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Evaluation of living donors for hereditary liver disease (siblings, heterozygotes)

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JOURNAL OF HEPATOLOGY
卷 78, 期 6, 页码 1147-1156

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ELSEVIER
DOI: 10.1016/j.jhep.2022.10.013

关键词

Hereditary liver disease; Indication; Metabolic liver disease; Pediatric liver transplantation; Liver transplantation; Living donor liver transplantation

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Living donor liver transplantation (LDLT) is an alternative treatment approach that can reduce waiting list mortality and increase the pool of donors. It has been increasingly used for familial hereditary liver diseases. There are certain indications and contraindications for living donors in paediatric parental LDLT. While heterozygous donors have shown no mortality or morbidity related to the recurrence of metabolic diseases, there are exceptions for specific cases and risks associated with donor human leukocyte antigen homozygosity. Genetic and enzymatic assays should be considered in the parental donor selection criteria in certain circumstances.
Living donor liver transplantation (LDLT) is recognised as an alternative treatment modality to reduce waiting list mortality and expand the donor pool. Over recent decades, there have been an increasing number of reports on the use of LT and specifically LDLT for familial hereditary liver diseases. There are marginal indications and contraindications that should be considered for a living donor in paediatric parental LDLT. No mortality or morbidity related to recurrence of metabolic diseases has been observed with heterozygous donors, except for certain relevant cases, such as ornithine transcarbamylase deficiency, protein C deficiency, hypercholesterolemia, protoporphyria, and Alagille syndrome, while donor human leukocyte antigen homozygosity also poses a risk. It is not always essential to perform preoperative genetic assays for possible heterozygous carriers; however, genetic and enzymatic assays must hereafter be included in the parental donor selection criteria in the aforementioned circumstances.(c) 2022 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved.

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