Porto-sinusoidal vascular disease: proposal and description of a novel entity
Porto-sinusoidal vascular disease: proposal and description of a novel entity
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DOI：
10.1016/s2468-1253(19)30047-0
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发表时间：
2019-05-01
影响因子：
35.7
通讯作者：
Alonso, Cristina
中科院分区：
文献类型：
--
作者：
de Gottardi, Andrea;Rautou, Pierre-Emmanuel;Alonso, Cristina
不存在门静脉血栓形成且无肝硬化，但伴有轻度或中度肝脏组织学改变（例如闭塞性静脉病、结节性再生性增生或不完全性间隔性肝硬化）的门静脉高压症越来越受到人们的重视。由于原因和组织学结果的异质性，大量术语被用来描述这种特发性非肝硬化门静脉高压症。存在具有相同临床和组织学特征的患者，但在诊断时没有门静脉高压。因此，需要改进标准来定义这种形式的肝病。在这里，我们提出术语门静脉窦血管疾病，因为发现的所有病变都涉及门静脉或肝窦。该实体的定义是基于不存在肝硬化的特征，伴或不伴门脉高压或组织学病变的体征。存在已知的肝病病因并不排除门窦血管疾病，但血管性肝病的具体病因被排除在其定义之外。门窦血管疾病的诊断基于肝活检，可能包括门静脉高压的特异性体征，肝脏硬度值正常或轻度升高，且无完全门静脉血栓形成。我们提供简单的诊断标准，因为就统一命名达成一致是未来合作研究的基本要求。
Portal hypertension in the absence of portal vein thrombosis and without cirrhosis, but with mild or moderate alterations of liver histology (eg, obliterative venopathy, nodular regenerative hyperplasia, or incomplete septal cirrhosis) is being increasingly recognised. Owing to the heterogeneity of causes and histological findings, a substantial number of terms have been used to describe such idiopathic non-cirrhotic portal hypertension. Patients with the same clinical and histological features exist, but without portal hypertension at the time of diagnosis. Therefore, improved criteria are needed to define this form of liver disease. Here, we propose the term porto-sinusoidal vascular disease, since all lesions found involve the portal venules or sinusoids. The definition of this entity is based on the characteristic absence of cirrhosis with or without signs of portal hypertension or histological lesions. The presence of known causes of liver disease does not rule out porto-sinusoidal vascular disease, but specific causes of vascular liver disease are excluded from its definition. The diagnosis of porto-sinusoidal vascular disease is based on liver biopsy and might include signs specific for portal hypertension with normal or mildly elevated liver stiffness values and no complete portal vein thrombosis. We provide simple diagnostic criteria, because agreement on a uniform nomenclature is an essential requirement for future collaborative studies.