丰坦相关性肝病:一篇综述
Gabriel Heering1, Nachum Lebovics1,2, Raksheeth Agarwal1,3
1From the Department of Medicine, Westchester Medical Center/New York Medical College, Valhalla, NY.
Cardiology in review
|March 13, 2024
概括
丰坦相关性肝病 (FALD) 是丰坦手术后单心室循环的成年人严重并发症. 目前对FALD的诊断和治疗策略面临重大挑战,需要进一步研究.
科学领域:
- 心脏病学 心脏病学
- 肝病学 肝病学是一种肝病学.
- 儿科手术 儿科手术
背景情况:
- 丰坦相关性肝病 (FALD) 是一种在患有先天性心脏病的丰坦手术后成年人的渐进性并发症.
- 由于FALD,不断增长的Fontan人口面临显著的发病率和死亡率,包括纤维化,肝硬化和肝细胞癌.
- FALD病理生理学涉及到单心室生理学独特的复杂的血液动力学和炎症因素.
研究的目的:
- 审查当前关于Fontan相关性肝病 (FALD) 的文献.
- 突出诊断和管理FALD的挑战,包括不可靠的非侵入性测试和不一致的活检结果.
- 讨论当前的治疗争议,并确定未来研究的知识差距.
主要方法:
- 关于FALD流行病学,病理生理学,诊断和管理的综合文献综述.
- 分析当前诊断模式的挑战,包括非侵入性标记物和侵入性采样.
- 检查末期FALD的治疗选择,包括移植策略.
主要成果:
- FALD呈现了一系列的肝脏异常,从纤维化到癌症,由于拥堵和斑点性纤维化,造成诊断困难.
- 对于肝硬性的非侵入性测试在FALD中不可靠,组织采样不一致.
- 肝脏和心脏移植是治疗选择,联合心脏肝脏移植在美国越来越受欢迎.
结论:
- 对于不断增长的成年Fontan人口来说,FALD是一个主要问题,需要改进诊断和监测协议.
- 目前用于FALD的诊断方法不足,需要开发验证的方法.
- 进一步的研究对于解决FALD管理方面的知识差距和优化患者治疗结果至关重要.
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