特发性非肝硬化性门静脉高压症的研究进展

陈杰

特发性非肝硬化性门静脉高压症的研究进展

陈杰

天津医科大学宝坻临床学院消化科

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中图分类号: R575.2
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出版历程
- 出版日期: 2013-04-20

Idiopathic non-cirrhotic portal hypertension

Chen Jie

摘要

摘要:
特发性非肝硬化性门静脉高压是一种原因不明的门静脉高压症,无明显肝硬化特征;本病的发病机制尚不清楚,目前认为慢性感染、免疫、毒物接触、凝血机制障碍等均可能参与发病。临床主要表现为门静脉高压征象,如食管胃底静脉曲张或破裂出血、显著脾肿大,而肝功能基本正常,腹水及肝性脑病少见;病理改变主要表现为门静脉闭塞性病变,但无肝硬化改变。临床诊断主要是排他性诊断,有门静脉高压的临床证据,组织病理学检查除外肝硬化,排除引起肝硬化的慢性肝病以及引起非肝硬化性门静脉高压的其他临床疾病即可考虑本病。有关特发性非肝硬化性门静脉高压症研究较少,推荐按照肝硬化所致门静脉高压指南进行治疗。预后主要取决于门静脉高压的严重程度及其并发症的处理,一般优于肝硬化并食管胃底静脉曲张破裂出血。
- 特发性非肝硬化性门静脉高压 /
- 食管和胃静脉曲张 /
- 肝静脉闭塞性疾病
Abstract:
The pathogenesis of idiopathic non-cirrhotic portal hypertension (INCPH) remains unknown and the disease is diagnosed by the absence of recognized clinical indicators of cirrhosis and of any other known etiologies of portal hypertension.To promote understanding of this disease, a comprehensive overview of potential etiologies, clinical manifestations, histopathological features, methods of diagnosis and potential differential diagnoses, and outcome of clinical management is presented in this review.In particular, we discuss the findings from INCPH studies and their implications in regards to each of the above-mentioned categories.For example, associations with various comorbidities have suggested a possible immune system component to INCPH development and/or progression.In addition, the common clinical characteristics of patients upon presentation can not only help to recognize disease suspects but may also provide insights into the pathogenesis and prognosis.Finally, prognosis following the various intervention strategies appears to depend mainly on severity of the portal hypertension, as well as its various accompanying complications.
- idiopathic noncirrhotic portal hypertension /
- esophageal and gastric varices /
- hepatic veno-occlusive disease

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参考文献(21)

[1]Vispo E, Moreno A, Maida I, et al.Noncirrhotic portal hyper-tension in HIV-infected patients:unique clinical and patho-logical findings[J].AIDS, 2010, 24 (8) :1171-1176.

[2]Vernier-Massouille G, Cosnes J, Lemann M, et al.Nodularregenerative hyperplasia in patients with inflammatory boweldisease treated with azathioprine[J].Gut, 2007, 56 (10) :1404-1409.

[3]Guha Mazumder DN.Chronic arsenic toxicity&human health[J].Indian J Med Res, 2008, 128 (4) :436-447.

[4]Cazals-Hatem D, Hillaire S, Rudler M, et al.Obliterativeportal venopathy:portal hypertension is not always presentat diagnosis[J].J Hepatol, 2011, 54 (3) :455-461.

[5]Blendis LM, Banks DC, Ramboer C, et al.Spleen blood flowand splanchnic haemodynamics in blood dyscrasia and othersplenomegalies[J].Clin Sci, 1970, 38 (1) :73-84.

[6]Yamaguchi E, Yamanoi A, Ono T, et al.Experimental investiga-tion of the role of endothelin-1 in idiopathic portal hypertension[J].J Gastroenterol Hepatol, 2007, 22 (7) :1134-1140.

[7]Dabritz J, Worch J, Materna U, et al.Life-threatening hy-persplenism due to idiopathic portal hypertension in earlychildhood:case report and review of the literature[J].BMCGastroenterol, 2010, 10:122.

[8]Liu J, Zhang SG, Wei WM.et al.Clinical value of hand-as-sisted laparoscopic modified sugiura procedure in idiopathicportal hypertension[J].J Med Res, 2012, 41 (9) :139-142. (in Chinese) 刘佳, 张绍庚, 魏炜明, 等.手助腹腔镜改良sugiura术在特发性门静脉高压症的临床应用[J].医学研究杂志, 2012, 41 (9) :139-142.

[9]Sarin SK, Kumar A, Chawla YK, et al.Noncirrhotic portal fi-brosis/idiopathic portal hypertension:APASL recommenda-tions for diagnosis and treatment[J].Hepatol Int, 2007, 1 (3) :398-413.

[10]Chawla Y, Dhiman RK.Intrahepatic portal venopathy and re-lated disorders of the liver[J].Semin Liver Dis, 2008, 28 (3) :270-281.

[11]Dhiman RK, Chawla Y, Vasishta RK, et al.Non-cirrhoticportal fibrosis (idiopathic portal hypertension) :experiencewith 151 patients and a review of the literature[J].J Gastro-enterol Hepatol, 2002, 17 (1) :6-16.

[12]Sawada S, Sato Y, Aoyama H, et al.Pathological study ofidiopathic portal hypertension with an emphasis on cause ofdeath based on records of Annuals of Pathological AutopsyCases in Japan[J].J Gastroenterol Hepatol, 2007, 22 (2) :204-209.

[13]Ludwig J, Hashimoto E, Obata H, et al.Idiopathic portal hy-pertension[J].Hepatology, 1993, 17 (6) :1157-1162.

[14]International Working Party.Terminology of nodular hepato-cellular lesions[J].Hepatology, 1995, 22 (3) :983-993.

[15]Herlock S, Feldman CA, Moran B, et al.Partial nodular trans-formation of the liver with portal hypertension[J].Am J Med, 1966, 40 (2) :195-203.

[16]Sciot R, Staessen D, Van Damme B, et al.Incomplete sep-tal cirrhosis:histopathological aspects[J].Histopathology, 1988, 13 (6) :593-603.

[17]Schouten JN, Garcia-Pagan JC, Valla DC, et al.Idiopathicnoncirrhotic portal hypertension[J].Hepatology, 2011, 54 (3) :1071-1081.

[18]Gao R, Gao F, Hao JY.Clinical analysis of regional portal hyper-tension[J].Chin J Dig, 2011, 31 (1) :21-23. (in Chinese) 高茹, 高峰, 郝建宇.区域性门静脉高压症的临床分析[J].中华消化杂志, 2011, 31 (1) :21-23.

[19]Soga K, Tomikashi K, Miyawaki K, et al.Endoscopic injec-tion sclerotherapy with ethanolamine oleate with iopamidolfor esophagojejunal varices in idiopathic portal hypertension[J].Dig Dis Sci, 2009, 54 (7) :1592-1596.

[20]Romano M, Giojelli A, Capuano G, et al.Partial splenic em-bolization in patients with idiopathic portal hypertension[J].Eur J Radiol, 2004, 49 (3) :268-273.

[21]Krasinskas AM, Eghtesad B, Kamath PS, et al.Liver trans-plantation for severe intrahepatic noncirrhotic portal hyperten-sion[J].Liver Transpl, 2005, 11 (6) :627-634.

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