肝前性非肝硬化性门静脉高压症的诊疗进展
DOI: 10.3969/j.issn.1001-5256.2022.07.002
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:杨佳琪负责收集资料并撰写文章;尚玉龙负责文稿修订;韩英负责选题,并最终审阅定稿。
Research advances in the diagnosis and treatment of prehepatic noncirrhotic portal hypertension
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摘要: 肝前性非肝硬化性门静脉高压是一组由非肝硬化因素导致的门静脉高压性疾病,目前认为这组疾病的病因主要与JAK2突变、血液高凝状态有关。临床表现主要包括静脉曲张破裂出血、脾肿大等,患者肝功能正常或仅有轻微异常。对肝前性非肝硬化性门静脉高压疾病的诊断主要依靠其影像学特点,同时还需排除肝硬化门静脉高压。治疗方面主要采取抗凝治疗和内镜下治疗,患者预后一般较好。本文系统梳理了肝前性非肝硬化性门静脉高压的主要进展。
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关键词:
- 非肝硬化性门静脉高压 /
- 静脉血栓栓塞 /
- 诊断 /
- 治疗学
Abstract: Prehepatic noncirrhotic portal hypertension is a group of diseases caused by noncirrhotic factors, and currently it is believed that the etiology of these diseases is mainly associated with JAK2 mutation and blood hypercoagulability. Main clinical manifestations include variceal bleeding and splenomegaly, with normal or slightly abnormal liver function. The diagnosis of prehepatic noncirrhotic portal hypertension mainly depends on its imaging characteristics, and meanwhile cirrhotic portal hypertension should be excluded. Anticoagulant therapy and endoscopy are the major therapies for these diseases, and patients often have a good prognosis. This article systematically reviews the recent advances in prehepatic noncirrhotic portal hypertension.-
Key words:
- Noncirrhotic Portal Hypertension /
- Venous Thromboembolism /
- Diagnosis /
- Therapeutics
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[1] BOSCH J, IWAKIRI Y. The portal hypertension syndrome: etiology, classification, relevance, and animal models[J]. Hepatol Int, 2018, 12(Suppl 1): 1-10. DOI: 10.1007/s12072-017-9827-9. [2] de FRANCHIS R, Baveno VI Faculty. Expanding consensus in portal hypertension: Report of the Baveno VI Consensus Workshop: Stratifying risk and individualizing care for portal hypertension[J]. J Hepatol, 2015, 63(3): 743-752. DOI: 10.1016/j.jhep.2015.05.022. [3] FAGUNDES ED, FERREIRA AR, ROQUETE ML, et al. Clinical and laboratory predictors of esophageal varices in children and adolescents with portal hypertension syndrome[J]. J Pediatr Gastroenterol Nutr, 2008, 46(2): 178-183. DOI: 10.1097/MPG.0b013e318156ff07. [4] PRIMIGNANI M, BAROSI G, BERGAMASCHI G, et al. Role of the JAK2 mutation in the diagnosis of chronic myeloproliferative disorders in splanchnic vein thrombosis[J]. Hepatology, 2006, 44(6): 1528-1534. DOI: 10.1002/hep.21435. [5] SMALBERG JH, ARENDS LR, VALLA DC, et al. Myeloproliferative neoplasms in Budd-Chiari syndrome and portal vein thrombosis: a meta-analysis[J]. Blood, 2012, 120(25): 4921-4928. DOI: 10.1182/blood-2011-09-376517. [6] DENNINGER MH, CHAïT Y, CASADEVALL N, et al. Cause of portal or hepatic venous thrombosis in adults: the role of multiple concurrent factors[J]. Hepatology, 2000, 31(3): 587-591. DOI: 10.1002/hep.510310307. [7] PODDAR U, THAPA BR, RAO KL, et al. Etiological spectrum of esophageal varices due to portal hypertension in Indian children: is it different from the West?[J]. J Gastroenterol Hepatol, 2008, 23(9): 1354-1357. DOI: 10.1111/j.1440-1746.2007.05102.x. [8] FERRI PM, RODRIGUES FERREIRA A, FAGUNDES ED, et al. Evaluation of the presence of hereditary and acquired thrombophilias in Brazilian children and adolescents with diagnoses of portal vein thrombosis[J]. J Pediatr Gastroenterol Nutr, 2012, 55(5): 599-604. DOI: 10.1097/MPG.0b013e318261814d. [9] YADAV S, DUTTA AK, SARIN SK. Do umbilical vein catheterization and sepsis lead to portal vein thrombosis? A prospective, clinical, and sonographic evaluation[J]. J Pediatr Gastroenterol Nutr, 1993, 17(4): 392-396. DOI: 10.1097/00005176-199311000-00010. [10] KHANNA R, SARIN SK. Non-cirrhotic portal hypertension - diagnosis and management[J]. J Hepatol, 2014, 60(2): 421-441. DOI: 10.1016/j.jhep.2013.08.013. [11] EL-KARAKSY HM, EL-KOOFY N, MOHSEN N, et al. Extrahepatic portal vein obstruction in Egyptian children[J]. J Pediatr Gastroenterol Nutr, 2015, 60(1): 105-109. DOI: 10.1097/MPG.0000000000000548. [12] NORONHA FERREIRA C, SEIJO S, PLESSIER A, et al. Natural history and management of esophagogastric varices in chronic noncirrhotic, nontumoral portal vein thrombosis[J]. Hepatology, 2016, 63(5): 1640-1650. DOI: 10.1002/hep.28466. [13] RANGARI M, GUPTA R, JAIN M, et al. Hepatic dysfunction in patients with extrahepatic portal venous obstruction[J]. Liver Int, 2003, 23(6): 434-439. DOI: 10.1111/j.1478-3231.2003.00879.x. [14] SARIN SK, SHAHI HM, JAIN M, et al. The natural history of portal hypertensive gastropathy: influence of variceal eradication[J]. Am J Gastroenterol, 2000, 95(10): 2888-2893. DOI: 10.1111/j.1572-0241.2000.03200.x. [15] DUCHÉ M, DUCOT B, ACKERMANN O, et al. Portal hypertension in children: High-risk varices, primary prophylaxis and consequences of bleeding[J]. J Hepatol, 2017, 66(2): 320-327. DOI: 10.1016/j.jhep.2016.09.006. [16] KUMAR N, SRIVASTAVA A, KUMARI N, et al. Prevalence, nature, and predictors of colonic changes in children with extrahepatic portal vein obstruction[J]. Gastrointest Endosc, 2020, 91(4): 849-858. DOI: 10.1016/j.gie.2019.11.042. [17] MEHROTRA RN, BHATIA V, DABADGHAO P, et al. Extrahepatic portal vein obstruction in children: anthropometry, growth hormone, and insulin-like growth factor I[J]. J Pediatr Gastroenterol Nutr, 1997, 25(5): 520-523. DOI: 10.1097/00005176-199711000-00006. [18] SARIN SK, BANSAL A, SASAN S, et al. Portal-vein obstruction in children leads to growth retardation[J]. Hepatology, 1992, 15(2): 229-233. DOI: 10.1002/hep.1840150210. [19] XU HF, DING HG. Diagnosis and treatment of pre-hepatic (sinusoidal) non-cirrhotic portal hypertension and related difficulties[J]. J Clin Hepatol, 2019, 35(1): 13-17. DOI: 10.3969/j.issn.1001-5256.2019.01.002.徐航飞, 丁惠国. 肝(窦)前型非肝硬化门静脉高压症的诊断与治疗及其面临的困境[J]. 临床肝胆病杂志, 2019, 35(1): 13-17. DOI: 10.3969/j.issn.1001-5256.2019.01.002. [20] CHANDRA R, KAPOOR D, THARAKAN A, et al. Portal biliopathy[J]. J Gastroenterol Hepatol, 2001, 16(10): 1086-1092. DOI: 10.1046/j.1440-1746.2001.02562.x. [21] SHIN SM, KIM S, LEE JW, et al. Biliary abnormalities associated with portal biliopathy: evaluation on MR cholangiography[J]. AJR Am J Roentgenol, 2007, 188(4): W341-W347. DOI: 10.2214/AJR.05.1649. [22] SHARMA P, SHARMA BC, PURI V, et al. Natural history of minimal hepatic encephalopathy in patients with extrahepatic portal vein obstruction[J]. Am J Gastroenterol, 2009, 104(4): 885-890. DOI: 10.1038/ajg.2009.84. [23] YADAV SK, SRIVASTAVA A, SRIVASTAVA A, et al. Encephalopathy assessment in children with extra-hepatic portal vein obstruction with MR, psychometry and critical flicker frequency[J]. J Hepatol, 2010, 52(3): 348-354. DOI: 10.1016/j.jhep.2009.12.012. [24] SHNEIDER BL, de VILLE DE GOYET J, LEUNG DH, et al. Primary prophylaxis of variceal bleeding in children and the role of MesoRex Bypass: Summary of the Baveno VI Pediatric Satellite Symposium[J]. Hepatology, 2016, 63(4): 1368-1380. DOI: 10.1002/hep.28153. [25] SHARMA P, MISHRA SR, KUMAR M, et al. Liver and spleen stiffness in patients with extrahepatic portal vein obstruction[J]. Radiology, 2012, 263(3): 893-899. DOI: 10.1148/radiol.12111046. [26] SUPERINA R, BAMBINI DA, LOKAR J, et al. Correction of extrahepatic portal vein thrombosis by the mesenteric to left portal vein bypass[J]. Ann Surg, 2006, 243(4): 515-521. DOI: 10.1097/01.sla.0000205827.73706.97. [27] KNIGHT GM, CLARK J, BOIKE JR, et al. TIPS for adults without cirrhosis with chronic mesenteric venous thrombosis and EHPVO refractory to standard-of-care therapy[J]. Hepatology, 2021, 74(5): 2735-2744. DOI: 10.1002/hep.31915. [28] AGENO W, RIVA N, SCHULMAN S, et al. Long-term clinical outcomes of splanchnic vein thrombosis: results of an international registry[J]. JAMA Intern Med, 2015, 175(9): 1474-1480. DOI: 10.1001/jamainternmed.2015.3184. [29] BARBUI T, FINAZZI G, FALANGA A. Myeloproliferative neoplasms and thrombosis[J]. Blood, 2013, 122(13): 2176-2184. DOI: 10.1182/blood-2013-03-460154. [30] DENTALI F, SQUIZZATO A, BRIVIO L, et al. JAK2V617F mutation for the early diagnosis of Ph- myeloproliferative neoplasms in patients with venous thromboembolism: a meta-analysis[J]. Blood, 2009, 113(22): 5617-5623. DOI: 10.1182/blood-2008-12-196014. [31] PLESSIER A, DARWISH-MURAD S, HERNANDEZ-GUERRA M, et al. Acute portal vein thrombosis unrelated to cirrhosis: a prospective multicenter follow-up study[J]. Hepatology, 2010, 51(1): 210-218. DOI: 10.1002/hep.23259. [32] THATIPELLI MR, MCBANE RD, HODGE DO, et al. Survival and recurrence in patients with splanchnic vein thromboses[J]. Clin Gastroenterol Hepatol, 2010, 8(2): 200-205. DOI: 10.1016/j.cgh.2009.09.019. [33] AMITRANO L, GUARDASCIONE MA, SCAGLIONE M, et al. Prognostic factors in noncirrhotic patients with splanchnic vein thromboses[J]. Am J Gastroenterol, 2007, 102(11): 2464-2470. DOI: 10.1111/j.1572-0241.2007.01477.x. [34] STRINGER MD, HEATON ND, KARANI J, et al. Patterns of portal vein occlusion and their aetiological significance[J]. Br J Surg, 1994, 81(9): 1328-1331. DOI: 10.1002/bjs.1800810923. [35] BRADBURY MS, KAVANAGH PV, CHEN MY, et al. Noninvasive assessment of portomesenteric venous thrombosis: current concepts and imaging strategies[J]. J Comput Assist Tomogr, 2002, 26(3): 392-404. DOI: 10.1097/00004728-200205000-00014. [36] TURNES J, GARCÍA-PAGÁN JC, GONZÁLEZ M, et al. Portal hypertension-related complications after acute portal vein thrombosis: impact of early anticoagulation[J]. Clin Gastroenterol Hepatol, 2008, 6(12): 1412-1417. DOI: 10.1016/j.cgh.2008.07.031. [37] ELKRIEF L, CORCOS O, BRUNO O, et al. Type 2 diabetes mellitus as a risk factor for intestinal resection in patients with superior mesenteric vein thrombosis[J]. Liver Int, 2014, 34(9): 1314-1321. DOI: 10.1111/liv.12386. [38] SARIN SK, SOLLANO JD, CHAWLA YK, et al. Consensus on extra-hepatic portal vein obstruction[J]. Liver Int, 2006, 26(5): 512-519. DOI: 10.1111/j.1478-3231.2006.01269.x. [39] AGENO W, DENTALI F, SQUIZZATO A. How I treat splanchnic vein thrombosis[J]. Blood, 2014, 124(25): 3685-3691. DOI: 10.1182/blood-2014-07-551515.
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