丙酸血症患者肝移植后的存活率

IF 1.8 4区医学 Q3 GENETICS & HEREDITY

Molecular Genetics and Metabolism Reports Pub Date : 2024-05-27 DOI:10.1016/j.ymgmr.2024.101093

Tamás Zelei , Zoltán Vokó , Bertalan Németh , Zsuzsanna Petykó , Geetanjoli Banerjee , Vanja Sikirica

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引用次数: 0

摘要

丙酸血症（PA）是一种影响氨基酸代谢的罕见代谢性疾病。肝移植可改善某些预后，但对长期存活率的影响仍不清楚。一项系统性文献综述和生存分析显示，94 名接受移植手术的丙酸血症患者在 33 岁时的生存概率为 62%，而中位生存期估计为 40 年。这些研究结果突显出，尽管进行了肝移植，但与普通人群相比，PA 患者的存活率仍有很大差距。

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Survival of propionic acidemia patients with liver transplant

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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来源期刊

Molecular Genetics and Metabolism Reports Biochemistry, Genetics and Molecular Biology-Endocrinology

CiteScore

4.00

自引率

5.30%

发文量

105

审稿时长

33 days

期刊介绍： Molecular Genetics and Metabolism Reports is an open access journal that publishes molecular and metabolic reports describing investigations that use the tools of biochemistry and molecular biology for studies of normal and diseased states. In addition to original research articles, sequence reports, brief communication reports and letters to the editor are considered.