定义 概论 病因 病理生理 流行病学临床表现 TIPS适应症 结果


时间:2021-09-18 13:36来源:www.ynjr.net 作者:杨宁介入医学网
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 evi
When to Suspect 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.
In both the cases, known causes of chronic liver disease or portal hypertension should be excluded.
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.
These alterations are not associated to signs of portal hypertension [5, 6].
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.
Some of these patients developed clinical signs of portal hypertension during the follow-up.
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.

 Another challenge for the diagnosis of PSVD is with patients with chronic PVT.
 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.
 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.
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.

血栓形成倾向 Thrombophilia
骨髓增生性肿瘤 Myeloproliferative neoplasm
蛋白质s或c缺乏症Protein S or C deficiency
抗磷脂抗体 Antiphospholipid antibodies
狼疮抗凝物;Lupus anticoagulant
第五凝血因子 Factor V Leiden
凝血酶原突变 Prothrombin mutation
血液病 Hematologic disease
骨髓增生性肿瘤 Myeloproliferative neoplasm
     真性红细胞增多症 polycythemia vera
     慢性粒细胞白血病 chronic myelogenous leukaemia,
     特发性血小板增多症 essential thrombocythemia)
骨髓外化生 Myeloid metaplasia
淋巴增生性疾病 Lymphoproliferative conditions (
       何杰金氏病 Hodgkin’s disease
       非何杰金氏病 non-Hodgkin’s lymphoma
       慢性淋巴细胞白血病 chronic lymphocytic leukaemia
       多发性骨髓瘤 multiple myeloma
球形红细胞增多症 Spherocytosis
遗传性疾病 Genetic disorders
囊性纤维化 Cystic fibrosis
肢头皮和颅骨缺失缺陷 Adams Oliver syndrome
Turner‘病 ’Turner‘s disease
自身免疫性疾病 Autoimmune disease
类风湿关节炎 Rheumatoid arthritis
系统性红斑狼疮 Systemic lupus erythematosus
系统性硬化症 Systemic sclerosis
硬皮病 Scleroderma
肠道疾病 Gut diseases
腹部疾病 Celiac disease
炎性肠病 Inflammatory bowel disease
药物和毒性 Drug and toxics
奥沙利铂 Oxaliplatin
咪唑嘌呤 Azathioprine
66-硫代鸟嘌呤 -6-thioguanine
三氧化二砷 Arsenic
甲磺酸丁二醇二酯 Busulfan
阿糖胞甙 Cytosine arabinoside
环磷酰胺 Cyclophosphamide
博来霉素 Bleomycin
苯丁酸氮芥 Chlorambucil
阿霉素 Doxyrubicin
卡氯芥 Carmustine
获得性和先天性 免疫缺陷性疾病 Acquired and congenital immunodeficiency
人类免疫缺陷病毒 Human immunodeficiency virus
原发性抗体缺陷综合症 Primary antibody-deficiency syndrome

 It has been reported that more than a half of PSVD patients have an associated disease .
As a practical consequence, in patients affected by these diseases or exposed to these drugs, the searching of signs of portal hypertension is suggested.
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.
 When one of these alterations is present, the patient should be referred to a hepatologist.

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