Idiopathic non-cirrhotic portal hypertension: A review

Orphanet Journal of Rare Diseases

Volumn 10, Issue 1, 2015, Pages

  Schouten, Jeoffrey N L ^a   Verheij, Joanne ^b   Seijo, Susana ^c  

^a GHENT UNIVERSITY HOSPITAL   (Belgium)

^b UNIVERSITY OF AMSTERDAM   (Netherlands)

^c MOUNT SINAI SCHOOL OF MEDICINE   (United States)

Author keywords

Non cirrhotic portal hypertension; Portal hypertension; Portal vein thrombosis; Variceal bleeding

Indexed keywords

ANTIBIOTIC AGENT; ANTICOAGULANT AGENT; ANTIVITAMIN K; AZATHIOPRINE; LOW MOLECULAR WEIGHT HEPARIN; ORGANOARSENIC DERIVATIVE; VASOACTIVE AGENT;

ADAMS OLIVER SYNDROME; AGE DISTRIBUTION; ANTICOAGULANT THERAPY; ANTIPHOSPHOLIPID SYNDROME; ASCITES; BLEEDING; BLOOD TRANSFUSION; CHRONIC LIVER DISEASE; COMMON VARIABLE IMMUNODEFICIENCY; COMPUTED TOMOGRAPHIC ANGIOGRAPHY; CONNECTIVE TISSUE DISEASE; CROHN DISEASE; DIFFERENTIAL DIAGNOSIS; DISEASE ASSOCIATION; DISEASE CLASSIFICATION; ENZYME DEFICIENCY; ESOPHAGUS VARICES; GENETIC PREDISPOSITION; HEPATIC VENOUS PRESSURE GRADIENT; HUMAN; HUMAN IMMUNODEFICIENCY VIRUS INFECTION; IDIOPATHIC NONCIRRHOTIC PORTAL HYPERTENSION; IMMUNOPATHOLOGY; INFECTION; INTESTINE INFECTION; LIVER BIOPSY; LIVER CIRRHOSIS; LIVER FAILURE; LIVER FIBROSIS; LIVER FUNCTION TEST; LIVER STIFFNESS; LIVER TRANSPLANTATION; LIVER VEIN THROMBOSIS; MAGNETIC RESONANCE ANGIOGRAPHY; MYELOPROLIFERATIVE NEOPLASM; PATHOPHYSIOLOGY; PHOSPHOMANNOSE ISOMERASE DEFICIENCY; PORTAL HYPERTENSION; PORTAL VEIN THROMBOSIS; PRACTICE GUIDELINE; PREVALENCE; PROGNOSIS; RANDOMIZED CONTROLLED TRIAL (TOPIC); REVIEW; RISK FACTOR; SECONDARY PREVENTION; SEX DIFFERENCE; SPLENOMEGALY; STOMACH HEMORRHAGE; SURVIVAL RATE; SYMPTOMATOLOGY; THROMBOPHILIA; THROMBOSIS; TRANSJUGULAR INTRAHEPATIC PORTOSYSTEMIC SHUNT; TURNER SYNDROME; VARICEAL BLEEDING; HYPERTENSION, PORTAL;

HUMANS; HYPERTENSION, PORTAL; LIVER CIRRHOSIS;

EID: 84931074379     PISSN: None     EISSN: 17501172     Source Type: Journal    
DOI: 10.1186/s13023-015-0288-8     Document Type: Review

References (62)

1. Schouten JNL, García-Pagán JC, Valla DC, Janssen HLA. Idiopathic noncirrhotic portal hypertension. Hepatology. 2011;54:1071-81.
2. Siramolpiwat S, Seijo S, Miquel R, Berzigotti A, García-Criado A, Darnell A, et al. Idiopathic portal hypertension: natural history and long-term outcome. Hepatology. 2014;59:2276-85.
3. Eapen CE, Nightingale P, Hubscher SG, Lane PJ, Plant T, Velissaris D, et al. Non-cirrhotic intrahepatic portal hypertension: associated Gut diseases and prognostic factors. Dig Dis Sci. 2010;56:227-35.
4. Sarin SK, Kapoor D. Non-cirrhotic portal fibrosis: current concepts and management. J Gastroenterol Hepatol. 2002;17:526-34.
5. Austin A. Nodular regenerative hyperplasia of the liver and coeliac disease: potential role of IgA anticardiolipin antibody. Gut. 2004;53:1032-4.
6. Hillaire S, Bonte E, Denninger M-H, Casadevall N, Cadranel J-F, Lebrec D, et al. Idiopathic non-cirrhotic intrahepatic portal hypertension in the West: a re-evaluation in 28 patients. Gut. 2002;51:275-80.
7. Chang P-E, Miquel R, Blanco J-L, Laguno M, Bruguera M, Abraldes JG, et al. Idiopathic portal hypertension in patients with HIV infection treated with highly active antiretroviral therapy. Am J Gastroenterol. 2009;104:1707-14.
8. Tsuneyama K, Harada K, Katayanagi K, Watanabe K, Kurumaya H, Minato H, et al. Overlap of idiopathic portal hypertension and scleroderma: report of two autopsy cases and a review of literature. J Gastroenterol Hepatol. 2002;17:217-23.
9. Schouten JNL, Van der Ende ME, Koëter T, Rossing HHM, Komuta M, Verheij J, et al. Risk factors and outcome of HIV-associated idiopathic noncirrhotic portal hypertension. Aliment Pharmacol Ther. 2012;36(9):875-85.
10. Schouten JNL, Nevens F, Hansen B, Laleman W, den Born M, Komuta M, et al. Idiopathic noncirrhotic portal hypertension is associated with poor survival: results of a long-term cohort study. Aliment Pharmacol Ther. 2012;35:1424-33.
11. Cazals-Hatem D, Hillaire S, Rudler M, Plessier A, Paradis V, Condat B, et al. Obliterative portal venopathy: portal hypertension is not always present at diagnosis. J Hepatol. 2011;54:455-61.
12. Madhu K, Avinash B, Ramakrishna B, Eapen CE, Shyamkumar NK, Zachariah U, et al. Idiopathic non-cirrhotic intrahepatic portal hypertension: common cause of cryptogenic intrahepatic portal hypertension in a Southern Indian tertiary hospital. Indian J Gastroenterol. 2009;28:83-7.
13. Okudaira M, Ohbu M, Okuda K. Idiopathic portal hypertension and its pathology. Semin Liver Dis. 2002;22(1):59-72.
14. Mallet V, Blanchard P, Verkarre V, Vallet-Pichard A, Fontaine H, Lascoux-Combe C, et al. Nodular regenerative hyperplasia is a new cause of chronic liver disease in HIV-infected patients. AIDS. 2007;21:187-92.
15. Okuda K. Non-cirrhotic portal hypertension versus idiopathic portal hypertension. J Gastroenterol Hepatol. 2002;17 Suppl 3:S204-13.
16. Mallet VO, Varthaman A, Lasne D, Viard J-P, Gouya H, Borgel D, et al. Acquired protein S deficiency leads to obliterative portal venopathy and to compensatory nodular regenerative hyperplasia in HIV-infected patients. AIDS. 2009;23:1511-8.
17. Vispo E, Moreno A, Maida I, Barreiro P, Cuevas A, Albertos S, et al. Noncirrhotic portal hypertension in HIV-infected patients: unique clinical and pathological findings. AIDS. 2010;24:1171-6.
18. Umeyama K, Yui S, Fukamizu A, Yoshikawa K, Yamashita T. Idiopathic portal hypertension associated with progressive systemic sclerosis. Am J Gastroenterol. 1982;77:645-8.
19. Carreras LO, Defreyn G, Machin SJ, Vermylen J, Deman R, Spitz B, et al. Arterial thrombosis, intrauterine death and "lupus" antiocoagulant: detection of immunoglobulin interfering with prostacyclin formation. Lancet. 1981;1:244-6.
20. Malamut G, Ziol M, Suarez F, Beaugrand M, Viallard JF, Lascaux AS, et al. Nodular regenerative hyperplasia: the main liver disease in patients with primary hypogammaglobulinemia and hepatic abnormalities. J Hepatol. 2008;48:74-82.
21. Kono K, Ohnishi K, Omata M, Saito M, Nakayama T, Hatano H, et al. Experimental portal fibrosis produced by intraportal injection of killed nonpathogenic Escherichia coli in rabbits. Gastroenterology. 1988;94:787-96.
22. Maida I, Garcia-Gasco P, Sotgiu G, Ríos MJ, Vispo ME, Martín-Carbonero L, et al. Antiretroviral-associated portal hypertension: a new clinical condition? Prevalence, predictors and outcome. Antivir Ther. 2008;13:103-7.
23. Saifee S, Joelson D, Braude J, Shrestha R, Johnson M, Sellers M, et al. Noncirrhotic portal hypertension in patients with human immunodeficiency virus-1 infection. Clin Gastroenterol Hepatol. 2008;6:1167-9.
24. Martinez-Palli G, Drake BB, García-Pagán JC, Barbera J-A, Arguedas MR, Rodriguez-Roisin R, et al. Effect of transjugular intrahepatic portosystemic shunt on pulmonary gas exchange in patients with portal hypertension and hepatopulmonary syndrome. World J Gastroenterol. 2005;11:6858-62.
25. Vispo E, Cevik M, Rockstroh JK, Barreiro P, Nelson M, Scourfield A, et al. Genetic determinants of idiopathic noncirrhotic portal hypertension in HIV-infected patients. Clin Infect Dis. 2013;56:1117-22.
26. de Boer NKH, Tuynman H, Bloemena E, Westerga J, Van Der Peet DL, Mulder CJJ, et al. Histopathology of liver biopsies from a thiopurine-naïve inflammatory bowel disease cohort: prevalence of nodular regenerative hyperplasia. Scand J Gastroenterol. 2008;43:604-8.
27. Vernier-Massouille G, Cosnes J, Lemann M, Marteau P, Reinisch W, Laharie D, et al. Nodular regenerative hyperplasia in patients with inflammatory bowel disease treated with azathioprine. Gut. 2007;56:1404-9.
28. Nevens F, Fevery J, Van Steenbergen W, Sciot R, Desmet V, De Groote J. Arsenic and non-cirrhotic portal hypertension. A report of eight cases. J Hepatol. 1990;11:80-5.
29. Moran CA, Mullick FG, Ishak KG. Nodular regenerative hyperplasia of the liver in children. Am J Surg Pathol. 1991;15:449-54.
30. Roulot D. Liver involvement in Turner syndrome. Liver Int. 2012;33:24-30.
31. Sarin SK, Mehra NK, Agarwal A, Malhotra V, Anand BS, Taneja V. Familial aggregation in noncirrhotic portal fibrosis: a report of four families. Am J Gastroenterol. 1987;82:1130-3.
32. Girard M, Amiel J, Fabre M, Pariente D, Lyonnet S, Jacquemin E. Adams-Oliver syndrome and hepatoportal sclerosis: occasional association or common mechanism? Am J Med Genet. 2005;135A:186-9.
33. Barnett JL, Appelman HD, Moseley RH. A familial form of incomplete septal cirrhosis. Gastroenterology. 1992;102:674-8.
34. Sarin SK, Khanna R. Non-cirrhotic portal hypertension. Clin Liver Dis. 2014;18:451-76.
35. Verheij J, Schouten JNL, Komuta M, Nevens F, Hansen BE, Janssen HLA, et al. Histological features in western patients with idiopathic non-cirrhotic portal hypertension. Histopathology. 2013;62:1083-91.
36. Wanless IR, Bernier V, Seger M. Intrahepatic portal vein sclerosis in patients without a history of liver disease. Am J Pathol. 1982;106(1):63-70.
37. Okuda K, Kono K, Ohnishi K, Kimura K, Omata M, Koen H, et al. Clinical study of eighty-six cases of idiopathic portal hypertension and comparison with cirrhosis with splenomegaly. Gastroenterology. 1984;86:600-10.
38. Nakanuma Y, Hoso M, Sasaki M, Terada T, Katayanagi K, Nonomura A, et al. Histopathology of the liver in non-cirrhotic portal hypertension of unknown aetiology. Histopathology. 1996;28:195-204.
39. Khanna R, Sarin SK. Non-cirrhotic portal hypertension - Diagnosis and management. J Hepatol. 2014;60:421-41.
40. Ziol M, Poirel H, Kountchou GN, Boyer O, Mohand D, Mouthon L, et al. Intrasinusoidal cytotoxic CD8+ T cells in nodular regenerative hyperplasia of the liver. Hum Pathol. 2004;35:1241-51.
41. Ohnishi K, Saito M, Sato S, Terabayashi H, Iida S, Nomura F, et al. Portal hemodynamics in idiopathic portal hypertension (Banti's syndrome). Comparison with chronic persistent hepatitis and normal subjects. Gastroenterology. 1987;92:751-8.
42. Blendis LM, Banks DC, Ramboer C, Williams R. Spleen blood flow and splanchnic haemodynamics in blood dyscrasia and other splenomegalies. Clin Sci. 1970;38:73-84.
43. Sato Y, Sawada S, Kozaka K, Harada K, Sasaki M, Matsui O, et al. Significance of enhanced expression of nitric oxide syntheses in splenic sinus lining cells in altered portal hemodynamics of idiopathic portal hypertension. Dig Dis Sci. 2007;52:1987-94.
44. Berzigotti A, Seijo S, Reverter E, Bosch J. Assessing portal hypertension in liver diseases. Expert Rev Gastroenterol Hepatol. 2013;7:141-55.
45. Krasinskas AM, Eghtesad B, Kamath PS, Demetris AJ, Abraham SC. Liver transplantation for severe intrahepatic noncirrhotic portal hypertension. Liver Transpl. 2005;11:627-34.
46. Wanless IR, Nakashima E, Sherman M. Regression of human cirrhosis. Morphologic features and the genesis of incomplete septal cirrhosis. Arch Pathol Lab Med. 2000;124:1599-607.
47. Verrijken A, Francque S, Mertens I, Prawitt J, Caron S, Hubens G, et al. Prothrombotic factors in histologically proven nonalcoholic fatty liver disease and nonalcoholic steatohepatitis. Hepatology. 2013;59:121-9.
48. Bioulac-Sage P, Le Bail B, Bernard PH, Balabaud C. Hepatoportal sclerosis. Semin Liver Dis. 1995;15:329-39.
49. Wanless IR. Micronodular transformation (nodular regenerative hyperplasia) of the liver: a report of 64 cases among 2500 autopsies and a new classification of benign hepatocellular nodules. Hepatology. 1990;11:787-97.
50. Sarin SK, Sethi KK, Nanda R. Measurement and correlation of wedged hepatic, intrahepatic, intrasplenic and intravariceal pressures in patients with cirrhosis of liver and non-cirrhotic portal fibrosis. Gut. 1987;28(3):260-6.
51. Seijo S, Reverter E, Miquel R, Berzigotti A, Abraldes JG, Bosch J, et al. Role of hepatic vein catheterisation and transient elastography in the diagnosis of idiopathic portal hypertension. Dig Liver Dis. 2012;44:855-60.
52. Chang P-E, Garcia-Pagan J. Causes of Noncirrhotic Portal Hypertension. Up to date; 2014:1-28.
53. Bihl F, Janssens F, Boehlen F, Rubbia-Brandt L, Hadengue A, Spahr L. Anticoagulant therapy for nodular regenerative hyperplasia in a HIV-infected patient. BMC Gastroenterol. 2010;10:6.
54. Fuss IJ, Friend J, Yang Z, He JP, Hooda L, Boyer J, et al. Nodular regenerative hyperplasia in common variable immunodeficiency. J Clin Immunol. 2013;33:748-58.
55. Vaiphei K, Bhatia A, Sinha SK. Liver pathology in collagen vascular disorders highlighting the vascular changes within portal tracts. Indian J Pathol Microbiol. 2011;54:25-31.
56. Allison MC, Mowat A, McCruden EA, McGregor E, Burt AD, Briggs JD, et al. The spectrum of chronic liver disease in renal transplant recipients. Q J Med. 1992;83:355-67.
57. Gane E, Portmann B, Saxena R, Wong P, Ramage J, Williams R. Nodular regenerative hyperplasia of the liver graft after liver transplantation. Hepatology. 1994;20:88-94.
58. Calabrese E, Hanauer SB. Assessment of non-cirrhotic portal hypertension associated with thiopurine therapy in inflammatory bowel disease. J Crohns Colitis. 2011;5:48-53.
59. Geubel AP, De Galocsy C, Alves N, Rahier J, Dive C. Liver damage caused by therapeutic vitamin A administration: estimate of dose-related toxicity in 41 cases. Gastroenterology. 1991;100:1701-9.
60. de Lonlay P, Seta N. The clinical spectrum of phosphomannose isomerase deficiency, with an evaluation of mannose treatment for CDG-Ib. Biochim Biophys Acta. 1792;2009:841-3.
61. Franchi-Abella S, Fabre M, Mselati E, De Marsillac ME, Bayari M, Pariente D, et al. Obliterative portal venopathy: a study of 48 children. J Pediatr. 2014;165:190-3. e2.
62. Bayan K, Tüzün Y, Yilmaz Ş, Canoruc N, Dursun M. Analysis of inherited thrombophilic mutations and natural anticoagulant deficiency in patients with idiopathic portal hypertension. J Thromb Thrombolysis. 2008;28:57-62.