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波尔托鼻状血管疾病:分类和临床相关性

Madhumita Premkumar¹, Anil C Anand²

¹Department of Hepatology, Postgraduate Institute of Medical Education and Research, Chandigarh, 160012, India.

Journal of clinical and experimental hepatology

|April 11, 2024

概括

非肝硬化门高血压 (NCPH) 在没有肝硬化的情况下被诊断出来. 新的 porto-sinusoidal血管疾病 (PSVD) 标准提供了他的病理学诊断,但需要肝脏活检,促使人们对其临床实用性的讨论.

科学领域:

肝病学肝病学是一种肝病学.
血管性肝脏疾病血管性肝脏疾病

背景情况:

非肝硬化门外高血压 (NCPH) 呈现出门外高血压 (PHT) 的迹象,但没有肝硬化.
异常性NCPH (INCPH) 缺乏已知的风险因素或肝外门静脉血栓,病因不明.
波尔图鼻状血管疾病 (PSVD) 是一种拟议的综合征实体,对NCPH诊断具有特定的遗传病理学标准.

研究的目的:

审查INCPH/PSVD的诊断和治疗.
讨论新的PSVD分类与NCPH传统临床诊断的优缺点.
探索INCPH/PSVD的病理生物学和临床相关物,特别是在亚洲的背景下.

主要方法:

对NCPH和PSVD现有文献的审查.
对PSVD分类的诊断标准和影响的分析.
在亚洲人口中讨论临床相关性和病理生物学.

主要成果:

PSVD为NCPH提供了组织病理学标准 (消灭性门静脉病变,结节再生性增生,不完全隔膜纤维化).
PSVD定义扩大了诊断范围,包括没有PHT或门静脉血栓症 (PVT) 的患者.
PSVD的一个关键缺点是诊断的强制性侵入性肝活检,与亚洲患者之前的NCPH临床诊断形成鲜明对比.

结论:

像PSVD这样的标准化诊断标准对于比较临床队列非常有价值.
在全球队列中对PSVD的未来临床验证对于防止错误分类肝血管疾病至关重要.
亚临床PSVD的自然史及其向PHT的进展仍然未知.

关键词:

国家医疗健康中心 (NCPH)在PSVD中使用PSVD.肝外门静脉阻塞肝外门静脉阻塞门外高血压,非肝炎门外高血压门高血压门高血压携带状血管疾病的血管疾病.

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