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门脉肝窦血管病的研究进展

何福亮 贾继东

引用本文:
Citation:

门脉肝窦血管病的研究进展

DOI: 10.12449/JCH240103
利益冲突声明:本文不存在任何利益冲突。
作者贡献声明:何福亮负责查阅文献,撰写论文;贾继东负责拟定文章思路,修改并最终定稿。
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    通信作者:

    何福亮, hefuliangjiayuan@163.com (ORCID: 0000-0003-1643-1465)

Research advances in porto-sinusoidal vascular disease

More Information
  • 摘要: 门脉肝窦血管病(PSVD)是近年提出的新的疾病命名,是特发性非硬化性门静脉高压的重要补充,纳入了病理符合但不合并门静脉高压症状、合并门静脉血栓及合并病毒性肝炎等患者。本文对门脉肝窦血管性疾病的命名、流行病学、病因学、临床表现、预后及治疗进行阐述,以期提高临床医生对该病的认识。

     

  • 表  1  PSVD的诊断条件

    Table  1.   Diagnostic conditions of PSVD

    肝活检病理无肝硬化,标本长度≥20 mm + 符合1条门静脉高压特征性表现或符合1条PSVD病理表现
    肝活检病理无肝硬化,标本长度≥20 mm + 符合1条门静脉高压非特征性表现和符合1条PSVD病理表现
    下载: 导出CSV

    表  2  PSVD的临床及病理特征

    Table  2.   The clinical and pathological features of PSVD

    项目 门静脉高压表现 PSVD病理表现1)
    特征性 ·胃、食管或异位静脉曲张 ·门静脉高压性出血 ·影像学发现门体侧支循环 ·门静脉闭塞(血管壁增厚、管腔闭塞、门静脉分支消失) ·结节再生性增生 ·不完全间隔纤维化(也称为不完全间隔硬化);该特征只能通过大体肝标本而非肝活检评估
    非特征性 ·腹水 ·PLT<150 000/mm3 ·脾脏最大长度≥13 cm ·门静脉支异常(动脉增殖、扩张,汇管区内与门静脉紧密相连的异常血管) ·血管结构紊乱:门静脉和中央静脉分布不规则 ·非带状肝窦扩张 ·轻度窦周纤维化
    注:1)需由1名病理学专家明确。
    下载: 导出CSV
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  • 收稿日期:  2023-12-14
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  • 出版日期:  2024-01-23
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