Living related versus deceased donor liver transplantation for maple syrup urine disease
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- 作者：Flavia Feiera ; b ; Ida Vanessa D. Schwartzc ; d ; Abigail R. Benkerte ; Joao Seda Netof ; Irene Miuraf ; Paulo Chapchapf ; Eduardo Antunes da Fonsecaf ; Sandra Vieirag ; h ; Maria Lú ; cia Zanotellih ; Filippo Pinto e Vairoc ; Jose Simon Camelo Jri ; Ana Vitoria Barban Marguttii ; George V. Mazariegosj ; Erik G. Puffenbergere ; k ; Kevin A. Strausse ; k ; l ; kstrauss@clinicforspecialchildren.org" class="auth_mail" title="E-mail the corresponding author
- 关键词：Maple syrup urine disease ; Branched chain ketoacid dehydrogenase ; Living related donor liver transplantation ; Domino transplantation
- 刊名：Molecular Genetics and Metabolism
- 出版年：2016
- 出版时间：March 2016
- 年：2016
- 卷：117
- 期：3
- 页码：336-343
- 全文大小：783 K
文摘
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Transplantation of a liver segment from a mutation heterozygous living donor effectively controls classical maple syrup urine disease (MSUD).
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Living related versus deceased donor transplants for MSUD result in similar but not identical metabolic outcomes.
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Live donor liver transplantation for MSUD improves access to effective treatment in resource-limited clinical settings.
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Through the domino procedure, live donor transplant adds a new liver to the donor pool.