Vascular diseases of the liver. Clinical Guidelines from the Catalan Society of Digestology and the Spanish Association for the Study of the Liver

Gastroenterología y Hepatología (English Edition) - Tập 40 - Trang 538-580 - 2017
Marta Martín-Llahí1, Agustín Albillos2,3, Rafael Bañares3,4, Annalisa Berzigotti5, M. Ángeles García-Criado6, Joan Genescà3,7, Virginia Hernández-Gea3,8, Elba Llop-Herrera3,9, Helena Masnou-Ridaura3,10, José Mateo11, Carmen A. Navascués12, Ángela Puente13, Marta Romero-Gutiérrez14, Macarena Simón-Talero3,7, Luis Téllez2,3, Fanny Turon3,10, Cándido Villanueva3,15, Roberto Zarrabeitia16, Juan Carlos García-Pagán3,8
1Servicio de Aparato Digestivo, Hospital Moisès Broggi, Sant Joan Despí, Barcelona, Spain
2Servicio de Gastroenterología y Hepatología, Hospital Universitario Ramón y Cajal, IRYCIS, Universidad de Alcalá, Madrid, Spain
3Centro de Investigación Biomédica en Enfermedades Hepáticas y Digestivas, CIBERehd, Instituto de Salud Carlos III, Spain
4Servicio de Aparato Digestivo, Hospital General Universitario Gregorio Marañón, Facultad de Medicina, Universidad Complutense, Madrid, Spain
5Hepatology, UVCM, Inselspital, Berna, Switzerland
6Servicio de Radiodiagnóstico, Hospital Clínic, Barcelona, Spain
7Hepatología, Departamento de Medicina Interna, Hospital Universitario Vall d’Hebron, VHIR, Barcelona, Spain
8Laboratorio de Hemodinámica Hepática, Servicio de Hepatología, Hospital Clínic, IDIBAPS, Barcelona, Spain
9Servicio de Aparato Digestivo, Hospital Universitario Puerta de Hierro, Madrid, Spain
10Servicio de Aparato Digestivo, Hospital Universitari Germans Trias i Pujol, Badalona, Barcelona, Spain
11Unidad de Hemostasia y Servicio de Hematología, Hospital de Sant Pau, Barcelona, Spain
12Servicio de Aparato Digestivo, Hospital Universitario Central de Asturias, Oviedo, Spain
13Servicio de Digestivo, Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain
14Servicio de Aparato Digestivo, Complejo Hospitalario de Toledo, Toledo, Spain
15Servicio de Patología Digestiva, Hospital de Sant Pau, Barcelona, Spain
16Unidad HHT, Hospital Sierrallana, Torrelavega, Cantabria, Spain

Tài liệu tham khảo

DeLeve, 2009, Vascular disorders of the liver, Hepatology, 49, 1729, 10.1002/hep.22772 Valla, 2004, Hepatic venous outflow tract obstruction etiopathogenesis: Asia versus the West, J Gastroenterol Hepatol, 19, S204, 10.1111/j.1440-1746.2004.03642.x Rajani, 2009, Budd–Chiari syndrome in Sweden: epidemiology, clinical characteristics and survival – an 18-year experience, Liver Int, 29, 253, 10.1111/j.1478-3231.2008.01838.x Okuda, 1995, Epidemiological and clinical features of Budd–Chiari syndrome in Japan, J Hepatol, 22, 1, 10.1016/0168-8278(95)80252-5 Mahmoud, 1996, Clinical spectrum, investigations and treatment of Budd–Chiari syndrome, Q J Med, 89, 37, 10.1093/oxfordjournals.qjmed.a030135 Plessier, 2008, Budd–Chiari syndrome, Semin Liver Dis, 28, 259, 10.1055/s-0028-1085094 Murad, 2004, Determinants of survival and the effect of portosystemic shunting in patients with Budd–Chiari syndrome, Hepatology, 39, 500, 10.1002/hep.20064 Darwish Murad, 2009, Etiology, management, and outcome of the Budd–Chiari syndrome, Ann Intern Med, 151, 167, 10.7326/0003-4819-151-3-200908040-00004 Ferral, 2012, Budd–Chiari syndrome, Am. J. Roentgenol, 199, 737, 10.2214/AJR.12.9098 Fickert, 1996, Acute Budd–Chiari syndrome with fulminant hepatic failure in a pregnant woman with factor V Leiden mutation, Gastroenterology, 111, 1670, 10.1016/S0016-5085(96)70031-8 Hadengue, 1994, The changing scene of hepatic vein thrombosis: recognition of asymptomatic cases, Gastroenterology, 106, 1042, 10.1016/0016-5085(94)90765-X Menon, 2004, The Budd–Chiari syndrome, N Engl J Med, 350, 578, 10.1056/NEJMra020282 Mitchell, 1982, Budd–Chiari syndrome: etiology, diagnosis and management, Medicine (Baltimore), 61, 199, 10.1097/00005792-198207000-00001 Moucari, 2008, Hepatocellular carcinoma in Budd–Chiari syndrome: characteristics and risk factors, Gut, 57, 828, 10.1136/gut.2007.139477 Janssen, 2003, Budd–Chiari syndrome: a review by an expert panel, J Hepatol, 38, 364, 10.1016/S0168-8278(02)00434-8 Cazals-Hatem, 2003, Arterial and portal circulation and parenchymal changes in Budd–Chiari syndrome: a study in 17 explanted livers, Hepatology, 37, 510, 10.1053/jhep.2003.50076 Tang, 2001, The prognostic value of histology in the assessment of patients with Budd–Chiari syndrome, J Hepatol, 35, 338, 10.1016/S0168-8278(01)00131-3 Dilawari, 1994, Hepatic outflow obstruction (Budd–Chiari syndrome). Experience with 177 patients and a review of the literature, Medicine (Baltimore), 73, 21, 10.1097/00005792-199401000-00003 Cabot, 1987, Case 51-1987, N Engl J Med, 317, 1587, 10.1056/NEJM198712173172507 Powell-Jackson, 1982, Budd–Chiari syndrome: clinical patterns and therapy, Q J Med, 51, 79 Gupta, 1986, Budd–Chiari syndrome: long-term survival and factors affecting mortality, Q J Med, 60, 781 Murphy, 1986, The Budd–Chiari syndrome: aA review, Am J Roentgenol, 147, 9, 10.2214/ajr.147.1.9 Maddrey, 1984, Hepatic vein thrombosis (Budd–Chiari syndrome), Hepatology, 4, 44S, 10.1002/hep.1840040713 Patel, 2006, Prevalence of the activating JAK2 tyrosine kinase mutation V617F in the Budd–Chiari syndrome, Gastroenterology, 130, 2031, 10.1053/j.gastro.2006.04.008 Qi, 2011, Meta-analysis: the significance of screening for JAK2V617F mutation in Budd–Chiari syndrome and portal venous system thrombosis, Aliment Pharmacol Ther, 33, 1087, 10.1111/j.1365-2036.2011.04627.x Klampfl, 2013, Somatic mutations of calreticulin in myeloproliferative neoplasms, N Engl J Med, 369, 2379, 10.1056/NEJMoa1311347 Nangalia, 2013, Somatic CALR mutations in myeloproliferative neoplasms with nonmutated JAK2, N Engl J Med, 369, 2391, 10.1056/NEJMoa1312542 Turon, 2015, Role of calreticulin mutations in the aetiological diagnosis of splanchnic vein thrombosis, J Hepatol, 62, 72, 10.1016/j.jhep.2014.08.032 Plompen, 2015, Somatic calreticulin mutations in patients with Budd–Chiari syndrome and portal vein thrombosis, Haematologica, 100, e226, 10.3324/haematol.2014.120857 Valla, 1986, Risk of hepatic vein thrombosis in relation to recent use of oral contraceptives. A case–control study, Gastroenterology, 90, 807, 10.1016/0016-5085(86)90855-3 Lewis, 1983, Budd–Chiari syndrome associated with oral contraceptive steroids. Review of treatment of 47 cases, Dig Dis Sci, 28, 673, 10.1007/BF01312555 Lalonde, 1982, Inferior vena cava stenosis and Budd–Chiari syndrome in a woman taking oral contraceptives, Gastroenterology, 82, 1452, 10.1016/0016-5085(82)90083-X Khuroo, 1980, Budd–Chiari syndrome following pregnancy. Report of 16 cases, with roentgenologic, hemodynamic and histologic studies of the hepatic outflow tract, Am J Med, 68, 113, 10.1016/0002-9343(80)90180-1 Perarnau, 2008, Hepatic vascular involvement related to pregnancy, oral contraceptives, and estrogen replacement therapy, Semin Liver Dis, 28, 315, 10.1055/s-0028-1085099 Zeitoun, 1999, Outcome of Budd–Chiari syndrome: a multivariate analysis of factors related to survival including surgical portosystemic shunting, Hepatology, 30, 84, 10.1002/hep.510300125 Garcia-Pagán, 2008, TIPS for Budd–Chiari syndrome: long-term results and prognostics factors in 124 patients, Gastroenterology, 135, 808, 10.1053/j.gastro.2008.05.051 Qi, 2015, Survival and prognostic indicators of Budd–Chiari syndrome: a systematic review of 79 studies, Expert Rev Gastroenterol Hepatol, 4124, 1, 10.1586/17474124.2015.1024224 Rautou, 2009, Prognostic indices for Budd–Chiari syndrome: valid for clinical studies but insufficient for individual management, Am J Gastroenterol, 104, 1140, 10.1038/ajg.2009.63 Seijo, 2013, Good long-term outcome of Budd–Chiari syndrome with a step-wise management, Hepatology, 57, 1962, 10.1002/hep.26306 Min, 1997, Reassessing the role of medical therapy in the management of hepatic vein thrombosis, Liver Transplant Surg, 3, 423, 10.1002/lt.500030410 2016, EASL Clinical Practice Guidelines: vascular diseases of the liver, J Hepatol, 64, 179, 10.1016/j.jhep.2015.07.040 Raju, 1996, Thrombolysis for acute Budd–Chiari syndrome: case report and literature review, Am J Gastroenterol, 91, 1262 Sharma, 2004, Pharmacological thrombolysis in Budd Chiari syndrome: a single centre experience and review of the literature, J Hepatol, 40, 172, 10.1016/j.jhep.2003.09.028 Cui, 2016, Percutaneous recanalization for hepatic vein-type Budd–Chiari syndrome: long-term patency and survival, Hepatol Int, 10, 363, 10.1007/s12072-015-9676-3 Shalimar, 2016, Hepatic venous outflow tract obstruction: treatment outcomes and development of a new prognostic score, Aliment Pharmacol Ther, 43, 1154, 10.1111/apt.13604 Orloff, 2000, A 27-year experience with surgical treatment of Budd–Chiari syndrome, Ann Surg, 232, 340, 10.1097/00000658-200009000-00006 Panis, 1994, Portosystemic shunt in Budd–Chiari syndrome: long-term survival and factors affecting shunt patency in 25 patients in Western countries, Surgery, 115, 276 Orloff, 1992, Long-term results of treatment of Budd–Chiari syndrome with portal decompression, Arch Surg, 127, 1182, 10.1001/archsurg.1992.01420100040007 Hernández-Guerra, 2004, PTFE-covered stents improve TIPS patency in Budd–Chiari syndrome, Hepatology, 40, 1197, 10.1002/hep.20436 Tripathi, 2014, Good clinical outcomes following transjugular intrahepatic portosystemic stent-shunts in Budd–Chiari syndrome, Aliment Pharmacol Ther, 39, 864, 10.1111/apt.12668 Ulrich, 2008, Eighteen years of liver transplantation experience in patients with advanced Budd–Chiari syndrome, Liver Transplant, 14, 144, 10.1002/lt.21282 Schouten, 2011, Idiopathic noncirrhotic portal hypertension, Hepatology, 54, 1071, 10.1002/hep.24422 Hillaire, 2002, Idiopathic non-cirrhotic intrahepatic portal hypertension in the West: a re-evaluation in 28 patients, Gut, 51, 275, 10.1136/gut.51.2.275 Eapen, 2011, Non-cirrhotic intrahepatic portal hypertension: associated gut diseases and prognostic factors, Dig Dis Sci, 56, 227, 10.1007/s10620-010-1278-2 Moran, 1991, Nodular regenerative hyperplasia of the liver in children, Am J Surg Pathol, 15, 449, 10.1097/00000478-199105000-00004 Vilarinho, 2016, Recurrent recessive mutation in deoxyguanosine kinase causes idiopathic noncirrhotic portal hypertension, Hepatology, 63, 1977, 10.1002/hep.28499 Koot, 2016, A de novo mutation in KCNN3 associated with autosomal dominant idiopathic non-cirrhotic portal hypertension, J Hepatol, 64, 974, 10.1016/j.jhep.2015.11.027 Sarin, 2007, Noncirrhotic portal fibrosis/idiopathic portal hypertension: APASL recommendations for diagnosis and treatment, Hepatol Int, 1, 398, 10.1007/s12072-007-9010-9 Krasinskas, 2005, Liver transplantation for severe intrahepatic noncirrhotic portal hypertension, Liver Transplant, 11, 627, 10.1002/lt.20431 Siramolpiwat, 2014, Idiopathic portal hypertension: natural history and long-term outcome, Hepatology, 59, 2276, 10.1002/hep.26904 Schouten, 2012, Idiopathic noncirrhotic portal hypertension is associated with poor survival: results of a long-term cohort study, Aliment Pharmacol Ther, 35, 1424, 10.1111/j.1365-2036.2012.05112.x Chang, 2009, Idiopathic portal hypertension in patients with HIV infection treated with highly active antiretroviral therapy, Am J Gastroenterol, 104, 1707, 10.1038/ajg.2009.165 Seijo, 2013, Metabolomics discloses potential biomarkers for the noninvasive diagnosis of idiopathic portal hypertension, Am J Gastroenterol, 108, 926, 10.1038/ajg.2013.11 Chawla, 1997, Sclerotherapy in noncirrhotic portal fibrosis, Dig Dis Sci, 42, 1449, 10.1023/A:1018858525312 Bhargava, 1991, Efficacy of endoscopic sclerotherapy on long-term management of oesophageal varices: a comparative study of results in patients with cirrhosis of the liver, non-cirrhotic portal fibrosis (NCPF) and extrahepatic portal venous obstruction (EHO), J Gastroenterol Hepatol, 6, 471, 10.1111/j.1440-1746.1991.tb00890.x Bissonnette, 2016, Role of the transjugular intrahepatic portosystemic shunt in the management of severe complications of portal hypertension in idiopathic noncirrhotic portal hypertension, Hepatology, 64, 224, 10.1002/hep.28547 Karagul, 2016, Portosystemic shunt surgery in patients with idiopathic noncirrhotic portal hypertension, Ann Transplant, 21, 317, 10.12659/AOT.898253 Rajani, 2010, The epidemiology and clinical features of portal vein thrombosis: a multicentre study, Aliment Pharmacol Ther, 32, 1154, 10.1111/j.1365-2036.2010.04454.x Sarin, 2006, Noncirrhotic portal hypertension, Clin Liver Dis, 10, 627, 10.1016/j.cld.2006.08.021 Kumar, 2001, Mesenteric venous thrombosis, N Engl J Med, 345, 1683, 10.1056/NEJMra010076 Clavien, 1990, Diagnosis and management of mesenteric infarction, Br J Surg, 77, 601, 10.1002/bjs.1800770602 de Franchis, 2015, Expanding consensus in portal hypertension: report of the Baveno VI Consensus Workshop: stratifying risk and individualizing care for portal hypertension, J Hepatol, 63, 743, 10.1016/j.jhep.2015.05.022 Sogaard, 2007, Portal vein thrombosis; risk factors, clinical presentation and treatment, BMC Gastroenterol, 7, 34, 10.1186/1471-230X-7-34 Sarin, 2006, Consensus on extra-hepatic portal vein obstruction, Liver Int, 26, 512, 10.1111/j.1478-3231.2006.01269.x Plessier, 2010, Acute portal vein thrombosis unrelated to cirrhosis: a prospective multicenter follow-up study, Hepatology, 51, 210, 10.1002/hep.23259 Choudhry, 2016, Pylephlebitis: a review of 95 cases, J Gastrointest Surg, 20, 656, 10.1007/s11605-015-2875-3 Kanellopoulou, 2010, Pylephlebitis: an overview of non-cirrhotic cases and factors related to outcome, Scand J Infect Dis, 42, 804, 10.3109/00365548.2010.508464 Condat, 2000, Recent portal or mesenteric venous thrombosis: increased recognition and frequent recanalization on anticoagulant therapy, Hepatology, 32, 466, 10.1053/jhep.2000.16597 Chawla, 2015, Portal vein thrombosis, J Clin Exp Hepatol, 5, 22, 10.1016/j.jceh.2014.12.008 Turnes, 2008, Portal hypertension-related complications after acute portal vein thrombosis: impact of early anticoagulation, Clin Gastroenterol Hepatol, 6, 1412, 10.1016/j.cgh.2008.07.031 Mínguez, 2006, Noncirrhotic portal vein thrombosis exhibits neuropsychological and MR changes consistent with minimal hepatic encephalopathy, Hepatology, 43, 707, 10.1002/hep.21126 Tessler, 1991, Diagnosis of portal vein thrombosis: value of color Doppler imaging, Am J Roentgenol, 157, 293, 10.2214/ajr.157.2.1853809 Ueno, 1997, Color Doppler ultrasonography in the diagnosis of cavernous transformation of the portal vein, J Clin Ultrasound, 25, 227, 10.1002/(SICI)1097-0096(199706)25:5<227::AID-JCU2>3.0.CO;2-F Van Gansbeke, 1985, Sonographic features of portal vein thrombosis, Am J Roentgenol, 144, 749, 10.2214/ajr.144.4.749 Taylor, 1992, Computed tomography in the evaluation of the portal venous system, J Clin Gastroenterol, 14, 167, 10.1097/00004836-199203000-00021 Marn, 1992, CT of portal venous occlusion, Am J Roentgenol, 159, 717, 10.2214/ajr.159.4.1326882 Berzigotti, 2014, Imaging in clinical decision-making for portal vein thrombosis, Nat Rev Gastroenterol Hepatol, 11, 308, 10.1038/nrgastro.2013.258 Condat, 2006, Nonmalignant portal vein thrombosis in adults, Nat Clin Pract Gastroenterol Hepatol, 3, 505, 10.1038/ncpgasthep0577 Llop, 2016, Actuación ante la trombosis portal no cirrótica no tumoral, Gastroenterol Hepatol, 39, 403, 10.1016/j.gastrohep.2015.09.007 Garcia-Pagán, 2008, Extrahepatic portal vein thrombosis, Semin Liver Dis, 28, 282, 10.1055/s-0028-1085096 D’Amico, 2013, Thrombophilic genetic factors PAI-1, MTHFRC677T, V Leiden 506Q, and prothrombin 20210A in noncirrhotic portal vein thrombosis and Budd–Chiari syndrome in a Caucasian population, Int J Vasc Med, 2013, 1, 10.1155/2013/717480 Harmanci, 2007, The etiologic distribution of thrombophilic factors in chronic portal vein thrombosis, J Clin Gastroenterol, 41, 521, 10.1097/01.mcg.0000225635.52780.47 Bureau, 2016, Central obesity is associated with non-cirrhotic portal vein thrombosis, J Hepatol, 64, 427, 10.1016/j.jhep.2015.08.024 Noronha Ferreira, 2016, Natural history and management of esophagogastric varices in chronic non-cirrhotic non-tumoral portal vein thrombosis, Hepatology, 63, 1640, 10.1002/hep.28466 Dhiman, 2014, Portal cavernoma cholangiopathy: Consensus Statement of a Working Party of the Indian National Association for Study of the Liver, J Clin Exp Hepatol, 4, S2, 10.1016/j.jceh.2014.02.003 Llop, 2011, Portal cholangiopathy: radiological classification and natural history, Gut, 60, 853, 10.1136/gut.2010.230201 Condat, 2003, Portal cavernoma-associated cholangiopathy: a clinical and MR cholangiography coupled with MR portography imaging study, Hepatology, 37, 1302, 10.1053/jhep.2003.50232 Chandra, 2001, Portal biliopathy, J Gastroenterol Hepatol, 16, 1086, 10.1046/j.1440-1746.2001.02562.x Hall, 2011, Management of acute non-cirrhotic and non-malignant portal vein thrombosis: a systematic review, World J Surg, 35, 2510, 10.1007/s00268-011-1198-0 Hall, 2013, Impact of anticoagulation on outcomes in acute non-cirrhotic and non-malignant portal vein thrombosis: a retrospective observational study, Hepatogastroenterology, 60, 311 Smalberg, 2008, Risks and benefits of transcatheter thrombolytic therapy in patients with splanchnic venous thrombosis, Thromb Haemost, 100, 1084, 10.1160/TH08-01-0015 Ferro, 2007, Transjugular intrahepatic portosystemic shunt, mechanical aspiration thrombectomy, and direct thrombolysis in the treatment of acute portal and superior mesenteric vein thrombosis, Cardiovasc Intervent Radiol, 30, 1070, 10.1007/s00270-007-9137-z Hollingshead, 2005, Transcatheter thrombolytic therapy for acute mesenteric and portal vein thrombosis, J Vasc Interv Radiol, 16, 651, 10.1097/01.RVI.0000156265.79960.86 Liu, 2010, Interventional therapy for symptomatic-benign portal vein occlusion, Hepatogastroenterology, 57, 1367 Wang, 2011, Acute symptomatic mesenteric venous thrombosis: treatment by catheter-directed thrombolysis with transjugular intrahepatic route, Abdom Imaging, 36, 390, 10.1007/s00261-010-9637-1 Malkowski, 2003, Thrombolytic treatment of portal thrombosis, Hepatogastroenterology, 50, 2098 Cao, 2013, Treatment of postoperative main portal vein and superior mesenteric vein thrombosis with balloon angioplasty and/or stent placement, Acta Radiol, 54, 526, 10.1177/0284185113475917 Spaander, 2013, Anticoagulant therapy in patients with non-cirrhotic portal vein thrombosis: effect on new thrombotic events and gastrointestinal bleeding, J Thromb Haemost, 11, 452, 10.1111/jth.12121 Condat, 2001, Current outcome of portal vein thrombosis in adults: risk and benefit of anticoagulant therapy, Gastroenterology, 120, 490, 10.1053/gast.2001.21209 Francoz, 2012, Portal vein thrombosis, cirrhosis, and liver transplantation, J Hepatol, 57, 203, 10.1016/j.jhep.2011.12.034 Qi, 2012, Transjugular intrahepatic portosystemic shunt for portal cavernoma with symptomatic portal hypertension in non-cirrhotic patients, Dig Dis Sci, 57, 1072, 10.1007/s10620-011-1975-5 Senzolo, 2007, Transjugular intrahepatic portosystemic shunt for portal vein thrombosis with and without underlying cirrhosis, Cardiovasc Intervent Radiol, 30, 545, 10.1007/s00270-006-0321-3 Orloff, 2014, Unshuntable extrahepatic portal hypertension revisited: 43 years’ experience with radical esophagogastrectomy treatment of bleeding esophagogastric varices, Am J Surg, 207, 46, 10.1016/j.amjsurg.2013.06.001 Mack, 2006, Surgically restoring portal blood flow to the liver in children with primary extrahepatic portal vein thrombosis improves fluid neurocognitive ability, Pediatrics, 117, e405, 10.1542/peds.2005-1177 Superina, 2006, Medical and surgical management of portal hypertension in children, Curr Treat Options Gastroenterol, 9, 432, 10.1007/BF02738533 Varma, 2014, Surgical management of portal cavernoma cholangiopathy, J Clin Exp Hepatol, 4, S77, 10.1016/j.jceh.2013.07.005 Denninger, 2000, Cause of portal or hepatic venous thrombosis in adults: the role of multiple concurrent factors, Hepatology, 31, 587, 10.1002/hep.510310307 Tripodi, 2011, The coagulopathy of chronic liver disease, N Engl J Med, 365, 147, 10.1056/NEJMra1011170 Northup, 2006, Coagulopathy does not fully protect hospitalized cirrhosis patients from peripheral venous thromboembolism, Am J Gastroenterol, 101, 1524, 10.1111/j.1572-0241.2006.00588.x Søgaard, 2009, Risk of venous thromboembolism in patients with liver disease: a nationwide population-based case-control study, Am J Gastroenterol, 104, 96, 10.1038/ajg.2008.34 Rodríguez-Castro, 2012, Management of nonneoplastic portal vein thrombosis in the setting of liver transplantation, Transplant J, 94, 1145, 10.1097/TP.0b013e31826e8e53 Francoz, 2005, Splanchnic vein thrombosis in candidates for liver transplantation: usefulness of screening and anticoagulation, Gut, 54, 691, 10.1136/gut.2004.042796 Zocco, 2009, Thrombotic risk factors in patients with liver cirrhosis: correlation with MELD scoring system and portal vein thrombosis development, J Hepatol, 51, 682, 10.1016/j.jhep.2009.03.013 Nery, 2015, Causes and consequences of portal vein thrombosis in 1,243 patients with cirrhosis: results of a longitudinal study, Hepatology, 61, 660, 10.1002/hep.27546 Violi, 2016, Portal vein thrombosis relevance on liver cirrhosis: Italian Venous Thrombotic Events Registry, Intern Emerg Med, 11, 1059, 10.1007/s11739-016-1416-8 Ögren, 2006, Portal vein thrombosis: prevalence, patient characteristics and lifetime risk: a population study based on 23796 consecutive autopsies, World J Gastroenterol, 12, 2115, 10.3748/wjg.v12.i13.2115 Amitrano, 2012, Splanchnic vein thrombosis and variceal rebleeding in patients with cirrhosis, Eur J Gastroenterol Hepatol, 24, 1381, 10.1097/MEG.0b013e328357d5d4 Augustin, 2011, Effectiveness of combined pharmacologic and ligation therapy in high-risk patients with acute esophageal variceal bleeding, Am J Gastroenterol, 106, 1787, 10.1038/ajg.2011.173 D’Amico, 2003, Upper digestive bleeding in cirrhosis. Post-therapeutic outcome and prognostic indicators, Hepatology, 38, 599, 10.1053/jhep.2003.50385 Amitrano, 2004, Risk factors and clinical presentation of portal vein thrombosis in patients with liver cirrhosis, J Hepatol, 40, 736, 10.1016/j.jhep.2004.01.001 Englesbe, 2010, Portal vein thrombosis and survival in patients with cirrhosis, Liver Transplant, 16, 83, 10.1002/lt.21941 Tripodi, 2009, An Imbalance of pro- vs anti-coagulation factors in plasma from patients with cirrhosis, Gastroenterology, 137, 2105, 10.1053/j.gastro.2009.08.045 Amitrano, 2000, Inherited coagulation disorders in cirrhotic patients with portal vein thrombosis, Hepatology, 31, 345, 10.1002/hep.510310213 Erkan, 2005, Thrombophilic gene mutations in cirrhotic patients with portal vein thrombosis, Eur J Gastroenterol Hepatol, 17, 339, 10.1097/00042737-200503000-00013 Amitrano, 2002, Portal vein thrombosis after variceal endoscopic sclerotherapy in cirrhotic patients: role of genetic thrombophilia, Endoscopy, 34, 535, 10.1055/s-2002-33210 Tublin, 1997, Benign and malignant portal vein thrombosis: differentiation by CT characteristics, Am J Roentgenol, 168, 719, 10.2214/ajr.168.3.9057522 Rossi, 2006, Contrast-enhanced versus conventional and color doppler sonography for the detection of thrombosis of the portal and hepatic venous systems, Am J Roentgenol, 186, 763, 10.2214/AJR.04.1218 Luca, 2012, Natural course of extrahepatic nonmalignant partial portal vein thrombosis in patients with cirrhosis, Radiology, 265, 124, 10.1148/radiol.12112236 John, 2013, Impact of untreated portal vein thrombosis on pre and post liver transplant outcomes in cirrhosis, Ann Hepatol, 12, 952, 10.1016/S1665-2681(19)31301-8 Senzolo, 2012, Prospective evaluation of anticoagulation and transjugular intrahepatic portosistemic shunt for the management of portal vein thrombosis in cirrhosis, Liver Int, 32, 919, 10.1111/j.1478-3231.2012.02785.x Villa, 2012, Enoxaparin prevents portal vein thrombosis and liver decompensation in patients with advanced cirrhosis, Gastroenterology, 143, 1253, 10.1053/j.gastro.2012.07.018 Amitrano, 2010, Safety and efficacy of anticoagulation therapy with low molecular weight heparin for portal vein thrombosis in patients with liver cirrhosis, JClinGastroenterol, 44, 448 Delgado, 2012, Efficacy and safety of anticoagulation on patients with cirrhosis and portal vein thrombosis, Clin Gastroenterol Hepatol, 10, 776, 10.1016/j.cgh.2012.01.012 Werner, 2013, Portal vein thrombosis in patients with end stage liver disease awaiting liver transplantation: outcome of anticoagulation, Dig Dis Sci, 58, 1776, 10.1007/s10620-012-2548-y Senzolo, 2016, PS064-Long term outcome of splanchnic vein thrombosis in cirrhosis, J Hepatol, 64, S168, 10.1016/S0168-8278(16)01680-9 Cerini, 2015, Impact of anticoagulation on upper-gastrointestinal bleeding in cirrhosis. A retrospective multicenter study, Hepatology, 62, 575, 10.1002/hep.27783 Martinez, 2014, Treatment of acute portal vein thrombosis by nontraditional anticoagulation, Hepatology, 60, 425, 10.1002/hep.26998 Intagliata, 2015, Treating thrombosis in cirrhosis patients with new oral agents: ready or not?, Hepatology, 61, 738, 10.1002/hep.27225 Intagliata, 2016, Direct oral anticoagulants in cirrhosis patients pose similar risks of bleeding when compared to traditional anticoagulation, Dig Dis Sci, 61, 1721, 10.1007/s10620-015-4012-2 Senzolo, 2006, Transjugular intrahepatic portosystemic shunt for portal vein thrombosis with and without cavernous transformation, Aliment Pharmacol Ther, 23, 767, 10.1111/j.1365-2036.2006.02820.x Luca, 2011, Short- and long-term effects of the transjugular intrahepatic portosystemic shunt on portal vein thrombosis in patients with cirrhosis, Gut, 60, 846, 10.1136/gut.2010.228023 Perarnau, 2010, Feasibility and long-term evolution of TIPS in cirrhotic patients with portal thrombosis, Eur J Gastroenterol Hepatol, 22, 1093, 10.1097/MEG.0b013e328338d995 Han, 2011, Transjugular intrahepatic portosystemic shunt for portal vein thrombosis with symptomatic portal hypertension in liver cirrhosis, J Hepatol, 54, 78, 10.1016/j.jhep.2010.06.029 Senzolo, 2011, Tips for portal vein thrombosis (pvt) in cirrhosis: not only unblocking a pipe, J Hepatol, 55, 945, 10.1016/j.jhep.2011.02.027 Vauthey, 1997, The arterioportal fistula syndrome: clinicopathologic features, diagnosis, and therapy, Gastroenterology, 113, 1390, 10.1053/gast.1997.v113.pm9322535 Hernández, 2016, Arterioportal fistula, a rare cause of presinusoidal portal hypertension, Gastroenterol Hepatol, 39, 43, 10.1016/j.gastrohep.2015.04.010 Okuda, 1978, Frequency of intrahepatic arteriovenous fistula as a sequela to percutaneous needle puncture of the liver, Gastroenterology, 74, 1204, 10.1016/0016-5085(78)90693-5 Hertzanu, 1984, Massive arterioportal fistula in hepatocellular carcinoma, Am J Gastroenterol, 79, 403 Guzman, 2006, Arterioportal fistulas introduction of a novel classification with therapeutic implications, J Gastrointest Surg, 10, 543, 10.1016/j.gassur.2005.06.022 Zhang, 2015, Portal hypertension induced by congenital hepatic arterioportal fistula: report of four clinical cases and review of the literature, World J Gastroenterol, 21, 2229, 10.3748/wjg.v21.i7.2229 Roman, 1987, Transcatheter embolization of hepatic arteriovenous fistula in osler-weber-rendu disease — a case report, Angiology, 38, 484, 10.1177/000331978703800610 Kahn, 1988, The Ehlers-Danlos syndrome, typeIV, with an unusual combination of organ malformations, Cardiovasc Interv Radiol, 11, 288, 10.1007/BF02577038 Capron, 1984, Inferior mesenteric arteriovenous fistula associated with portal hypertension and acute ischemic colitis. Successful occlusion by intraarterial embolization with steel coils, Gastroenterology, 86, 351, 10.1016/0016-5085(84)90422-0 Strodel, 1987, Presentation and perioperative management of arterioportal fistulas, Arch Surg, 122, 563, 10.1001/archsurg.1987.01400170069010 Lumsden, 1993, Hepatic arterioportal fistula, Am Surg, 59, 722 Ahn, 2010, Nontumorous arterioportal shunts in the liver: CT and MRI findings considering mechanisms and fate, Eur Radiol, 20, 385, 10.1007/s00330-009-1542-z Choi, 2002, Hepatic arterioportal shunts: dynamic CT and MR features, Korean J Radiol, 3, 1, 10.3348/kjr.2002.3.1.1 Detry, 2006, Selective coil occlusion of a large arterioportal fistula in a liver graft, Liver Transplant, 12, 888, 10.1002/lt.20695 Hirakawa, 2013, Clinical outcomes of symptomatic arterioportal fistulas after transcatheter arterial embolization, World J Radiol, 5, 33, 10.4329/wjr.v5.i2.33 Kumar, 2012, Hepatic arteriovenous fistulae: role of interventional radiology, Dig Dis Sci, 57, 2703, 10.1007/s10620-012-2331-0 Stringer, 2008, The clinical anatomy of congenital portosystemic venous shunts, Clin Anat, 21, 147, 10.1002/ca.20574 Witters, 2008, Congenital veno-venous malformations of the liver: widely variable clinical presentations, J Gastroenterol Hepatol, 23, e390, 10.1111/j.1440-1746.2007.05156.x Park, 1990, Intrahepatic portosystemic venous shunt, Am J Roentgenol, 155, 527, 10.2214/ajr.155.3.2117349 Hu, 2008, Insight into congenital absence of the portal vein: is it rare?, World J Gastroenterol, 14, 5969, 10.3748/wjg.14.5969 Morgan, 1994, Congenital absence of the portal vein: two cases and a proposed classification system for portasystemic vascular anomalies, J Pediatr Surg, 29, 1239, 10.1016/0022-3468(94)90812-5 Raskin, 1964, Portal-systemic encephalopathy due to congenital intrahepatic shunts, N Engl J Med, 270, 225, 10.1056/NEJM196401302700503 Chagnon, 1986, Aneurysmal portahepatic venous fistula: report of two cases, Radiology, 159, 693, 10.1148/radiology.159.3.3517953 Turkbey, 2006, Multiple hyperplastic nodules in the liver with congenital absence of portal vein: MRI findings, Pediatr Radiol, 36, 445, 10.1007/s00247-005-0103-0 Kim, 2004, Hepatic nodular lesions associated with abnormal development of the portal vein, Am J Roentgenol, 183, 1333, 10.2214/ajr.183.5.1831333 Barton, 1989, Liver transplantation for hepatoblastoma in a child with congenital absence of the portal vein, Pediatr Radiol, 20, 113, 10.1007/BF02010653 Ohno, 2008, Pulmonary hypertension in patients with congenital portosystemic venous shunt: a previously unrecognized association, Pediatrics, 121, e892, 10.1542/peds.2006-3411 Alvarez, 2002, Abernethy malformation: one of the etiologies of hepatopulmonary syndrome, Pediatr Pulmonol, 34, 391, 10.1002/ppul.10182 Gallego, 2004, Congenital hepatic shunts, RadioGraphics, 24, 755, 10.1148/rg.243035046 Gitzelmann, 1997, Hypergalactosaemia in a newborn: self-limiting intrahepatic portosystemic venous shunt, Eur J Pediatr, 156, 719, 10.1007/s004310050698 Franchi-Abella, 2010, Complications of congenital portosystemic shunts in children: therapeutic options and outcomes, J Pediatr Gastroenterol Nutr, 51, 1, 10.1097/MPG.0b013e3181d9cb92 Kim, 2012, Clinical features of congenital portosystemic shunt in children, Eur J Pediatr, 171, 395, 10.1007/s00431-011-1564-9 Evans, 2009, Congenital portosystemic shunts and AMPLATZER vascular plug occlusion in newborns, Pediatr Cardiol, 30, 1083, 10.1007/s00246-009-9501-7 Lautz, 2011, Management and classification of type II congenital portosystemic shunts, J Pediatr Surg, 46, 308, 10.1016/j.jpedsurg.2010.11.009 Lam, 1981, Spontaneous portosystemic shunt: relationship to spontaneous encephalopathy and gastrointestinal hemorrhage, Dig Dis Sci, 26, 346, 10.1007/BF01308377 Ohnishi, 1986, Clinical and portal hemodynamic features in cirrhotic patients having a large spontaneous splenorenal and/or gastrorenal shunt, Am J Gastroenterol, 81, 450 Riggio, 2005, High prevalence of spontaneous portal-systemic shunts in persistent hepatic encephalopathy: a case–control study, Hepatology, 42, 1158, 10.1002/hep.20905 2014, Hepatic encephalopathy in chronic liver disease: 2014 Practice Guideline by the European Association for the Study of the Liver and the American Association for the Study of Liver Diseases, J Hepatol, 61, 642, 10.1016/j.jhep.2014.05.042 Vilstrup, 2014, Hepatic encephalopathy in chronic liver disease: 2014 Practice Guideline by the American Association for the Study of Liver Diseases and the European Association for the Study of the Liver, Hepatology, 60, 715, 10.1002/hep.27210 Laleman, 2013, Embolization of large spontaneous portosystemic shunts for refractory hepatic encephalopathy: a multicenter survey on safety and efficacy, Hepatology, 57, 2448, 10.1002/hep.26314 Lynn, 2016, Embolization of portosystemic shunts for treatment of medically refractory hepatic encephalopathy, Liver Transplant, 22, 723, 10.1002/lt.24440 Lenato, 2006, Hereditary haemorrhagic telangiectasia (HHT): genetic and molecular aspects, Curr Pharm Des, 12, 1173, 10.2174/138161206776361291 Aassar, 1991, The natural history of epistaxis in hereditary hemorrhagic telangiectasia, Laryngoscope, 101, 977, 10.1288/00005537-199109000-00008 Govani, 2009, Hereditary haemorrhagic telangiectasia: a clinical and scientific review, Eur J Hum Genet, 17, 860, 10.1038/ejhg.2009.35 Lesca, 2008, Hereditary hemorrhagic telangiectasia: evidence for regional founder effects of ACVRL1 mutations in French and Italian patients, Eur J Hum Genet, 16, 742, 10.1038/ejhg.2008.3 Latino, 2014, Targeting under-diagnosis in hereditary hemorrhagic telangiectasia: a model approach for rare diseases?, Orphanet J Rare Dis, 9, 115, 10.1186/s13023-014-0115-7 Lux, 2013, HHT diagnosis by mid-infrared spectroscopy and artificial neural network analysis, Orphanet J Rare Dis, 8, 94, 10.1186/1750-1172-8-94 Shovlin, 2000, Diagnostic criteria for hereditary hemorrhagic telangiectasia (Rendu–Osler–Weber syndrome), Am J Med Genet, 91, 66, 10.1002/(SICI)1096-8628(20000306)91:1<66::AID-AJMG12>3.0.CO;2-P Faughnan, 2011, International guidelines for the diagnosis and management of hereditary haemorrhagic telangiectasia, J Med Genet, 48, 73, 10.1136/jmg.2009.069013 Gougos, 1990, Primary structure of endoglin, an RGD-containing glycoprotein of human endothelial cells, J Biol Chem, 265, 8361, 10.1016/S0021-9258(19)38892-1 McDonald, 1994, A disease locus for hereditary haemorrhagic telangiectasia maps to chromosome 9q33-34, Nat Genet, 6, 197, 10.1038/ng0294-197 McAllister, 1994, Endoglin, a TGF-β binding protein of endothelial cells, is the gene for hereditary haemorrhagic telangiectasia type 1, Nat Genet, 8, 345, 10.1038/ng1294-345 Johnson, 1995, A second locus for hereditary hemorrhagic telangiectasia maps to chromosome 12, Genome Res, 5, 21, 10.1101/gr.5.1.21 Vincent, 1995, A third locus for hereditary haemorrhagic telangiectasia maps to chromosome 12q, Hum Mol Genet, 4, 945, 10.1093/hmg/4.5.945 Gallione, 2004, A combined syndrome of juvenile polyposis and hereditary haemorrhagic telangiectasia associated with mutations in MADH4 (SMAD4), Lancet, 363, 852, 10.1016/S0140-6736(04)15732-2 Canzonieri, 2014, Endoscopic evaluation of gastrointestinal tract in patients with hereditary hemorrhagic telangiectasia and correlation with their genotypes, Genet Med, 16, 3, 10.1038/gim.2013.62 Cole, 2005, A new locus for hereditary haemorrhagic telangiectasia (HHT3) maps to chromosome 5, J Med Genet, 42, 577, 10.1136/jmg.2004.028712 Bayrak-Toydemir, 2006, A fourth locus for hereditary hemorrhagic telangiectasia maps to chromosome 7, Am J Med Genet A, 140, 2155, 10.1002/ajmg.a.31450 Wooderchak-Donahue, 2013, BMP9 mutations cause a vascular-anomaly syndrome with phenotypic overlap with hereditary hemorrhagic telangiectasia, Am J Hum Genet, 93, 530, 10.1016/j.ajhg.2013.07.004 Guttmacher, 2004, The family history—more important than ever, N Engl J Med, 351, 2333, 10.1056/NEJMsb042979 Fontalba, 2008, Mutation study of Spanish patients with hereditary hemorrhagic telangiectasia, BMC Med Genet, 9, 75, 10.1186/1471-2350-9-75 Bayrak-Toydemir, 2006, Genotype–phenotype correlation in hereditary hemorrhagic telangiectasia: mutations and manifestations, Am J Med Genet A, 140, 463, 10.1002/ajmg.a.31101 Letteboer, 2005, Genotype–phenotype relationship in hereditary haemorrhagic telangiectasia, J Med Genet, 43, 371, 10.1136/jmg.2005.035451 Garcia-Tsao, 2000, Liver disease in patients with hereditary hemorrhagic telangiectasia, N Engl J Med, 343, 931, 10.1056/NEJM200009283431305 Buscarini, 2006, Liver involvement in hereditary hemorrhagic telangiectasia: consensus recommendations, Liver Int, 26, 1040, 10.1111/j.1478-3231.2006.01340.x Buscarini, 2004, Doppler ultrasonographic grading of hepatic vascular malformations in hereditary hemorrhagic telangiectasia—results of extensive screening, Ultraschall Med, 25, 348, 10.1055/s-2004-813549 Buonamico, 2008, Liver involvement in a large cohort of patients with hereditary hemorrhagic telangiectasia: echo-color-Doppler vs multislice computed tomography study, J Hepatol, 48, 811, 10.1016/j.jhep.2007.12.022 Ianora, 2004, Hereditary hemorrhagic telangiectasia: multi-detector row helical CT assessment of hepatic involvement, Radiology, 230, 250, 10.1148/radiol.2301021745 Shovlin, 2010, Hereditary haemorrhagic telangiectasia: pathophysiology, diagnosis and treatment, Blood Rev, 24, 203, 10.1016/j.blre.2010.07.001 Gincul, 2008, Evaluation of previously nonscreened hereditary hemorrhagic telangiectasia patients shows frequent liver involvement and early cardiac consequences, Hepatology, 48, 1570, 10.1002/hep.22514 Caselitz, 2003, Sonographic criteria for the diagnosis of hepatic involvement in hereditary hemorrhagic telangiectasia (HHT), Hepatology, 37, 1139, 10.1053/jhep.2003.50197 Sabbà, 2008, Review article: the hepatic manifestations of hereditary haemorrhagic telangiectasia, Aliment Pharmacol Ther, 28, 523, 10.1111/j.1365-2036.2008.03775.x Ginon, 2013, Hereditary hemorrhagic telangiectasia, liver vascular malformations and cardiac consequences, Eur J Intern Med, 24, e35, 10.1016/j.ejim.2012.12.013 Wu, 2006, Liver involvement in hereditary hemorrhagic telangiectasia: CT and clinical findings do not correlate in symptomatic patients, Am J Roentgenol, 187, W399, 10.2214/AJR.05.1068 Scardapane, 2013, Hepatic nodular regenerative lesions in patients with hereditary haemorrhagic telangiectasia: computed tomography and magnetic resonance findings, Radiol Med, 118, 1, 10.1007/s11547-012-0789-3 Larson, 2003, Liver disease in hereditary hemorrhagic telangiectasia, J Clin Gastroenterol, 36, 149, 10.1097/00004836-200302000-00013 Ford, 2014, Pulmonary hypertension and hepatic encephalopathy: lethal complications of Rendu–Osler–Weber disease, J R Coll Physicians Edinb, 44, 126, 10.4997/JRCPE.2014.207 Brenard, 2010, Large spectrum of liver vascular lesions including high prevalence of focal nodular hyperplasia in patients with hereditary haemorrhagic telangiectasia: the Belgian Registry based on 30 patients, Eur J Gastroenterol Hepatol, 22, 1253, 10.1097/MEG.0b013e32833ac4a1 Khalid, 2008, Hepatic vascular malformations in hereditary hemorrhagic telangiectasia, Semin Liver Dis, 28, 247, 10.1055/s-0028-1085093 Singh, 2014, Identifying the presence of clinically significant hepatic involvement in hereditary haemorrhagic telangiectasia using a simple clinical scoring index, J Hepatol, 61, 124, 10.1016/j.jhep.2014.02.028 Buscarini, 2011, Natural history and outcome of hepatic vascular malformations in a large cohort of patients with hereditary hemorrhagic teleangiectasia, Dig Dis Sci, 56, 2166, 10.1007/s10620-011-1585-2 Costa Macedo, 2003, Hemobilia in hereditary hemorrhagic telangiectasia: an unusual complication of endoscopic retrograde cholangiopancreatography, Endoscopy, 35, 531, 10.1055/s-2003-39659 Candelli, 2012, Liver involvement in hereditary hemorrhagic telangiectasia: can breath test unmask impaired hepatic first-pass effect?, Intern Emerg Med, 7, 323, 10.1007/s11739-011-0531-9 Buscarini, 2005, Liver involvement in hereditary haemorrhagic telangiectasia or Rendu–Osler–Weber disease, Dig Liver Dis, 37, 635, 10.1016/j.dld.2005.04.010 Buscarini, 1994, Hepatic vascular malformations in hereditary hemorrhagic telangiectasia: imaging findings, Am J Roentgenol, 163, 1105, 10.2214/ajr.163.5.7976883 Cavel, 2016, Comparison between Doppler ultrasonography and multiphase multidetector-row computed tomography in the detection of liver involvement in Rendu–Osler disease: an analysis of 62 patients, Diagn Interv Imaging, 97, 451, 10.1016/j.diii.2014.01.023 Lacout, 2010, Pancreatic involvement in hereditary hemorrhagic telangiectasia: assessment with multidetector helical CT, Radiology, 254, 479, 10.1148/radiol.09090096 Barral, 2012, Hepatic and pancreatic involvement in hereditary hemorrhagic telangiectasia: quantitative and qualitative evaluation with 64-section CT in asymptomatic adult patients, Eur Radiol, 22, 161, 10.1007/s00330-011-2243-y Binsse, 2015, P16 left adrenal arteriovenous malformation growing during the 8-year follow-up of a patient with hereditary hemorrhagic telangiectasia: successful treatment by embolization, Angiogenesis, 18, 550 Bueno López, 2010 Scardapane, 2012, Dynamic 4D MR angiography versus multislice CT angiography in the evaluation of vascular hepatic involvement in hereditary haemorrhagic telangiectasia, Radiol Med, 117, 29, 10.1007/s11547-011-0688-3 Ponikowski, 2016, 2016 ESC Guidelines for the diagnosis and treatment of acute and chronic heart failure, Eur J Heart Fail, 18, 891, 10.1002/ejhf.592 Albiñana, 2012, Propranolol as antiangiogenic candidate for the therapy of hereditary haemorrhagic telangiectasia, Thromb Haemost, 108, 41, 10.1160/TH11-11-0809 Dupuis-Girod, 2012, Bevacizumab in patients with hereditary hemorrhagic telangiectasia and severe hepatic vascular malformations and high cardiac output, JAMA, 307, 948, 10.1001/jama.2012.250 Sato, 2008, Successful treatment for gastro-intestinal bleeding of Osler–Weber–Rendu disease by argon plasma coagulation using double-balloon enteroscopy, Endoscopy, 40, E228, 10.1055/s-2007-966562 Oviedo, 2002, Osler–Weber–Rendu syndrome, Gastrointest Endosc, 55, 910, 10.1067/mge.2002.121798 Kitamura, 2001, Rendu–Osler–Weber disease successfully treated by argon plasma coagulation, Gastrointest Endosc, 54, 525, 10.1067/mge.2001.116885 Zarrabeitia, 2010, A review on clinical management and pharmacological therapy on hereditary haemorrhagic telangiectasia (HHT), Curr Vasc Pharmacol, 8, 473, 10.2174/157016110791330771 Chavan, 2013, Complications and clinical outcome of hepatic artery embolisation in patients with hereditary haemorrhagic telangiectasia, Eur Radiol, 23, 951, 10.1007/s00330-012-2694-9 Sabbà, 2004, Orthotopic liver transplantation and hereditary hemorrhagic telangiectasia: do hepatic vascular malformations relapse? A long term follow up study on two patients, J Hepatol, 41, 687, 10.1016/j.jhep.2004.06.007 Núñez Viejo, 2010, Enfermedad de Rendu-Osler con afectación hepática: primer trasplante en España, Med Clin (Barc), 135, 552, 10.1016/j.medcli.2010.03.025 Dupuis-Girod, 2010, Long-term outcome of patients with hereditary hemorrhagic telangiectasia and severe hepatic involvement after orthotopic liver transplantation: a single-center study, Liver Transplant, 16, 340, 10.1002/lt.21990 Lerut, 2006, Liver transplantation for hereditary hemorrhagic telangiectasia: report of the European liver transplant registry, Ann Surg, 244, 854, 10.1097/01.sla.0000247258.35406.a4 Cag, 2011, Successful liver transplantation for Rendu–Weber–Osler disease, a single centre experience, Hepatol Int, 5, 834, 10.1007/s12072-011-9259-x DeLeve, 1999, Characterization of a reproducible rat model of hepatic veno-occlusive disease, Hepatology, 29, 1779, 10.1002/hep.510290615 Carreras, 1998, Incidence and outcome of hepatic veno-occlusive disease after blood or marrow transplantation: a prospective cohort study of the European Group for Blood and Marrow Transplantation, Blood, 92, 3599 Harb, 2009, Bone marrow progenitor cells repair rat hepatic sinusoidal endothelial cells after liver injury, Gastroenterology, 137, 704, 10.1053/j.gastro.2009.05.009 Viganò, 2013, Liver resection for colorectal metastases after chemotherapy, Ann Surg, 258, 731, 10.1097/SLA.0b013e3182a6183e Nguyen-Khac, 2013, A reappraisal of chemotherapy-induced liver injury in colorectal liver metastases before the era of antiangiogenics, Int J Hepatol, 2013, 1, 10.1155/2013/314868 Rubbia-Brandt, 2010, Sinusoidal obstruction syndrome and nodular regenerative hyperplasia are frequent oxaliplatin-associated liver lesions and partially prevented by bevacizumab in patients with hepatic colorectal metastasis, Histopathology, 56, 430, 10.1111/j.1365-2559.2010.03511.x Tallman, 2013, Incidence of sinusoidal obstruction syndrome following Mylotarg (gemtuzumab ozogamicin): a prospective observational study of 482 patients in routine clinical practice, Int J Hematol, 97, 456, 10.1007/s12185-013-1275-2 Mcdonald, 1984, Venocclusive disease of the liver after bone marrow transplantation: diagnosis, incidence, and predisposing factors, Hepatology, 4, 116, 10.1002/hep.1840040121 Jones, 1987, Venoocclusive disease of the liver following bone marrow transplantation, Transplantation, 44, 778, 10.1097/00007890-198712000-00011 Mohty, 2016, Revised diagnosis and severity criteria for sinusoidal obstruction syndrome/veno-occlusive disease in adult patients: a new classification from the European Society for Blood and Marrow Transplantation, Bone Marrow Transplant, 51, 906, 10.1038/bmt.2016.130 Lee, 1998, Relevance of proteins C and S, antithrombin III, von Willebrand factor, and factor VIII for the development of hepatic veno-occlusive disease in patients undergoing allogeneic bone marrow transplantation: a prospective study, Bone Marrow Transplant, 22, 883, 10.1038/sj.bmt.1701445 Scrobohaci, 1991, Liver veno-occlusive disease after bone marrow transplantation changes in coagulation parameters and endothelial markers, Thromb Res, 63, 509, 10.1016/0049-3848(91)90176-W Helmy, 2006, Review article: updates in the pathogenesis and therapy of hepatic sinusoidal obstruction syndrome, Aliment Pharmacol Ther, 23, 11, 10.1111/j.1365-2036.2006.02742.x Shulman, 1995, Utility of transvenous liver biopsies and wedged hepatic venous pressure measurements in sixty marrow transplant recipients, Transplantation, 59, 1015, 10.1097/00007890-199504150-00017 Rubbia-Brandt, 2010, Sinusoidal obstruction syndrome, Clin Liver Dis, 14, 651, 10.1016/j.cld.2010.07.009 Shin, 2012, Accuracy of gadoxetic acid-enhanced magnetic resonance imaging for the diagnosis of sinusoidal obstruction syndrome in patients with chemotherapy-treated colorectal liver metastases, Eur Radiol, 22, 864, 10.1007/s00330-011-2333-x Ward, 2008, Sinusoidal obstructive syndrome diagnosed with superparamagnetic iron oxide-enhanced magnetic resonance imaging in patients with chemotherapy-treated colorectal liver metastases, J Clin Oncol, 26, 4304, 10.1200/JCO.2008.16.1893 Mohty, 2015, Sinusoidal obstruction syndrome/veno-occlusive disease: current situation and perspectives—a position statement from the European Society for Blood and Marrow Transplantation (EBMT), Bone Marrow Transplant, 50, 781, 10.1038/bmt.2015.52 Imran, 2006, Use of prophylactic anticoagulation and the risk of hepatic veno-occlusive disease in patients undergoing hematopoietic stem cell transplantation: a systematic review and meta-analysis, Bone Marrow Transplant, 37, 677, 10.1038/sj.bmt.1705297 Ruutu, 2002, Ursodeoxycholic acid for the prevention of hepatic complications in allogeneic stem cell transplantation, Blood, 100, 1977, 10.1182/blood-2001-12-0159 Cheuk, 2015, Interventions for prophylaxis of hepatic veno-occlusive disease in people undergoing haematopoietic stem cell transplantation, Cochrane Database Syst Rev, 5, CD009311 Corbacioglu, 2012, Defibrotide for prophylaxis of hepatic veno-occlusive disease in paediatric haemopoietic stem-cell transplantation: an open-label, phase 3, randomised controlled trial, Lancet, 379, 1301, 10.1016/S0140-6736(11)61938-7 Dignan, 2013, BCSH/BSBMT guideline: diagnosis and management of veno-occlusive disease (sinusoidal obstruction syndrome) following haematopoietic stem cell transplantation, Br J Haematol, 163, 444, 10.1111/bjh.12558 Campos-Varela, 2012, Transjugular intrahepatic portosystemic shunt for the treatment of sinusoidal obstruction syndrome in a liver transplant recipient and review of the literature, Liver Transplant, 18, 201, 10.1002/lt.22351 Azoulay, 2000, Transjugular intrahepatic portosystemic shunt (TIPS) for severe veno-occlusive disease of the liver following bone marrow transplantation, Bone Marrow Transplant, 25, 987, 10.1038/sj.bmt.1702386 Kim, 2002, A successful liver transplantation for refractory hepatic veno-occlusive disease originating from cord blood transplantation, Am J Transplant, 2, 796, 10.1034/j.1600-6143.2002.20815.x Richardson, 2016, Phase 3 trial of defibrotide for the treatment of severe veno-occlusive disease and multi-organ failure, Blood, 127, 1656, 10.1182/blood-2015-10-676924 Senzolo, 2011, Vascular disorders of the liver: recommendations from the Italian Association for the Study of the Liver (AISF) ad hoc committee, Dig Liver Dis, 43, 503, 10.1016/j.dld.2010.11.006 Tirumani, 2014, Imaging of the porta hepatis: spectrum of disease, RadioGraphics, 34, 73, 10.1148/rg.341125190 Bach, 1996, Portal vein evaluation with US: comparison to angiography combined with CT arterial portography, Radiology, 201, 149, 10.1148/radiology.201.1.8816536 Rossi, 2008, Contrast-enhanced ultrasonography and spiral computed tomography in the detection and characterization of portal vein thrombosis complicating hepatocellular carcinoma, Eur Radiol, 18, 1749, 10.1007/s00330-008-0931-z Venturi, 2007, Role of real-time contrast-enhanced ultrasound in the assessment of metastatic portal vein thrombosis, Ultraschall Med, 28, 75, 10.1055/s-2007-962847 Hom, 2006, Prospective evaluation of vascular complications after liver transplantation: comparison of conventional and microbubble contrast-enhanced US, Radiology, 241, 267, 10.1148/radiol.2411050597 Piscaglia, 2014, The role of ultrasound elastographic techniques in chronic liver disease: current status and future perspectives, Eur J Radiol, 83, 450, 10.1016/j.ejrad.2013.06.009 Silverman, 1995, Portal vein patency in candidates for liver transplantation: MR angiographic analysis, Radiology, 197, 147, 10.1148/radiology.197.1.7568813 Brancatelli, 2001, Portal venous thrombosis or sclerosis in liver transplantation candidates: preoperative CT findings and correlation with surgical procedure, Radiology, 220, 321, 10.1148/radiology.220.2.r01au23321 Bradbury, 2002, Mesenteric venous thrombosis: diagnosis and noninvasive imaging, RadioGraphics, 22, 527, 10.1148/radiographics.22.3.g02ma10527 Perri, 2008, A prospective evaluation of computerized tomographic (CT) scanning as a screening modality for esophageal varices, Hepatology, 47, 1587, 10.1002/hep.22219 Kim, 2007, Esophageal varices in patients with cirrhosis: multidetector CT esophagography—comparison with endoscopy, Radiology, 242, 759, 10.1148/radiol.2423050784 Kim, 2007, Esophageal varices in cirrhotic patients: evaluation with liver CT, Am J Roentgenol, 188, 139, 10.2214/AJR.05.1737 Kang, 2002, Three-dimensional multi-detector row CT portal venography in the evaluation of portosystemic collateral vessels in liver cirrhosis, RadioGraphics, 22, 1053, 10.1148/radiographics.22.5.g02se011053 Kim, 2000, Portosystemic collaterals of the upper abdomen: review of anatomy and demonstration on MR imaging, Abdom Imaging, 25, 462, 10.1007/s002610000014 Matsuo, 2003, Esophageal varices: diagnosis with gadolinium-enhanced MR imaging of the liver for patients with chronic liver damage, Am J Roentgenol, 180, 461, 10.2214/ajr.180.2.1800461 Sodickson, 2009, Recurrent CT, cumulative radiation exposure, and associated radiation-induced cancer risks from CT of adults, Radiology, 251, 175, 10.1148/radiol.2511081296 Thomsen, 2009, How to avoid nephrogenic systemic fibrosis: current guidelines in Europe and the United States, Radiol Clin N Am, 47, 871, 10.1016/j.rcl.2009.05.002 Shimada, 2009, Non-contrast-enhanced MR portography with time-spatial labeling inversion pulses: comparison of imaging with three-dimensional half-Fourier fast spin-echo and true steady-state free-precession sequences, J Magn Reson Imaging, 29, 1140, 10.1002/jmri.21753 Wu, 2014, Evaluations of non-contrast enhanced MR venography with inflow inversion recovery sequence in diagnosing Budd–Chiari syndrome, Clin Imaging, 38, 627, 10.1016/j.clinimag.2014.06.006 Faraoun, 2015, Budd–Chiari syndrome: a prospective analysis of hepatic vein obstruction on ultrasonography, multidetector-row computed tomography and MR imaging, Abdom Imaging, 40, 1500, 10.1007/s00261-015-0380-5 Bargalló, 2006, Sonography of Budd–Chiari syndrome, Am J Roentgenol, 187, W33, 10.2214/AJR.04.0918 Zhang, 2015, Ultrasonography and computed tomography diagnostic evaluation of Budd–Chiari syndrome based on radical resection exploration results, Ultrasound Q, 31, 124, 10.1097/RUQ.0000000000000122 Buckley, 2007, Imaging of Budd–Chiari syndrome, Eur Radiol, 17, 2071, 10.1007/s00330-006-0537-2 Miller, 1993, Budd–Chiari syndrome: imaging with pathologic correlation, Abdom Imaging, 18, 329, 10.1007/BF00201775 Noone, 2000, Budd–Chiari syndrome: spectrum of appearances of acute, subacute, and chronic disease with magnetic resonance imaging, J Magn Reson Imaging, 11, 44, 10.1002/(SICI)1522-2586(200001)11:1<44::AID-JMRI6>3.0.CO;2-O Brancatelli, 2007, Budd–Chiari syndrome: spectrum of imaging findings, Am J Roentgenol, 188, W168, 10.2214/AJR.05.0168 Camera, 2006, Triphasic helical CT in Budd–Chiari syndrome: patterns of enhancement in acute, subacute and chronic disease, Clin Radiol, 61, 331, 10.1016/j.crad.2005.12.001 Cheng, 2015, Comparative study of MRI manifestations of acute and chronic Budd–Chiari syndrome, Abdom Imaging, 40, 76, 10.1007/s00261-014-0193-y Bargalló, 2003, Sonography of the caudate vein: value in diagnosing Budd–Chiari syndrome, Am J Roentgenol, 181, 1641, 10.2214/ajr.181.6.1811641 Cai, 2015, Ultrasonographic visualization of accessory hepatic veins and their lesions in Budd–Chiari syndrome, Ultrasound Med Biol, 41, 2091, 10.1016/j.ultrasmedbio.2015.03.023 Cai, 2014, Computed tomography angiography manifestations of collateral circulations in Budd–Chiari syndrome, Exp Ther Med, 9, 399, 10.3892/etm.2014.2125 Cho, 1996, Collateral pathways in Budd–Chiari syndrome: CT and venographic correlation, Am J Roentgenol, 167, 1163, 10.2214/ajr.167.5.8911174 Vilgrain, 1999, Hepatic nodules in Budd–Chiari syndrome: imaging features, Radiology, 210, 443, 10.1148/radiology.210.2.r99fe13443 Zhou, 2000, Multiple macroregenerative nodules in liver cirrhosis due to Budd–Chiari syndrome. Case reports and review of the literature, Hepatogastroenterology, 47, 522 Brancatelli, 2002, Large regenerative nodules in Budd–Chiari syndrome and other vascular disorders of the liver, Am J Roentgenol, 178, 877, 10.2214/ajr.178.4.1780877 Tanaka, 1998, Pathology of the liver in Budd–Chiari syndrome: portal vein thrombosis and the histogenesis of veno-centric cirrhosis, veno-portal cirrhosis, and large regenerative nodules, Hepatology, 27, 488, 10.1002/hep.510270224 Brancatelli, 2002, Benign regenerative nodules in Budd–Chiari syndrome and other vascular disorders of the liver: radiologic–pathologic and clinical correlation, RadioGraphics, 22, 847, 10.1148/radiographics.22.4.g02jl17847 Maetani, 2002, Benign hepatic nodules in Budd–Chiari syndrome, Am J Roentgenol, 178, 869, 10.2214/ajr.178.4.1780869 Park, 2012, Hepatocellular carcinoma in Budd–Chiari syndrome: a single center experience with long-term follow-up in South Korea, World J Gastroenterol, 18, 1946, 10.3748/wjg.v18.i16.1946 Gwon, 2010, Hepatocellular carcinoma associated with membranous obstruction of the inferior vena cava: incidence, characteristics, and risk factors and clinical efficacy of TACE, Radiology, 254, 617, 10.1148/radiol.09090738 Renzulli, 2011, Large regenerative nodules in a patient with Budd–Chiari syndrome after TIPS positioning while on the liver transplantation list diagnosed by Gd-EOB-DTPA MRI, Hepatobiliary Pancreat Dis Int, 10, 439, 10.1016/S1499-3872(11)60075-1 Kreel, 1967, Vascular radiology in the Budd–Chiari syndrome, Br J Radiol, 40, 755, 10.1259/0007-1285-40-478-755 Krishan Dhiman, 2002, Non-cirrhotic portal fibrosis (idiopathic portal hypertension): experience with 151 patients and a review of the literature, J Gastroenterol Hepatol, 17, 6, 10.1046/j.1440-1746.2002.02596.x Dhyani, 2015, Feasibility study for assessing liver fibrosis in paediatric and adolescent patients using real-time shear wave elastography, J Med Imaging Radiat Oncol, 59, 687, 10.1111/1754-9485.12388 Okuda, 1984, Clinical study of eighty-six cases of idiopathic portal hypertension and comparison with cirrhosis with splenomegaly, Gastroenterology, 86, 600, 10.1016/S0016-5085(84)80106-7 Futagawa, 1981, Hepatic venography in noncirrhotic idiopathic portal hypertension. Comparison with cirrhosis of the liver, Radiology, 141, 303, 10.1148/radiology.141.2.7291551 Maruyama, 2012, Non-invasive portography: a microbubble-induced three-dimensional sonogram for discriminating idiopathic portal hypertension from cirrhosis, Br J Radiol, 85, 587, 10.1259/bjr/25522379 Maruyama, 2012, Delayed periportal enhancement: a characteristic finding on contrast ultrasound in idiopathic portal hypertension, Hepatol Int, 6, 511, 10.1007/s12072-011-9285-8 Maruyama, 2009, Effect of signal intensity from the accumulated microbubbles in the liver for differentiation of idiopathic portal hypertension from liver cirrhosis, Radiology, 252, 587, 10.1148/radiol.2522081899 Krishnan, 2012, Hepatoportal sclerosis: CT and MRI appearance with histopathologic correlation, Am J Roentgenol, 198, 370, 10.2214/AJR.11.6855 Arai, 1991, MR imaging in idiopathic portal hypertension, J Comput Assist Tomogr, 15, 405, 10.1097/00004728-199105000-00011 Arora, 2015, Multimodality imaging of primary extrahepatic portal vein obstruction (EHPVO): what every radiologist should know, Br J Radiol, 88 Kobayashi, 2013, Intrahepatic periportal high intensity on hepatobiliary phase images of Gd-EOB-DTPA-enhanced MRI: imaging findings and prevalence in various hepatobiliary diseases, Jpn J Radiol, 31, 9, 10.1007/s11604-012-0136-x Waguri, 2002, Heterogeneous hepatic enhancement on CT angiography in idiopathic portal hypertension, Liver Int, 22, 276, 10.1046/j.0106-9543.2002.01663.x Glatard, 2012, Obliterative portal venopathy: findings at CT imaging, Radiology, 263, 741, 10.1148/radiol.12111785 Seijo, 2012, Role of hepatic vein catheterisation and transient elastography in the diagnosis of idiopathic portal hypertension, Dig Liver Dis, 44, 855, 10.1016/j.dld.2012.05.005 Furuichi, 2013, Noninvasive diagnostic method for idiopathic portal hypertension based on measurements of liver and spleen stiffness by ARFI elastography, J Gastroenterol, 48, 1061, 10.1007/s00535-012-0703-z Abraldes, 2013, Spleen stiffness: toward a noninvasive portal sphygmomanometer?, Hepatology, 57, 1278, 10.1002/hep.26239 Kuszyk, 1998, Portal venous system thrombosis: helical CT angiography before transjugular intrahepatic portosystemic shunt creation, Radiology, 206, 179, 10.1148/radiology.206.1.9423670 Sharma, 2012, Liver and spleen stiffness in patients with extrahepatic portal vein obstruction, Radiology, 263, 893, 10.1148/radiol.12111046 Tarantino, 2015, Contrast-enhanced ultrasound in differentiating malignant from benign portal vein thrombosis in hepatocellular carcinoma, World J Gastroenterol, 21, 9457, 10.3748/wjg.v21.i32.9457 Tarantino, 2006, Diagnosis of benign and malignant portal vein thrombosis in cirrhotic patients with hepatocellular carcinoma: color Doppler US, contrast-enhanced US, and fine-needle biopsy, Abdom Imaging, 31, 537, 10.1007/s00261-005-0150-x Piscaglia, 2010, Criteria for diagnosing benign portal vein thrombosis in the assessment of patients with cirrhosis and hepatocellular carcinoma for liver transplantation, Liver Transplant, 16, 658, 10.1002/lt.22044 Cloogman, 1984, Hereditary hemorrhagic telangiectasia: sonographic findings in the liver, Radiology, 150, 521, 10.1148/radiology.150.2.6691112 Vilgrain, 1991, Doppler sonography in Osler–Weber–Rendu disease, Am J Roentgenol, 157, 413, 10.2214/ajr.157.2.1853834 Ralls, 1992, Hereditary hemorrhagic telangiectasia: findings in the liver with color Doppler sonography, Am J Roentgenol, 159, 59, 10.2214/ajr.159.1.1609722 Ravard, 2004, Hepatic involvement in hereditary hemorrhagic telangiectasia: helical computed tomography features in 24 consecutive patients, J Comput Assist Tomogr, 28, 488, 10.1097/00004728-200407000-00009 Siddiki, 2008, Abdominal findings in hereditary hemorrhagic telangiectasia: pictorial essay on 2D and 3D findings with isotropic multiphase CT, RadioGraphics, 28, 171, 10.1148/rg.281075037 Vilgrain, 1998, Images in hepatology. Hepatic pseudotumor in Osler–Weber–Rendu disease, J Hepatol, 28, 343, 10.1016/0168-8278(88)80023-0 Wanless, 1986, Nodular transformation of the liver in hereditary hemorrhagic telangiectasia, Arch Pathol Lab Med, 110, 331 Lassau, 1997, Hepatic veno-occlusive disease after myeloablative treatment and bone marrow transplantation: value of gray-scale and Doppler US in 100 patients, Radiology, 204, 545, 10.1148/radiology.204.2.9240551 Lassau, 2002, Prognostic value of doppler-ultrasonography in hepatic veno-occlusive disease, Transplantation, 74, 6, 10.1097/00007890-200207150-00011 Fontanilla, 2011, Acoustic radiation force impulse elastography and contrast-enhanced sonography of sinusoidal obstructive syndrome (veno-occlusive disease), J Ultrasound Med, 30, 1593, 10.7863/jum.2011.30.11.1593 Richardson, 1999, The pathology, diagnosis, and treatment of hepatic veno-occlusive disease: current status and novel approaches, Br J Haematol, 107, 485, 10.1046/j.1365-2141.1999.01680.x Torrisi, 2011, CT Findings of chemotherapy-induced toxicity: what radiologists need to know about the clinical and radiologic manifestations of chemotherapy toxicity, Radiology, 258, 41, 10.1148/radiol.10092129 Zhou, 2014, Hepatic sinusoidal obstruction syndrome caused by herbal medicine: CT and MRI features, Korean J Radiol, 15, 218, 10.3348/kjr.2014.15.2.218 Wu, 2012, Hepatic veno-occlusive disease after taking Gynura rhizome: the value of multidetector computed tomography in diagnosing the disease and evaluating the clinical therapeutic effect, Hepatol Res, 42, 304, 10.1111/j.1872-034X.2011.00918.x O’Rourke, 2009, Accuracy of liver-specific magnetic resonance imaging as a predictor of chemotherapy-associated hepatic cellular injury prior to liver resection, Eur J Surg Oncol, 35, 1085, 10.1016/j.ejso.2009.01.015 Yoneda, 2015, Correlation between Gd-EOB-DTPA-enhanced MR imaging findings and OATP1B3 expression in chemotherapy-associated sinusoidal obstruction syndrome, Abdom Imaging, 40, 3099, 10.1007/s00261-015-0503-z Heit, 2007, Thrombophilia: common questions on laboratory assessment and management, Hematology, 2007, 127, 10.1182/asheducation-2007.1.127 Dahlback, 2008, Advances in understanding pathogenic mechanisms of thrombophilic disorders, Blood, 112, 19, 10.1182/blood-2008-01-077909 Rosendaal, 2009, Genetics of venous thrombosis, J Thromb Haemost, 7, 301, 10.1111/j.1538-7836.2009.03394.x Janssen, 1998, Factor V Leiden mutation, prothrombin gene mutation and deficiencies in coagulation inhibitors associated with Budd–Chiari syndrome and portal vein thrombosis, Eur J Gastroenterol Hepatol, 10, A69, 10.1097/00042737-199812000-00222 Smalberg, 2006, Myeloproliferative disease in the pathogenesis and survival of Budd–Chiari syndrome, Haematologica, 91, 1712 Primignani, 2005, Risk factors for thrombophilia in extrahepatic portal vein obstruction, Hepatology, 41, 603, 10.1002/hep.20591 Smalberg, 2011, Hypercoagulability and hypofibrinolysis and risk of deep vein thrombosis and splanchnic vein thrombosis: similarities and differences, Arterioscler Thromb Vasc Biol, 31, 485, 10.1161/ATVBAHA.110.213371 Dentali, 2008, Inherited thrombophilic abnormalities and risk of portal vein thrombosis. A meta-analysis, Thromb Haemost, 99, 675, 10.1160/TH07-08-0526 Miyakis, 2006, International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS), J Thromb Haemost, 4, 295, 10.1111/j.1538-7836.2006.01753.x Martinelli, 2009, High levels of factor VIII and risk of extra-hepatic portal vein obstruction, J Hepatol, 50, 916, 10.1016/j.jhep.2008.12.020 Raffa, 2012, Hypercoagulability in patients with chronic noncirrhotic portal vein thrombosis, Clin Gastroenterol Hepatol, 10, 72, 10.1016/j.cgh.2011.09.010 Marchetti, 2008, Thrombin generation and activated protein C resistance in patients with essential thrombocythemia and polycythemia vera, Blood, 112, 4061, 10.1182/blood-2008-06-164087 Kiladjian, 2008, The impact of JAK2 and MPL mutations on diagnosis and prognosis of splanchnic vein thrombosis: a report on 241 cases, Blood, 111, 4922, 10.1182/blood-2007-11-125328 Colaizzo, 2007, A new JAK2 gene mutation in patients with polycythemia vera and splanchnic vein thrombosis, Blood, 110, 2768, 10.1182/blood-2007-05-092502 Smalberg, 2012, Myeloproliferative neoplasms in Budd–Chiari syndrome and portal vein thrombosis: a meta-analysis, Blood, 120, 4921, 10.1182/blood-2011-09-376517 Landolfi, 2008, Thrombosis in myeloproliferative disorders: pathogenetic facts and speculation, Leukemia, 22, 2020, 10.1038/leu.2008.253 Hoekstra, 2009, Paroxysmal nocturnal hemoglobinuria in Budd–Chiari syndrome: findings from a cohort study, J Hepatol, 51, 696, 10.1016/j.jhep.2009.06.019 Brodsky, 2008, Narrative review paroxysmal nocturnal hemoglobinuria: the physiology of complement-related hemolytic anemia, Ann Intern Med, 148, 587, 10.7326/0003-4819-148-8-200804150-00003 Amitrano, 2007, Prognostic factors in noncirrhotic patients with splanchnic vein thromboses, Am J Gastroenterol, 102, 2464, 10.1111/j.1572-0241.2007.01477.x Plessier, 2012, Management of hepatic vascular diseases, J Hepatol, 56, S25, 10.1016/S0168-8278(12)60004-X Tait, 2012, Guidelines on the investigation and management of venous thrombosis at unusual sites, Br J Haematol, 159, 28, 10.1111/j.1365-2141.2012.09249.x Hoekstra, 2011, Long-term follow-up of patients with portal vein thrombosis and myeloproliferative neoplasms, J Thromb Haemost, 9, 2208, 10.1111/j.1538-7836.2011.04484.x Bijnen, 2012, Possible high risk of thrombotic events in patients with paroxysmal nocturnal haemoglobinuria after discontinuation of eculizumab, Br J Haematol, 157, 762, 10.1111/j.1365-2141.2012.09073.x Plessier, 2006, Aiming at minimal invasiveness as a therapeutic strategy for Budd–Chiari syndrome, Hepatology, 44, 1308, 10.1002/hep.21354 Kearon, 2012, Antithrombotic therapy for VTE disease, Chest, 141, e419S, 10.1378/chest.11-2301 Randi, 2010, Heparin-induced thrombocytopenia in patients with Philadelphia-negative myeloproliferative disorders and unusual splanchnic or cerebral vein thrombosis, Acta Haematol, 123, 140, 10.1159/000280466 Orr, 2007, Chronic mesenteric venous thrombosis: evaluation and determinants of survival during long-term follow-up, Clin Gastroenterol Hepatol, 5, 80, 10.1016/j.cgh.2006.09.030 Kearon, 2016, Antithrombotic therapy for VTE Disease, Chest, 149, 315, 10.1016/j.chest.2015.11.026 Garcia, 2012, Parenteral anticoagulants: antithrombotic therapy and prevention of thrombosis, 9th ed: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines, Chest, 141, e24S, 10.1378/chest.11-2291 Rodriguez-Castro, 2012, Anticoagulation for the treatment of thrombotic complications in patients with cirrhosis, Liver Int, 32, 1465, 10.1111/j.1478-3231.2012.02839.x Bechmann, 2011, Dosing and monitoring of low-molecular-weight heparin in cirrhotic patients, Liver Int, 31, 1064, 10.1111/j.1478-3231.2011.02548.x Ageno, 2012, Oral anticoagulant therapy: antithrombotic therapy and prevention of thrombosis, 9th ed: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines, Chest, 141, e44S, 10.1378/chest.11-2292 Van den Besselaar, 2004, Guidelines on preparation, certification, and use of certified plasmas for ISI calibration and INR determination, J Thromb Haemost, 2, 1946, 10.1111/j.1538-7836.2004.00970.x