肝脏移植在初级高氧化尿症:一个单一中心的10年经验
Zahra Beyzaei1, Alireza Shamsaeefar2, Kiana Ghatei1
1Transplant Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.
Pediatric transplantation
|May 21, 2025
概括
肝移植 (LT) 显著改善了原发性高氧化 (PHs) 患者的结局,这是一个罕见的代谢障碍. 移植后,患者的肝酶正常化,尿液中氧化酸盐水平降低,提高了生存率和生活质量.
科学领域:
- 代谢障碍 代谢障碍 代谢障碍
- 器官移植 器官移植
- 腎臟病學 (nephrology) 是一種醫學專業.
背景情况:
- 初级高氧化尿 (PHs) 是一种罕见的遗传代谢障碍,其特点是过度的氧化酸盐生产和沉积.
- 未经治疗的PH可以导致严重的并发症,包括末期病 (ESKD) 和死亡率.
- 肝移植 (LT) 是一种已确立的PH治疗方法,特别是在儿科病例中.
研究的目的:
- 为了评估肝移植 (LT) 在患有原发性高氧化尿症 (PHs) 的患者的长期结果.
- 分析移植后的结果,包括生存率和生化标志物,在伊朗的单中心队列中.
主要方法:
- 从2012年到2023年,对33名患有PH的肝移植接受者的回顾性分析.
- 对已故供体肝移植 (DDLT) 和活供体肝移植 (LDLT) 的评估.
- 使用卡普兰-梅尔分析评估生化数据,术后因素和生存率.
主要成果:
- 所有33名患者的肝酶在LT后正常化,尿液中氧酸盐水平显著降低.
- 5年患者存活率为85%,移植存活率为97%.
- 8名患者经历了急性排斥,5名患者慢性排斥;21名患者需要先前或同时进行移植.
结论:
- 肝移植 (LT) 对患有原发性高氧化尿症 (PHs) 的患者提供了显著的生存和生活质量益处.
- 该研究表明,在LT后,长期结果有利,氧酸盐水平降低,但移植可用性和移植损失等挑战仍然存在.
- 密切监测和多学科护理对于移植后的PH患者的管理至关重要.
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