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Survival of propionic acidemia patients with liver transplant.
Zelei, Tamás; Vokó, Zoltán; Németh, Bertalan; Petykó, Zsuzsanna; Banerjee, Geetanjoli; Sikirica, Vanja.
Afiliação
  • Zelei T; Syreon Research Institute, Budapest, Hungary.
  • Vokó Z; Syreon Research Institute, Budapest, Hungary.
  • Németh B; Center for Health Technology Assessment, Semmelweis University, Budapest, Hungary.
  • Petykó Z; Syreon Research Institute, Budapest, Hungary.
  • Banerjee G; Syreon Research Institute, Budapest, Hungary.
  • Sikirica V; Center for Health Technology Assessment, Semmelweis University, Budapest, Hungary.
Mol Genet Metab Rep ; 40: 101093, 2024 Sep.
Article em En | MEDLINE | ID: mdl-38846517
ABSTRACT
Propionic acidemia (PA) is a rare metabolic disorder affecting amino acid metabolism. Liver transplantation improves some outcomes, but the impact on long-term survival remains unclear. A systematic literature review and survival analysis, identifying 94 PA patients who underwent transplantation, revealed a survival probability of 62% at age 33; while median survival was estimated at 40 years. These findings highlight a substantial survival deficit of PA patients compared to the general population despite liver transplantation.
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Texto completo: 1 Base de dados: MEDLINE Idioma: En Ano de publicação: 2024 Tipo de documento: Article

Texto completo: 1 Base de dados: MEDLINE Idioma: En Ano de publicação: 2024 Tipo de documento: Article