多囊性肝病:基于证据的管理和手术决策中的关键差距
Evangelia Florou1, Andreas Prachalias2, Parthi Srinivasan1
1Institute of Liver Studies, Hepato-Pancreato-Biliary Surgery Department, King's College Hospital, Denmark Hill, London, SE5 9RS, United Kingdom.
多囊性肝病的管理缺乏统一的方法. 目前的治疗方法提供了适度的好处,手术和移植具有显著的风险,突出了综合护理策略的需要.
科学领域:
- 肝病学 肝病学是一种肝病学.
- 医学遗传学 医学遗传学
- 临床管理 临床管理
背景情况:
- 多囊性肝病 (PLD) 涉及逐渐的囊发育与不同的症状.
- 有相当数量的患者出现衰弱症状,但缺乏统一的治疗算法.
研究的目的:
- 审查目前管理PLD的证据.
- 确定阻碍PLD综合,基于证据的护理途径的差距.
主要方法:
- 对PLD的医疗,干预和手术管理策略的系统审查.
- 对治疗结果和局限性的分析.
主要成果:
- 索马托斯塔丁类似物 (SSAs) 在选择的PLD患者中提供适度的肝体积减少 (3-7%) 和症状改善.
- 其他药理选择显示的益处有限;干预和手术方法取决于表型.
- 部分肝切除术提供了显著的脱皮,但患病率很高;肝移植 (LT) 可以治疗晚期疾病,但会带来外科风险.
结论:
- 疗法间的异质终点阻碍了有意义的比较和整合.
- 以表型为驱动的框架,标准化指标和多中心研究对于最佳的PLD治疗顺序至关重要.
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