内科理论与实践 ›› 2026, Vol. 21 ›› Issue (02): 171-175.doi: 10.16138/j.1673-6087.2026.02.11
收稿日期:2026-02-27
修回日期:2026-03-04
接受日期:2026-04-14
出版日期:2026-04-25
发布日期:2026-06-15
通讯作者:
王 剑 E-mail:wangjian890113@126.com
作者简介:作者贡献/Authors’ Contributions张璐负责文献整理与综述初稿撰写;王剑负责综述设计、指导与修改;林勇负责内容审核。
ZHANG Lu, WANG Jian(
), LIN Yong
Received:2026-02-27
Revised:2026-03-04
Accepted:2026-04-14
Online:2026-04-25
Published:2026-06-15
摘要:
门静脉高压往往被认为是肝硬化的标志性伴随表现,以门静脉系统压力升高和侧支循环建立为特征。然而,非肝硬化性门静脉高压 (non-cirrhotic portal hypertension, NCPH)在临床中并不罕见。根据发病机制不同,NCPH可分为肝前性、肝性和肝后性。NCPH的临床特点和管理策略均有别于肝硬化门静脉高压。近年来,一系列研究发现,代谢相关脂肪性肝病可引起肝窦内皮功能障碍和肝内血管改变,在发生肝硬化之前即可导致门静脉压力显著升高,已成为NCPH的重要病因之一。本文总结了代谢相关NCPH的发病机制、临床特点、诊断与鉴别诊断、治疗与管理策略及未来研究方向等,旨在提高临床医师对该类疾病的认知水平和精准管理能力。
张璐, 王剑, 林勇. 代谢相关非肝硬化性门静脉高压:从初步认识到精准管理[J]. 内科理论与实践, 2026, 21(02): 171-175.
ZHANG Lu, WANG Jian, LIN Yong. Metabolic-associated non-cirrhotic portal hypertension: from preliminary recognition to precision management[J]. Journal of Internal Medicine Concepts & Practice, 2026, 21(02): 171-175.
| [1] |
Simonetto DA, Liu M, Kamath PS. Portal hypertension and related complications: diagnosis and management[J]. Mayo Clin Proc,2019,94(4):714-726.
doi: 10.1016/j.mayocp.2018.12.020 |
| [2] | 何福亮, 马琳, 李悦榕, 等. 非肝硬化性门脉高压的临床诊断[J]. 实用肝脏病杂志,2022,25(1):1-4. |
| He FL, Ma L, Li YR, et al. Non-cirrhotic portal hypertension: the highlight of its diagnosis[J]. J Prac Hepatol,2022,25(1):1-4. | |
| [3] | 张博静, 韩国宏, 樊代明. 非肝硬化性门静脉高压症的研究现状[J]. 临床肝胆病杂志,2016,32(2):245-249. |
| Zhang BJ, Han GH, Fan DM. Research advances in non-cirrhotic portal hypertension[J]. J Clin Hepatol,2016,32(2):245-249. | |
| [4] |
De Gottardi A, Rautou PE, Schouten J, et al. Porto-sinusoidal vascular disease: proposal and description of a novel entity[J]. Lancet Gastroenterol Hepatol,2019,4(5):399-411.
doi: 10.1016/S2468-1253(19)30047-0 |
| [5] |
Shukla A, Rockey DC, Kamath PS, et al. Non-cirrhotic portal fibrosis/idiopathic portal hypertension: APASL recommendations for diagnosis and management[J]. Hepatol Int,2024,18(6):1684-1711.
doi: 10.1007/s12072-024-10739-6 |
| [6] |
Ryou M, Stylopoulos N, Baffy G. Nonalcoholic fatty liver disease and portal hypertension[J]. Explor Med,2020,1:149-169.
doi: 10.37349/emed.2020.00011 |
| [7] | Hirayama AB, Taliberti IB, Oliveira CPMS, et al. Lipotoxicity plays a key role in the development of angiogenesis and microcirculatory modulation in MASLD spectrum[J]. Arq Gastroenterol,2025,62:e25053. |
| [8] |
Seifalian AM, Chidambaram V, Rolles K, et al. In vivo demonstration of impaired microcirculation in steatotic human liver grafts[J]. Liver Transpl Surg,1998,4(1):71-77.
doi: 10.1002/lt.500040110 |
| [9] |
Seifalian AM, Piasecki C, Agarwal A, et al. The effect of graded steatosis on flow in the hepatic parenchymal microcirculation[J]. Transplantation,1999,68(6):780-784.
doi: 10.1097/00007890-199909270-00009 |
| [10] |
McCuskey RS, Ito Y, Robertson GR, et al. Hepatic microvascular dysfunction during evolution of dietary steatohepatitis in mice[J]. Hepatology,2004,40(2):386-393.
doi: 10.1002/hep.20302 |
| [11] |
Wang XK, Peng ZG. Targeting liver sinusoidal endothelial cells: an attractive therapeutic strategy to control inflammation in nonalcoholic fatty liver disease[J]. Front Pharmacol,2021,12:655557.
doi: 10.3389/fphar.2021.655557 |
| [12] |
Minciuna I, Taru MG, Procopet B, et al. The interplay between liver sinusoidal endothelial cells, platelets, and neutrophil extracellular traps in the development and progression of metabolic dysfunction-associated steatotic liver disease[J]. J Clin Med,2024,13(5):1406.
doi: 10.3390/jcm13051406 |
| [13] | Baffy G, Bosch J. Overlooked subclinical portal hypertension in non-cirrhotic NAFLD:is it real and how to measure it?[J]. J Hepatol,2022,76(2):458-463. |
| [14] |
Hilscher MB, Sehrawat T, Arab JP, et al. Mechanical stretch increases expression of cxcl1 in liver sinusoidal endothelial cells to recruit neutrophils, generate sinusoidal microthrombi, and promote portal hypertension[J]. Gastroenterology,2019,157(1):193-209.
doi: 10.1053/j.gastro.2019.03.013 |
| [15] |
Sun X, Harris EN. New aspects of hepatic endothelial cells in physiology and nonalcoholic fatty liver disease[J]. Am J Physiol Cell Physiol,2020,318(6):C1200-C1213.
doi: 10.1152/ajpcell.00062.2020 |
| [16] |
Zadorozhna M, Di Gioia S, Conese M, et al. Neovascularization is a key feature of liver fibrosis progression: anti-angiogenesis as an innovative way of liver fibrosis treatment[J]. Mol Biol Rep,2020,47(3):2279-2288.
doi: 10.1007/s11033-020-05290-0 |
| [17] |
Ucar F, Sezer S, Erdogan S, et al. The relationship between oxidative stress and nonalcoholic fatty liver disease: its effects on the development of nonalcoholic steatohepatitis[J]. Redox Rep,2013,18(4):127-133.
doi: 10.1179/1351000213Y.0000000050 |
| [18] | Li Y, Yang P, Ye J, et al. Updated mechanisms of MASLD pathogenesis [J/OL]. Lipids Health Dis, 2024, 23(1):117[2024-04-22]. https://link.springer.com/article/10.1186/s12944-024-02108-x. |
| [19] |
Iwakiri Y, Trebicka J. Portal hypertension in cirrhosis: pathophysiological mechanisms and therapy[J]. JHEP Rep,2021,3(4):100316.
doi: 10.1016/j.jhepr.2021.100316 |
| [20] |
Zhao F, Ding J, Cui J, et al. Platelet activation is upregulated in cirrhotic patients with portal vein thrombosis[J]. Thromb Haemost,2024,124(8):739-752.
doi: 10.1055/a-2190-8705 |
| [21] |
Baffy G. Potential mechanisms linking gut microbiota and portal hypertension[J]. Liver Int,2019,39(4):598-609.
doi: 10.1111/liv.13986 |
| [22] |
Maruyama H, Kobayashi K, Kiyono S, et al. Interrelationship between insulin resistance and portal haemodynamic abnormality in cirrhosis[J]. Int J Med Sci,2017,14(3):240-245.
doi: 10.7150/ijms.17738 |
| [23] |
European Association for the Study of the Liver (EASL); European Association for the Study of Diabetes (EASD); European Association for the Study of Obesity (EASO). EASL-EASD-EASO clinical practice guidelines on the management of metabolic dysfunction-associated steatotic liver disease (MASLD)[J]. J Hepatol,2024,81(3):492-542.
doi: 10.1016/j.jhep.2024.04.031 |
| [24] |
Bao J, Zhao Y, Xu X, et al. Advances in the use of metformin for liver disease[J]. Curr Med Chem,2025,32(18):3591-3605.
doi: 10.2174/0109298673274268231215110330 |
| [25] |
Jang H, Kim Y, Lee DH, et al. Outcomes of various classes of oral antidiabetic drugs on nonalcoholic fatty liver disease[J]. JAMA Intern Med,2024,184(4):375-383.
doi: 10.1001/jamainternmed.2023.8029 |
| [26] |
Nevola R, Epifani R, Imbriani S, et al. GLP-1 receptor agonists in non-alcoholic fatty liver disease: current evidence and future perspectives[J]. Int J Mol Sci,2023,24(2):1703.
doi: 10.3390/ijms24021703 |
| [27] | Brusilovskaya K, Königshofer P, Schwabl P, et al. Vascular targets for the treatment of portal hypertension[J]. Semin Liver Dis,2019,39(4):483-501. |
| [28] |
Gracia-Sancho J, Maeso-Díaz R, Fernández-Iglesias A, et al. New cellular and molecular targets for the treatment of portal hypertension[J]. Hepatol Int,2015,9(2):183-191.
doi: 10.1007/s12072-015-9613-5 |
| [29] |
Schwabl P, Hambruch E, Seeland BA, et al. The FXR agonist PX20606 ameliorates portal hypertension by targeting vascular remodelling and sinusoidal dysfunction[J]. J Hepatol,2017,66(4):724-733.
doi: 10.1016/j.jhep.2016.12.005 |
| [30] |
Fernandez M, Vizzutti F, Garcia-Pagan JC, et al. Anti-VEGF receptor-2 monoclonal antibody prevents portal-systemic collateral vessel formation in portal hypertensive mice[J]. Gastroenterology,2004,126(3):886-894.
doi: 10.1053/j.gastro.2003.12.012 |
| [31] |
Shepherd AI, Wilkerson DP, Fulford J, et al. Effect of nitrate supplementation on hepatic blood flow and glucose homeostasis: a double-blind, placebo-controlled, randomized control trial[J]. Am J Physiol Gastrointest Liver Physiol,2016,311(3):G356-364.
doi: 10.1152/ajpgi.00203.2016 |
| [32] |
Owen NE, Williams TL, Maguire JJ, et al. Microarray analysis demonstrates up-regulation of the endothelin-1 gene with compensatory down-regulation of the ETA receptor gene in human portal vein[J]. Biosci Rep,2024,44(7):BSR20240528.
doi: 10.1042/BSR20240528 |
| [33] |
Deibert P, Schumacher YO, Ruecker G, et al. Effect of vardenafil, an inhibitor of phosphodiesterase-5, on portal haemodynamics in normal and cirrhotic liver — results of a pilot study[J]. Aliment Pharmacol Ther,2006,23(1):121-128.
doi: 10.1111/j.1365-2036.2006.02735.x |
| [34] |
孙成林, 王冰琼, 孙亚朦, 等. 肝硬化门静脉高压的无创诊断及预后评估新进展[J]. 肝脏,2025,30(6):756-758.
doi: 10.3969/j.issn.1008-1704.2025.06.003 |
|
Sun CL, Wang BQ, Sun YM, et al. Recent advances in non-invasive diagnosis and prognosis assessment of cirrhotic portal hypertension[J]. Chinese Hepatology,2025,30(6):756-758.
doi: 10.3969/j.issn.1008-1704.2025.06.003 |
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