儿童门静脉肝窦血管性疾病的诊疗进展

摘要/Abstract

摘要： 门静脉肝窦血管性疾病(Porto-sinusoidal vascular disease,PSVD)是一组各种原因导致肝窦和窦前性门脉血管改变而发生的肝病,根据有无门静脉高压(portal hypertension,PH)征象,分为PH-PSVD和noPH-PSVD两种亚型,后者表现为慢性轻度转氨酶升高为特征。儿童PSVD无明显性别差异,各年龄段均有发病,诊断往往延误。超声诊断在PSVD中的异常发现率高,闭塞性门静脉病(OPV)是小儿PSVD的标志性病理改变,在PH-PSVD中更易见。noPH-PSVD预后良好,PH-PSVD往往进展发生不良事件,5年和20年无肝移植生存率约为90.0%和67.1%。本病目前尚无有效治疗手段,可考虑去除诱发因素、抗凝、原发病的治疗和门静脉高压并发症的治疗。

关键词: 门静脉肝窦血管性疾病, 儿童, 病因, 治疗

吕欣桐, 陆怡. 儿童门静脉肝窦血管性疾病的诊疗进展[J]. 肝脏, 2024, 29(12): 1457-1460.

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