在患有 lysinuric 蛋白不耐受症的患者中,移植的结果
Tomi Rautanen1, Kaisa Ahopelto1, Harri Niinikoski2
1Transplantation and Liver Surgery, Helsinki University Hospital and University of Helsinki, Helsinki, Finland.
Clinical kidney journal
|January 13, 2025
概括
对于素蛋白不耐受 (LPI) 患者来说,脏移植是可行的,尽管急性排斥率高和潜在的并发症. 移植后的结果显示,移植和患者存活率中等.
科学领域:
- 腎臟病學 (nephrology) 是一種醫學專業.
- 代谢障碍 代谢障碍 代谢障碍
- 移植免疫学 移植免疫学
背景情况:
- lysinuric蛋白不耐受 (LPI) 是一种代谢障碍,影响氨基酸吸收和清除.
- 慢性脏病常常导致慢性脏病,其中很大一部分进展到末期脏病.
- 关于LPI患者移植结果的数据有限.
研究的目的:
- 分析芬兰患有 lysinuric 蛋白不耐受症的移植患者的结局.
- 评估LPI个体移植后的移植和患者存活率.
- 评估LPI患者移植后血氨基酸概况的变化.
主要方法:
- 所有接受脏移植的芬兰LPI患者 (2005-2023) 的回顾性队列研究.
- 数据来源于芬兰移植登记处和电子医疗记录.
- 在移植前和移植后分析的血氨基酸概况.
主要成果:
- 8名LPI患者接受了脏移植,其中2人接受了第二次移植.
- 四次移植 (40%) 经历了急性排斥 (T细胞或抗体介导).
- 一名患者死于膜蛋白质病;1,5年和10年的移植存活率分别为80%,68.6%和51.4%. 患者的生存率分别为88%,86%和50%.
结论:
- 移植是LPI患者可行的治疗选择.
- 报告指出,急性排斥率高,以及诸如膜蛋白质炎之类的严重并发症.
- 移植会改变氨酸和氨酸的血度.
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