When to Suspect PSVD?
何时怀疑PSVD？
There are two clinical scenarios in whom PSVD can be suspected: patients with chronic and unexplained alteration of liver enzymes without portal hypertension and patients with unexplained clinically evident portal hypertension.
有两种临床情况可怀疑PSVD：无门静脉高压患者慢性和不明原因的肝酶改变和不明原因的临床明显门静脉高压患者。
In both the cases, known causes of chronic liver disease or portal hypertension should be excluded.
在这两种情况下，都应排除慢性肝病或已知门静脉高压的原因。
The alterations of liver tests are various, and in particular they are represented by a mild elevation of ALT and AST, by an elevation of alkaline phosphatase 2 times upper the normal value or by an elevation of gamma-GT.
肝脏（功能）试验的改变是多种多样的，特别是ALT和AST的轻微升高、碱性磷酸酶比正常值高2倍或γ-GT的升高。
These alterations are not associated to signs of portal hypertension [5, 6].
这些改变与门脉高压症的体征无关。
Two recent European series showed that the histological lesions usually observed in patients affected by PSVD with portal hypertension (i.e.obliterative portal venopathy, OPV) were present in the 19% [7] and 25% [5] of the liver biopsies of patients with chronic elevation liver enzymes without cirrhosis and portal hypertension.
最近的两个欧洲系列研究显示，组织学病变通常在PSVD合并门脉高压症(i.e.在无肝硬化和肝门静脉高压症的慢性升高肝酶患者肝活检的19%和25%中存在闭塞性门静脉病，OPV)。
Some of these patients developed clinical signs of portal hypertension during the follow-up.
其中一些患者在随访中出现了门脉高压的临床体征。
These observations suggest first of all that PSVD should be suspected and actively searched among the patients with chronic liver test abnormalities of unknown aetiology and no signs of portal hypertension and that, such conditions, PSVD with and without portal hypertension may be different stages of the same disease where histological PSVD might represent an “early” pre-symptomatic stage of PSVD with portal hypertension.
这些观察结果表明，首先PSVD应该怀疑和积极搜索病因不明慢性肝脏（功能）检测异常的或许没有门脉高压迹象的患者，这样的条件下，PSVD有和没有门脉高压可能是不同阶段的疾病，组织学PSVD可能代表一个“早期”症状前阶段的PSVD门脉高压。
 Another challenge for the diagnosis of PSVD is with patients with chronic PVT.
诊断PSVD的另一个挑战是慢性PVT患者。
In fact, with new insights in the natural history of PSVD and its physiopathology, it is known that PSVD is frequently complicated by extrahepatic portal vein thrombosis [6, 8••], and, hypothetically, if a certain patient is investigated after PVT occurs, it may be impossible to determine if a pre-existent PSVD is the cause of the vascular disease.
事实上，伴随PSVD的自然史及其病理生理新的见解，已经知道PSVD经常伴有肝外门静脉血栓形成，假设，如果某一病人发现PVT发生后， 去确定是否先前PSVD是血管疾病的原因是不可能的。
In these cases, the only way to investigate PSVD might be performing a liver biopsy.
在这些病例中，调查PSVD的唯一方法可能是进行肝活检。
Hence, the presence of a pre-existing, undiagnosed PSVD should be suspected in patients with acute or chronic PVT, and that is why, the criteria of the patency of the portal vein has been eliminated in the last definition of PSVD.
因此，在急性或慢性PVT患者中应怀疑预先存在PSVD、未确诊的PSVD，这就是为什么在PSVD的最后一个定义中已经消除了门静脉通畅的标准。
Moreover, PSVD is frequently associated with several systemic conditions and with the chronic exposition to various drugs and toxins (Table (Table3)3) that may play a direct role in the pathophysiology of the liver alterations.
此外，PSVD经常与几种系统性疾病以及各种药物和毒素的慢性暴露有关，这可能在肝脏改变的病理生理学中发挥直接作用。
It has been reported that more than a half of PSVD patients have an associated disease .
据报道，超过一半的PSVD患者患有相关疾病。
As a practical consequence, in patients affected by these diseases or exposed to these drugs, the searching of signs of portal hypertension is suggested.
实际上，对于受这些疾病影响或接触过这些药物的患者，建议寻找门脉高压的征象。
In particular, physicians should be aware of the possibility to develop PSVD, and they should be careful about the presence of liver tests alteration, of indirect signs of portal hypertension such as thrombocytopenia and of splenomegaly or portal vein dilatation, when an imaging technique is performed.
特别是，医生应该意识到发生PSVD的可能性，他们应该在进行成像技术时，注意是否存在肝功能的改变、门静脉高压的间接征象，如血小板减少、脾肿大或门静脉扩张。
When one of these alterations is present, the patient should be referred to a hepatologist.
当其中这些改变之一存在时，患者应转给肝病医生。
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