肝内窦后性非肝硬化性门静脉高压症的研究进展

陈慧婷; 周永健

doi:10.3969/j.issn.1001-5256.2022.07.004

肝内窦后性非肝硬化性门静脉高压症的研究进展

DOI: 10.3969/j.issn.1001-5256.2022.07.004

陈慧婷^{1, 2, 3},
周永健^{1, 2, 3, , ,}

1.
广州市第一人民医院消化内科，广州 510180
2.
广州消化疾病中心，广州 510180
3.
华南理工大学附属第二医院消化内科，广州 510180

基金项目:

国家自然科学基金 (81970507);

国家自然科学基金 (82170585);

广东省自然科学基金 (2020A1515010100);

广东省自然科学基金 (2021A1515011290);

广州市科技计划项目 (201904010132);

广州市科技计划项目 (202102010023);

广东省医学科研基金 (A2020411);

广州市卫健委-高新技术项目 (2019GX05);

广州市医学重点学科 (2021-2023)

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：陈慧婷负责收集及分析资料、撰写论文；周永健负责拟定写作思路，指导撰写文章并最后定稿。

详细信息

通信作者:
周永健，eyzhouyongjian@scut.edu.cn

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出版历程
- 收稿日期: 2022-04-20
- 录用日期: 2022-05-22
- 出版日期: 2022-07-20

Research advances in post-sinusoidal non-cirrhotic portal hypertension

Huiting CHEN^{1, 2, 3},
Yongjian ZHOU^{1, 2, 3
, ,
,}

1.
Department of Gastroenterology, Guangzhou First People's Hospital, Guangzhou 510180, China
2.
Guangzhou Digestive Disease Center, Guangzhou 510180, China
3.
Department of Gastroenterology, The Second Affiliated Hospital of South China University of Technology, Guangzhou 510180, China

Research funding:

National Natural Science Foundation of China (81970507);

National Natural Science Foundation of China (82170585);

Natural Science Foundation of Guangdong Province (2020A1515010100);

Natural Science Foundation of Guangdong Province (2021A1515011290);

Technology Planning Project of Guangzhou City (201904010132);

Technology Planning Project of Guangzhou City (202102010023);

Guangdong Medical Science and Research Foundation (A2020411);

Guangzhou High Technology Project (2019GX05);

Project of Key Medical Discipline in Guangzhou (2021-2023)

More Information

Corresponding author: ZHOU Yongjian, eyzhouyongjian@scut.edu.cn(ORCID: 0000-0002-4036-4592)

摘要

摘要: 门静脉高压定义为门静脉系统压力病理性增高，其最常见的原因是肝硬化，但也见于无肝硬化时，称为非肝硬化性门静脉高压。根据累及的部位，非肝硬化性门静脉高压相关疾病可分为肝前、肝内或肝后三类；肝内病因所致可进一步细分为窦前性、窦性和窦后性。本文将总结肝后及窦后性门静脉高压的特点，并详细讨论布加综合征和肝窦阻塞综合征不同分类，简述了其临床表现、发病机制、诊断和治疗等方面的研究进展。
- 非肝硬化性门静脉高压 /
- Budd-Chiari综合征 /
- 肝静脉闭塞性疾病
Abstract: Portal hypertension (PH) is defined as the pathological increase in pressure in the portal venous system. The most common cause of PH is liver cirrhosis, but it is also observed in patients without liver cirrhosis, which is called non-cirrhotic portal hypertension. Non-cirrhotic portal hypertension can be classified in prehepatic, intrahepatic, and posthepatic types depending on the site of involvement, and the intrahepatic type can be further divided into presinusoidal, sinusoidal, and post-sinusoidal types. This article summarizes the features of posthepatic and post-sinusoidal portal hypertension, discusses in detail the different classifications of Budd-Chiari syndrome and hepatic sinusoidal obstruction syndrome, and briefly introduces the research advances in the clinical manifestations, pathogenesis, diagnosis, and treatment of these diseases.
- Noncirrhotic Portal Hypertension /
- Budd-Chiari Syndrome /
- Hepatic Veno-Occlusive Disease

HTML全文

表 1 HSCT-SOS严重程度分级

Table 1. New EBMT criteria for severity grading of a suspected HSCT-SOS in adult patients

表现	轻度	中度	重度	危重
出现第一个SOS临床症状的时间	＜7 d	5~7 d	≤4 d	任何时间
TBil(mg/dL)	2≤TBil＜3	3≤TBil＜5	5≤TBil＜8	≥8
胆红素上升的速度			48 h翻倍
转氨酶(正常值上限)	≤2	2＜转氨酶≤5	5＜转氨酶≤8	＞8
体质量增加	＜5%	5%≤体质量≤10%	5%≤体质量≤10%	＞10%
肾功能(移植前的基线值)	＜1.2	1.2≤肾功能＜1.5	1.5≤肾功能＜2	≥2

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表 2 HSCT-SOS临床诊断标准

Table 2. Diagnosis criteria of HSCT-SOS for adult patients

标准	描述
EBMT成人诊断标准	经典型SOS：造血干细胞移植后21 d内胆红素≥34.2 μmol/L，同时出现以下3项中的2项：痛性肝肿大，体质量增加≥5%，腹水
	迟发型SOS：造血干细胞移植后21 d后出现经典型SOS或经组织学证实的SOS，或≥2条经典型标准且同时具备超声或血液动力学证据证实SOS
改良Seatlle标准	造血干细胞移植后20 d内出现以下3项中的2项：胆红素≥34.2 μmol/L，肝肿大伴右上腹痛，液体潴留致体质量增加＞2%
Baltimore标准	造血干细胞移植后21 d内胆红素≥34.2 μmol/L，同时出现以下3项中的2项：痛性肝肿大，体质量增加≥5%，腹水

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