杨宁介入医学网

Porto sinusoidal vascular liver disease (PSVD) is a vascular liver disease characterized by portal hypertension in the absence of cirrhosis and other causes of liver disease[1].

门窦性血管性肝病(PSVD)是一种以血管性肝病为特征的门静脉高压，没有肝硬化和其他原因的肝病 [1] 。
 

 Contrary to the Eastern World and Developing Countries, in the Western World PSVD is considered infrequent.

与东方世界和发展中国家相反，在西方世界，PSVD被认为是不常见的。
 

 However, a low grade of suspicion can contribute, at least partially, to the low frequency of PSVD in Western Countries.

然而，至少部分低程度的怀疑（或者说对此病不敏感）导致西方国家PSVD的低频率。

 

 Indeed, in the last years, the deepening of the knowledge on PSVD and its clinical presentation led to the recognition of an increasing number of cases and to the awareness that, in the past, some patients affected by PSVD were misdiagnosed and classified as affected by cryptogenic cirrhosis[2].

事实上，在过去的几年里，PSVD及其临床表现的加深导致越来越多的病例被发现，在过去，一些PSVD患者被误诊和归类为隐源性肝硬化[2]。
 

Moreover, it has been recently shown that a number of disorders and drugs (Table ​(Table1)1) are associated with PSVD[1,2], and this observation suggests that an active search of signs of portal hypertension in patients affected by those diseases could lead to the identification of PSVD patients.

此外，最近有研究表明，许多疾病和药物与PSVD相关，这一观察表明，积极搜索这些疾病患者门脉高压的迹象可能导致PSVD患者的识别。

Diseases associated to porto sinusoidal vascular liver disease

分类	疾病
Thrombophilia	Myeloproliferative neoplasm Protein S or C deficiency Antiphospholipid antibodies Lupus anticoagulant Factor V Leiden Prothrombin mutation
Hematologic disease	Myeloproliferative neoplasm (polycythemia vera, chronic myelogenous leukaemia, essential thrombocythemia) Myeloid metaplasia Lymphoproliferative conditions (Hodgkin’s disease, non-Hodgkin’s lymphoma, chronic lymphocytic leukaemia and multiple myeloma) Spherocytosis
Genetic disorders	Cystic fibrosis Adams Oliver syndrome Turner‘s disease
Autoimmune disease	Rheumatoid arthritis Systemic lupus erythematosus Systemic sclerosis Scleroderma
Gut diseases	Celiac disease Inflammatory bowel disease
Drug and toxics	Oxaliplatin Azathioprine 6-thioguanine Arsenic Busulfan Cytosine arabinoside Cyclophosphamide Bleomycin Chlorambucil Doxyrubicin Carmustine
Acquired and congenital immunodeficiency	Human immunodeficiency virus Primary antibody-deficiency syndrome

The term PSVD replaced the term INCPH in order to include the patients with specific histological features but no clinical signs portal hypertension.

术语PSVD取代了术语INCPH，以包括具有特定组织学特征但没有临床体征的门静脉高压的患者。