Manti Sara, Marseglia Lucia, D'Angelo Gabriella, Filippelli Martina, Cuppari Caterina, Gitto Eloisa, Romano Claudio, Arrigo Teresa, Salpietro Carmelo
Department of Pediatric Sciences, Genetics and Pediatric Immunology Unit, University of Messina, Messina, Italy.
Hepat Mon. 2014 Jun 7;14(6):e18625. doi: 10.5812/hepatmon.18625. eCollection 2014 Jun.
Portal Hypertension (PH) is a progressive complication due to chronic liver disease. In addition to pathophysiologic changes in the micro-circulation, in PH are established fibrous tissue (periportal fibrous septal) and regenerative hyperplastic nodules (from micro- to macro-nodules) promoting hepatic architectural distortion.
A literature search of electronic databases was undertaken for the major studies published from 1981 to today. The databases searched were: PubMed, EMBASE, Orphanet, Midline and Cochrane Library. We used the keywords: "portal hypertension, children, immune system, endocrine system, liver fibrosis".
It is believed that PH results from three "phenotype": ischemia-reperfusion, involving nervous system (NS); edema and oxidative damage, involving immune system; inflammation and angiogenesis, involving endocrine system. However, its exact cause still underdiagnosed and unknown.
PH is a dynamic and potentially reversible process. Researchers have tried to demonstrate mechanisms underlying PH and its related-complications. This review focuses on the current knowledge regarding the pathogenesis, and immune, endocrine-metabolic factors of disease. The strong positive association between immune system and development of PH could be efficient to identify non-invasive markers of disease, to modify prognosis of PH, and to development and application of specific and individual anti-inflammatory therapy.
门静脉高压（PH）是慢性肝病导致的一种进行性并发症。除了微循环的病理生理变化外，PH还会形成促进肝脏结构扭曲的纤维组织（门静脉周围纤维间隔）和再生性增生结节（从微结节到巨结节）。
对1981年至今发表的主要研究进行了电子数据库文献检索。检索的数据库有：PubMed、EMBASE、Orphanet、Midline和Cochrane图书馆。我们使用的关键词为：“门静脉高压、儿童、免疫系统、内分泌系统、肝纤维化”。
人们认为PH由三种“表型”引起：缺血-再灌注，涉及神经系统（NS）；水肿和氧化损伤，涉及免疫系统；炎症和血管生成，涉及内分泌系统。然而，其确切病因仍诊断不足且未知。
PH是一个动态且可能可逆的过程。研究人员试图阐明PH及其相关并发症的潜在机制。本综述重点关注有关该疾病发病机制以及免疫、内分泌代谢因素的当前知识。免疫系统与PH发展之间的强正相关关系可能有助于识别疾病的非侵入性标志物、改善PH的预后以及开发和应用特定的个体化抗炎治疗。
