门脉肝窦血管病的研究进展

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。
- 门静脉肝窦血管病 /
- 预后 /
- 治疗学
Abstract: Porto-sinusoidal vascular disease （PSVD） is a new disease nomenclature proposed in recent years， which is an important supplement to idiopathic non-cirrhotic portal hypertension. PSVD includes the patients with specific pathological conditions， but without portal hypertension symptoms， and the patients with portal vein thrombosis or viral hepatitis. This article elaborates on the naming， epidemiology， etiology， clinical manifestations， prognosis， and treatment of PSVD， in order to improve the understanding of this disease among clinicians.
- Porto-Sinusoidal Vascular Disease /
- Prognosis /
- Therapeutics

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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
或
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

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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。

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