肝内窦前性非肝硬化性门静脉高压症的研究进展

夏帅; 吴晓宁; 王冰琼; 何志颖; 尤红

doi:10.3969/j.issn.1001-5256.2022.07.003

肝内窦前性非肝硬化性门静脉高压症的研究进展

DOI: 10.3969/j.issn.1001-5256.2022.07.003

夏帅^{1, 2},
吴晓宁^{1, 2},
王冰琼^{1, 2},
何志颖^{1, 2},
尤红^{1, 2, , ,}

1.
首都医科大学附属北京友谊医院肝病中心，北京 100050
2.
国家消化系统疾病临床医学研究中心，北京 100050

利益冲突声明：所有作者均声明不存在利益冲突。

作者贡献声明：夏帅、王冰琼负责课题设计，撰写论文；王冰琼、何志颖参与收集数据和修改论文；尤红、吴晓宁负责拟定写作思路，指导撰写文章并最后定稿。

详细信息

通信作者:
尤红，youhongliver@ccmu.edu.cn

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出版历程
- 收稿日期: 2022-05-04
- 录用日期: 2022-06-05
- 出版日期: 2022-07-20

Research advances in non-cirrhotic intrahepatic presinusoidal portal hypertension

Shuai XIA^{1, 2},
Xiaoning WU^{1, 2},
Bingqiong WANG^{1, 2},
Zhiying HE^{1, 2},
Hong YOU^{1, 2
, ,
,}

1.
Liver Research Center, Beijing Friendship Hospital, Capital Medical University, Beijing 100050, China
2.
National Clinical Research Center for Digestive Diseases, Beijing 100050, China

More Information

Corresponding author: YOU Hong, youhongliver@ccmu.edu.cn(ORCID: 0000-0001-9409-1158)

摘要

摘要: 肝内窦前性非肝硬化性门静脉高压是非肝硬化性门静脉高压的一种类型，患者出现门静脉高压相关症状而无肝硬化病理证据，肝静脉压力梯度正常或轻微异常。因其病因复杂，极易漏诊、误诊，因此需要结合影像学与组织学检查明确诊断，适时启动降门静脉压治疗，预防性抗凝治疗的效果有待验证。
- 非肝硬化性门静脉高压 /
- 肝静脉闭塞性疾病 /
- 诊断
Abstract: Non-cirrhotic intrahepatic presinusoidal portal hypertension is a subtype of non-cirrhotic portal hypertension, and patients with this disease have the symptoms of portal hypertension and do not have the pathological evidence for liver cirrhosis, with normal or slightly abnormal hepatic venous pressure gradient. This disease is easily missed or misdiagnosed due to its complex etiology; therefore, radiological examination and histological examination should be combined in clinical practice to make a confirmed diagnosis, and related therapies should be initiated at the right time to reduce portal venous pressure. Further studies are needed to verify the efficacy of prophylactic anticoagulant therapy.
- Noncirrhotic Portal Hypertension /
- Hepatic Veno-Occlusive Disease /
- Diagnosis

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表 1 美国肝病学会推荐的INCPH和PSVD的相关疾病^[6]

Table 1. Diseases related to INCPH and PSVD recommended by AASLD^[6]

诱因类型	相关疾病
血液疾病瘤凝血异常	再生障碍性贫血、骨髓增生性疾病、霍奇金淋巴瘤、多发性骨髓蛋白质C或S缺乏、莱顿因子V基因突变、抗磷脂抗体综合征、ADAMTS13缺乏症
免疫/炎症性疾病	常见变异型免疫缺陷、自身免疫性肝炎、系统性红斑狼疮、硬皮病、类风湿性关节炎、HIV感染、乳糜泻、反复胃肠道感染
药物诱导	地达诺新、硫唑嘌呤、硫鸟嘌呤、奥沙利铂
遗传性疾病	遗传性出血性毛细血管扩张症伴肝血管畸形、特纳综合征、Adams-Oliver综合征、端粒末端转移酶逆转录酶突变、囊性纤维化、家族性未知基因突变病例
注：PSVD，门静脉肝窦血管性疾病。

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表 2 肝门窦性血管性疾病的诊断标准

Table 2. The diagnostic criteria of portal-sinusoidal vascular disease

诊断	门静脉高压征象	组织学病理改变提示PSVD
特征性诊断	胃、食管或异位静脉曲张门脉高压性出血影像学提示的门静脉系统侧支循环建立	闭塞性门静脉病(血管壁增厚、管腔闭塞、门静脉消失)-结节性再生增生-不完全性间隔纤维化(也称不完全性间隔肝硬化，后一种仅能在肝移植和肝穿刺病理可见)
非特征性诊断	腹水血小板计数＜150/mm³ 脾脏长轴≥13 cm	门静脉异常(增多、动脉扩张、门静脉周围血管通道、异常血管) 结构紊乱：门静脉和中央静脉不规则分布非带状分布肝窦扩张轻度窦周纤维化

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表 3 根据胆道系统累及部位分类的纤维囊性肝脏疾病

Table 3. The classification of liver fibrocystic diseases based on involved site

疾病	疾病累及的胆管	胆管的直径
胆管错构瘤	小叶内胆管	＜20 μm
CHF	小叶间胆管	20~50 μm
Caroli’s病	肝内大胆管	＞50 μm
Caroli’s综合征	小叶间到肝内大胆管	＞20 μm
先天性胆总管囊肿	肝外胆管	2~8 mm

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