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Noncirrhotic portal fibrosis/idiopathic portal hypertension: APASL recommendations for diagnosis and treatment

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Abstract

The Asian Pacific Association for the Study of the Liver (APASL) Working Party on Portal Hypertension has developed consensus guidelines on the disease profile, diagnosis, and management of noncirrhotic portal fibrosis and idiopathic portal hypertension. The consensus statements, prepared and deliberated at length by the experts in this field, were presented at the annual meeting of the APASL at Kyoto in March 2007. This article includes the statements approved by the APASL along with brief backgrounds of various aspects of the disease.

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References

  1. de Franchis R. Evolving consensus in portal hypertension. Report of the Baveno IV consensus workshop on methodology of diagnosis and therapy in portal hypertension. J Hepatol 2005;43:167–76.

    PubMed  Google Scholar 

  2. Sarin SK, Sollano JD, Chawla YK, Amarapurkar D, Hamid S, Hashizume M et al. Members of the APASL Working Party on Portal Hypertension. Consensus on extra-hepatic portal vein obstruction. Liver Int 2006;26:512–9.

    PubMed  Google Scholar 

  3. Centre for Evidence Based Medicine. Department of Primary Care, Old Road Campus, Headington, Oxford, OX3 7LF, UK. http://www.cebm.net/index.aspx?o=1025.

  4. Mikkelsen WP, Edmondson HA, Peters RL, Redeker AG, Reynolds TB. Extra- and intra-hepatic portal hypertension without cirrhosis (hepatoportal sclerosis). Ann Surg 1965;162:602–20.

    Article  PubMed  CAS  Google Scholar 

  5. Bioulac-Sage, Le Bail B, Bernard PH, Balabaud C. Hepatoportal sclerosis. Semin Liver Dis 1995;15:329–39.

    PubMed  CAS  Google Scholar 

  6. Kingham JGC, Levison DA, Stansfeld AG, Dawson AM. Non-cirrhotic intrahepatic portal hypertension: a long term follow-up study. Q J Med 1981;50:259–68.

    PubMed  CAS  Google Scholar 

  7. Hillaire S, Bonte E, Denninger MH, Casadevall N, Cadranel JF, Lebrec D et al. Idiopathic non-cirrhotic intrahepatic portal hypertension in the West: a re-evaluation in 28 patients. Gut 2002;51:275–80.

    PubMed  CAS  Google Scholar 

  8. Dhiman RK, Chawla Y, Vasishta RK, Kakkar N, Dilawari JB, Trehan MS et al. Non-cirrhotic portal fibrosis (idiopathic portal hypertension): experience with 151 patients and a review of the literature. J Gastroenterol Hepatol 2002;17:6–16.

    PubMed  Google Scholar 

  9. Sarin SK. Non-cirrhotic portal hypertension. In: Advances in therapeutic hepatology: a world view. Postgraduate Course, AASLD 1998.

  10. Pande C, Kumar A, Sarin SK. Non-cirrhotic portal fibrosis: a clinical profile of 366 patients [abstract]. Am J Gastroenterol 2006;101:S191.

    Google Scholar 

  11. Okuda K. Non-cirrhotic portal hypertension: why is it so common in India? J Gastroenterol Hepatol 2002;17:1–5.

    PubMed  Google Scholar 

  12. Imanaga H, Yamamoto S, Kuroyanagi Y. Surgical treatment of portal hypertension according to state of intrahepatic circulation. Ann Surg 1962;155:43–50.

    Google Scholar 

  13. Kobayashi Y, Inokuchi K, Saku M et al. Epidemiology and clinical features of idiopathic portal hypertension. 1975 Report of the Ministry of Health and Welfare Research Committee on Idiopathic Portal Hypertension. Tokyo: Ministry of Health and Welfare, 1975:10–13.

  14. Iwata H, Nishikawa A, Tanaka H et al. National survey on idiopathic portal hypertension. In: Kameda H, editor. 1985 Report of the Research Committee on Aberrant Portal Hemodynamics. Tokyo: Ministry of Health and Welfare; 1986. p. 117–29.

  15. Imai F, Kuga K, Komaba M et al. Interim report on IPH survey. In: Futagawa S, editor. 1992 Report of the Research Committee on Aberrant Portal Hemodynamics. Tokyo: Ministry of health and Welfare; 1993. p. 107–10.

  16. Bhargava DK, Dasarathy S, Sundaram KR, Ahuja RK. Efficacy of endoscopic sclerotherapy on long-term management of esophageal varices: a comparative study of results in patients with cirrhosis of the liver, non-cirrhotic portal fibrosis (NCPF) and extra hepatic portal venous obstruction (EHO). J Gastroenterol Hepatol 1991;6:471–5.

    PubMed  CAS  Google Scholar 

  17. Banghar PK, Abraham P, Mistry FP, Bhatia SJ, Modi A. Profile of portal hypertension in Bombay. Indian J Gastroenterol 1992;11(Suppl 1):A25.

    Google Scholar 

  18. Abraham P, Malkan GH, Bhatia SJ, Parenjape AY, Nagral A, Mistry FP. Non-cirrhotic portal fibrosis in Bombay. Indian J Gastroenterol 1995;14(Suppl 1):A97.

    Google Scholar 

  19. Habibullah CM, Rao GN, Murthy DK, Padmanabhan CG, Kumar N, Chandra V et al. Non-cirrhotic portal fibrosis in Andhra Pradesh. (Clinical, radiological, biochemical, hemodynamic, and histological aspects). J Assoc Physicians India 1978;26:379–82.

    PubMed  CAS  Google Scholar 

  20. Okudaira M, Ohbu M, Okuda K. Idiopathic portal hypertension and its pathology. Semin Liver Dis 2002;22:59–72.

    PubMed  Google Scholar 

  21. Dhawan PS, Shah SS, Alvares JF, Kher A, Shankaran, Kandoth PW et al. Seroprevalence of hepatitis A virus in Mumbai, and immunogenicity and safety of hepatitis A vaccine. Indian J Gastroenterol 1998;17:16–8.

    PubMed  CAS  Google Scholar 

  22. Mall ML, Rai RR, Philip M, Naik G, Parekh P, Bhawnani SC et al. Seroepidemiology of hepatitis A infection in India: changing pattern. Indian J Gastroenterol 2001;20(4):132–5.

    PubMed  CAS  Google Scholar 

  23. Marra F, Valente AJ, Grandaliano G, Abboud HE. Thrombin stimulates proliferation of fat storing cells and expression of monocyte chemotactic protein-1. Hepatology 1995;22:780–7.

    PubMed  CAS  Google Scholar 

  24. Boyer JL. Non cirrhotic portal hypertension. In: Hoofnagle JH, Goodman editors. Liver biopsy. Interpretation for the 1990s; Clinicopathologic correlation in liver disease. Thorofare, NJ: Slack/AASLD; 1991. p. 428–39.

    Google Scholar 

  25. Wanless IR. Non-cirrhotic portal hypertension and nodular transformation (nodular regenerative hyperplasia). In: Hoofnagle JH, Goodman editors Liver Biopsy. Interpretation for the 1990s; Clinicopathologic correlation in liver disease. Thorofare, NJ: Slack/AASLD; 1991. p. 440–55.

    Google Scholar 

  26. Abernathy C, Liu YP, Longfellow D, Aposhian HV, Beck B, Fowler B et al. Arsenic: health effects, mechanisms of actions and research issues. Environ Health Perspective 1999;107:593–7.

    CAS  Google Scholar 

  27. Dutta DV, Mitra SK, Chhuttani PN, Chakravarti RN. Chronic oral arsenic intoxication as a possible aetiological factor in idiopathic portal hypertension (non cirrhotic portal fibrosis) in India. Gut 1979;20:378–84.

    Google Scholar 

  28. Guha Mazumder DN, Das Gupta J, Santra A, Pal A, Ghose A, Sarkar S. Chronic arsenic toxicity in West Bengal – the worst calamity in the world. J Indian Med Assoc 1998;96:4–7.

    Google Scholar 

  29. Santra A, Das Gupta JD, De B, Roy B, Guha Mazumder DN. Hepatic manifestations in chronic arsenic toxicity. Indian J Gastroenterol 1999;18:152–5.

    PubMed  CAS  Google Scholar 

  30. Morris JS, Schmid M, Newman S, Scheuer PJ, Sherlock S. Arsenic and non cirrhotic portal hypertension. Gastroenterology 1974;66:86–94.

    PubMed  CAS  Google Scholar 

  31. Huet PM, Guillaume E, Cote J, Legare A, Lavoie P, Viallet A. Noncirrhotic presinusoidal portal hypertension associated with chronic arsenical intoxication. Gastroenterology 1975;68:1270–7.

    PubMed  CAS  Google Scholar 

  32. Straub AC, Stolz DB, Ross MA, Hernandez-Zavala A, Soucy NV, Klei LR. Arsenic stimulates sinusoidal endothelial cell capillarization and vessel remodeling in mouse liver. Hepatology 2007;45:205–12.

    PubMed  CAS  Google Scholar 

  33. Nevens F, Fevery J, Steenbergen WV, Sciot R, Desmet V, De Groote J. Arsenic and non-cirrhotic portal hypertension. J Hepatol 1990;11:80–85.

    PubMed  CAS  Google Scholar 

  34. Santra A, Maiti A, Das S, Lahiri S, Charkaborty SK, Mazumder DN. Hepatic damage caused by arsenic (As) toxicity in experimental animals. Clinical Toxicology 2000;38:395–405.

    PubMed  CAS  Google Scholar 

  35. Das S, Santra A, Lahiri S, Guha Mazumder DN. Implications of oxidative stress and hepatic cytokine (TNF-a and IL-6) response in the pathogenesis of hepatic collagenesis in chronic arsenic toxicity. Toxicol Appl Pharmacol 2005;204:18–26.

    PubMed  CAS  Google Scholar 

  36. Sakurai T, Kaise T, Matsubara C. Inorganic and methylated arsenic compounds induced cell death in murine macrophages via different mechanisms. Chem Res Toxicol 1998;11:273–83.

    PubMed  CAS  Google Scholar 

  37. Guha Mazumder DN, Ghose S, Das K, Ghosh A, Ghose KK, Nag S. Immunological studies in cirrhotic and non-cirrhotic portal fibrosis. Indian J Med Res 1986;84:59–61.

    PubMed  CAS  Google Scholar 

  38. Nayyar AK, Sharma BK, Sarin SK, Malhotra P, Broor SL, Sachdev G. Characterization of peripheral blood lymphocytes in patients with non-cirrhotic portal fibrosis: A comparison with cirrhotic and healthy controls. J Gastroenterol Hepatol 1990;5:554–9.

    PubMed  CAS  Google Scholar 

  39. Tokushige K, Yamauchi K, Komatsu T, Takasaki K, Hayashi N. Predominant T helper 1 cells in patients with idiopathic portal hypertension. J Gastroenterol Hepatol 2000;15:1312–7.

    PubMed  CAS  Google Scholar 

  40. Yamaguchi N, Tokushige K, Haruta I, Yamauchi K, Hayashi N. Analysis of adhesion molecules in patients with idiopathic portal hypertension. J Gastroenterol Hepatol 1999;14:364–9.

    PubMed  CAS  Google Scholar 

  41. Keane HM, Sheron N, Goka J, Hughes RD, Williams R. Plasma inhibitory activity against tumor necrosis factor in fulminant failure. Clin Sci 1996;90:77–80.

    PubMed  CAS  Google Scholar 

  42. Miyazaki Y, Araki C, Vesin I, Garcia I, Kapanci Y, Whitsett JA et al. Expression of a tumor necrosis factor alpha transgene in murine lung causes lymphocytic and fibrosing alveolitis: A mouse model of progressive pulmonary fibrosis. J Clin Invest 1996;96:250–9.

    Article  Google Scholar 

  43. Albini B, Ito S, Brentjens J, Andres G. Splenomegaly and immune complex splenitis in rabbits with experimentally induced chronic serum sickness: immunopathological findings. J Reticuloendothel Soc 1983;34:485–500.

    PubMed  CAS  Google Scholar 

  44. 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.

    PubMed  CAS  Google Scholar 

  45. Shekhar KC, Pathmanathan R. Hepatosplenic lesions induced by experimental Schistosoma malayensis in rabbits. Southeast Asian J Trop Med Public Health 1993;24:333–9.

    PubMed  CAS  Google Scholar 

  46. Cheever AW, Duvall RH, Minker RG, Nash TE. Hepatic fibrosis in rabbits infected with Japanese and Philippine strains of Schistosoma japonicum. Am J Trop Med Hyg 1980;29:1327–39.

    PubMed  CAS  Google Scholar 

  47. Villeneuve JP, Huet PM, Joly JG, Marleau D, Cote J, Legare A et al. Idiopathic portal hypertension. Am J Med 1976;61:459–64.

    PubMed  CAS  Google Scholar 

  48. Narang AP. Arsenicosis in India. J Toxicol Clin Toxicol 1987;25:287–95.

    PubMed  CAS  Google Scholar 

  49. 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.

    CAS  Google Scholar 

  50. Sarin SK, Sharma G, Banerjee S, Kathayat R, Malhotra V. Hepatic fibrogenesis using chronic arsenic ingestion: studies in a murine model. Indian J Exp Biol 1999;37:147–51.

    PubMed  CAS  Google Scholar 

  51. Kathayat R, Pandey GK, Malhotra V, Omanwar S, Sharma BK, Sarin SK. Rabbit model of non-cirrhotic portal fibrosis with repeated immunosensitization by rabbit splenic extract. J Gastroenterol Hepatol 2002;17:1312–6.

    PubMed  CAS  Google Scholar 

  52. Omanwar S, Rizvi MR, Kathayat R, Sharma BK, Pandey GK, Alam MA et al. A rabbit model of non-cirrhotic portal hypertension by repeated injections of E. coli through indwelling cannulation of the gastrosplenic vein. Hepatobiliary Pancreat Dis Int 2004;3:417–22.

    PubMed  Google Scholar 

  53. Vasia M, Curciorello JO, Corron SF, Viola M, Zamboni E, Castagno M et al. Idiopathic portal hypertension with splenic infarct. An unreported complication. Acta Gastroenterol Latinoam 2001;31:27–30.

    Google Scholar 

  54. Soma J, Saieto T, Sato H, Ootaka T, Abe Ke. Membranoproliferative glomerulonephritis induced by portosystemic shunt surgery for non-cirrhotic portal hypertension. Clin Nephrol 1997;48:274–81.

    Google Scholar 

  55. Kumar A, Bhuyan UN, Nundy S. Glomerulonephritis complicating non-cirrhotic portal fibrosis. J Gastroenterol Hepatol 1998;13(Suppl 1):271–5.

    Google Scholar 

  56. Babbs C, Warnes TW, Haboubi NY. Non-cirrhotic portal hypertension with hypoxaemia. Gut 1998;29:129–31.

    Google Scholar 

  57. Rangari M, Sinha S, Kapoor D, Mohan JC, Sarin SK. Prevalence of autonomic dysfunction in cirrhotic and noncirrhotic portal hypertension. Am J Gastroenterol 2002;97:707–13.

    PubMed  Google Scholar 

  58. Qureshi H, Zuberi SJ, Maher M. Idiopathic portal hypertension an overlooked entity. Hepatol Res 1998; 12:169–76.

    Google Scholar 

  59. Qureshi H. Diagnosis and management of portal hypertension [thesis]. Pakistan: Karachi University; 1996.

  60. Okuda K, Nakshima T, Kameda H, Sugirua M, Ohinshi K, Kobyashi M. Idiopathic noncirrhotic portal hypertension: a national study, Ministry of Health and Welfare Research Committee on Idiopathic Portal Hypertension. In: Brunner H, Thaler H, editors. Hepatology: a festschrift for Hans Popper. New York: Raven; 1985.

  61. Okuda K, Nakashima T, Kameda H et al., for the Japan Ministry of Heath and Welfare Research Committee on Idiopathic Portal Hypertension. Idiopathic noncirrhotic portal hypertension: a national study. In: Brunner H, Thaler H, editors. Hepatology: a festschrift for Hans Popper. New York: Raven; 1985. p. 95–108.

  62. Okuda K, Obata H. Idiopathic portal hypertension (hepatoportal sclerosis). In: Okuda K, Benhamou JP editors. Portal hypertension: clinical and physiological aspects. Tokyo: Springer-Verlag; 1991. p. 271–87.

    Google Scholar 

  63. Sarin SK, Kapoor D. Non-cirrhotic portal fibrosis: Current concept and management. J Gastroenterol Hepatol 2002;17:526–34.

    PubMed  CAS  Google Scholar 

  64. Ishii M, Katada Y. Idiopathic portal hypertension in a systemic sclerosis patient heterozygous for factor V Leiden mutation. Rheumatol Int 2003;23:44–6.

    PubMed  Google Scholar 

  65. Okuda K, Nakashima T, Okudaira M, Kage M, Aida Y, Omata M et al. Liver pathology of idiopathic portal hypertension. Comparison with non-cirrhotic portal fibrosis of India. The Japan idiopathic portal hypertension study. Liver 1982;2:176–92.

    PubMed  CAS  Google Scholar 

  66. Okudaira M, Ohbu M, Okuda K. Idiopathic portal hypertension and its pathology. Semin Liver Dis 2002;22:59–72.

    PubMed  Google Scholar 

  67. Kage M, Arakawa M, Fukuda K, Kojiro M. Pathomorphologic study on the extrahepatic portal vein in idiopathic portal hypertension. Liver 1990;10:209–16.

    PubMed  CAS  Google Scholar 

  68. Nayak NC, Ramalingaswamy V. Obliterative portal venopathy of the liver. Arch Pathol 1979;87:359–69.

    Google Scholar 

  69. Fukuda K, Kage M, Arakawa M, Nakashima T. Portal vein or hepatic vein? A curious aberrant vasculature in the liver with idiopathic portal hypertension. Acta Pathol Jpn 1985;35:885–97.

    PubMed  CAS  Google Scholar 

  70. Bredfeldt JE, Enriquez RE, Groszmann RJ. Idiopathic portal hypertension in a renal transplant recipient. J Clin Gastroenterol 1982;4:157–61.

    PubMed  CAS  Google Scholar 

  71. Tsuneyama K, Kouda W, Nakanuma Y. Portal and parenchymal alterations of the liver in idiopathic portal hypertension: a histological and immunochemical study. Pathol Res Pract 2002;198:597–603.

    PubMed  Google Scholar 

  72. Ludwig J, Hashimoto E, Obata H, Baldus WP. Idiopathic portal hypertension; a histopathological study of 26 Japanese cases. Histopathology 1993;22:227–34.

    PubMed  CAS  Google Scholar 

  73. Sciot R, Staessen D, Van Damme B, Van Steenbergen W, Fevery J, De Groote J et al. Incomplete septal cirrhosis: histopathological aspects. Histopathology 1988;13:593–603.

    Article  PubMed  CAS  Google Scholar 

  74. Okuda K, Nakashima T, Kojiro M, Kondo Y, Wada K. Hepatocellular carcinoma without cirrhosis in Japanese patients. Gastroenterology 1989;97:140–6.

    PubMed  CAS  Google Scholar 

  75. Hidaka H, Ohbu M, Kokubu S, Shibuya A, Saigenji K, Okayasu I. Hepatocellular carcinoma associated with idiopathic portal hypertension: review of large nodules in seven non-cirrhotic portal hypertensive livers. J Gastroenterol Hepatol 2005;20:493–4.

    PubMed  Google Scholar 

  76. Nakanuma Y, Kouda W, Nakano T, Uneno K, Tachibana S, Araki I. A case report of early idiopathic portal hypertension. Pathol Res Pract 2001;197:759–63.

    PubMed  CAS  Google Scholar 

  77. Nakanuma Y, Tsuneyama K, Ohbu M, Katayanagi K. Pathology and pathogenesis of idiopathic portal hypertension with an emphasis on the liver. Pathol Res Pract 2001;197:65–76.

    PubMed  CAS  Google Scholar 

  78. Sarin SK, Aggarwal SR. Idiopathic portal hypertension. Digestion 1998;59:420–3.

    PubMed  CAS  Google Scholar 

  79. Bajaj JS, Bhattacharjee J, Sarin SK. Coagulation profile and platelet function in patients with extrahepatic portal vein obstruction and non-cirrhotic portal fibrosis. J Gastroenterol Hepatol 2001;16:641–6.

    PubMed  CAS  Google Scholar 

  80. Prasad CV, Kaur U, Marwaha N, Ghosh K, Chawla YK, Dilawari JB. Hemostatic alterations in non-cirrhotic portal fibrosis, extrahepatic portal venous obstruction and Budd–Chiari syndrome. Indian J Gastroenterol 1990;9:57–60.

    PubMed  CAS  Google Scholar 

  81. Matsutani S, Maruyama H, Akiike T, Kobayashi S, Yoshizumi H, Okugawa H et al. Study of portal vein thrombosis in patients with idiopathic portal hypertension in Japan. Liver Int 2005;25:978–83.

    PubMed  Google Scholar 

  82. Nakanuma Y, Nonomura A, Hayashi M, Doishita K, Takayanagi N, Uchida T et al. Pathology of the liver in “idiopathic portal hypertension” associated with autoimmune disease. The Ministry of Health and Welfare Disorders of Portal Circulation Research Committee. Acta Pathol Jpn 1989;39:586–92.

    PubMed  CAS  Google Scholar 

  83. Saito K, Nakanuma Y, Takegoshi K, Ohta G, Obata Y, Okuda K et al. Non-specific immunological abnormalities and association of autoimmune diseases in idiopathic portal hypertension. A study by questionnaire. Hepatogastroenterology 1993;40:163–6.

    CAS  Google Scholar 

  84. Taneja V, Mehra NK, Sarin SK, Sharma BK, Vaidya MC. Possible HLA influence in governing susceptibility to non-cirrhotic portal fibrosis. Tissue Antigens 1987;30:184–7.

    Article  PubMed  CAS  Google Scholar 

  85. Terada T, Nakanuma Y, Obata H. HLA-DR expression on the microvasculature of portal tracts in idiopathic portal hypertension. Immunohistochemical characteristics and relation to portal phlebosclerosis. Arch Pathol Lab Med 1991;115:993–7.

    PubMed  CAS  Google Scholar 

  86. Sarin SK, Nayyar AK, Malhotra P, Sharma BK, Kumar R, Broor SL. Immunological profile of patients with non-cirrhotic portal fibrosis. Gastroenterol Hepatol 1990;5:425–31.

    CAS  Google Scholar 

  87. Yamaue H, Tanimura H, Iwahashi M, Tsunoda T, Tani M, Tamai M et al. Impairment of autologous mixed lymphocyte reaction in the spleen and peripheral blood lymphocytes of patients with idiopathic portal hypertension. Gastroenterol Jpn 1990;25:193–8.

    PubMed  CAS  Google Scholar 

  88. Tokushige K, Komatsu T, Ohzu K, Yamauchi K, Obata H. A defective autologous mixed lymphocyte reaction in patients with idiopathic portal hypertension. J Gastroenterol Hepatol 1992;7:270–3.

    PubMed  CAS  Google Scholar 

  89. Tokushige K, Yamauchi K, Komatsu T, Takasaki K, Hayashi N. Predominant T helper 1 cells in patients with idiopathic portal hypertension. J Gastroenterol Hepatol 2000;15:1312–7.

    PubMed  CAS  Google Scholar 

  90. Walker RM. Treatment of portal hypertension in children. Proc R Soc Med 1962;55:770–2.

    PubMed  CAS  Google Scholar 

  91. Amarapurkar DN, Dhawan PS, Chopra K, Shankaran K, Kalro RH. Stomach in portal hypertension. J Assoc Physicians India 1993;41:638–40.

    PubMed  CAS  Google Scholar 

  92. Shah SR, Desai CS, Mathur SK. Incidence and fate of antral varices. Eur J Gastroenterol Hepatol 1999;11:1041–3.

    PubMed  CAS  Google Scholar 

  93. Chawla Y, Dilawari JB. Anorectal varices—their frequency in cirrhotic and non-cirrhotic portal hypertension. Gut 1991;32:309–11.

    PubMed  CAS  Google Scholar 

  94. Dilawari JB, Kaur U, Narayanan VA, Augustine P, Das J, Ali H, Bambery P. Pattern of upper gastrointestinal hemorrhage in northern India: an endoscopic study of 316 patients. J Gastroenterol Hepatol 1987;2:443–449.

    Google Scholar 

  95. Sarin SK, Sethi KK, Nanda R. Measurement and correlation of wedged hepatic, intrahepatic, intrasplenic and intravariceal pressure in patients with cirrhosis of liver and non-cirrhotic portal fibrosis. Gut 1987;28:260–6.

    PubMed  CAS  Google Scholar 

  96. El Atti EA, Nevens F, Bogaerts K, Verbeke G, Fevery J. Variceal pressure is a strong predictor of variceal hemorrhage in patients with cirrhosis as well as in patients with non-cirrhotic portal hypertension. Gut 1999;45:618–21.

    Article  PubMed  CAS  Google Scholar 

  97. Gow PJ, Chapman RW. Modern management of esophageal varices. Postgrad Med J 2001;77:75–81.

    PubMed  CAS  Google Scholar 

  98. Zaman A, Chalasani N. Bleeding caused by portal hypertension. Gastroenterol Clin North Am 2005;34:623–42.

    PubMed  Google Scholar 

  99. Grace ND, Groszmann RJ, Garcia-Tsao G, Burroughs AK, Pagliaro L, Makuch RW. Portal hypertension and variceal bleeding: an AASLD single topic symposium. Hepatology 1998;28:868–80.

    PubMed  CAS  Google Scholar 

  100. Laine L, Planas R, Nevens F, Banares R, Patch D, Bosch J. Treatment of the acute bleeding episode. In: de Franchis R (editor) Portal hypertension IV. Blackwell Publishing, 2006. p. 217–42.

  101. Goulis J, Patch D, Burroughs AK. Bacterial infection in the pathogenesis of variceal hemorrhage. Lancet 1999;353:139–42.

    PubMed  CAS  Google Scholar 

  102. Bernard B, Cadranell JF, Valla D, Escolano S, Jarlier V, Opolon P. Prognostic significance of bacterial infection in bleeding cirrhotic patients: a prospective study. Gastroenterology 1995;108:1828–34.

    PubMed  CAS  Google Scholar 

  103. Bernard B, Nguyen KE, Opolon P, Amiot X, Opolon P, Poynard T. Antibiotic prophylaxis (ABP) for the prevention of bacterial infections in cirrhotic patients with gastrointestinal bleeding (GB): a meta-analysis. Hepatology 1999;29:1655–61.

    PubMed  CAS  Google Scholar 

  104. Hou MC, Lin HC, Liu TT, Kuo BI, Lee FY, Chang FY et al. Antibiotic prophylaxis after endoscopic therapy prevents rebleeding in acute variceal hemorrhage: a randomized trial. Hepatology 2004;39:746–53.

    PubMed  CAS  Google Scholar 

  105. D’Amico G, Pagliaro L, Bosch J. The treatment of portal hypertension: a meta-analytic review. Hepatology 1995;22(1):332–54.

    PubMed  CAS  Google Scholar 

  106. McCormack G, McCormick PA. A practical guide to the management of esophageal varices. Drug 1999;57:327–35.

    CAS  Google Scholar 

  107. Garcia-Tsao G. Current management of the complications of cirrhosis and portal hypertension: variceal hemorrhage, ascites, and spontaneous bacterial peritonitis. Gastroenterology 2001;120:726–48.

    PubMed  CAS  Google Scholar 

  108. Moitinho E, Planas R, Banares R, Albillos A, Ruiz-del-Arbol L, Galvez C et al. Multicenter randomized controlled trial comparing different schedules of somatostatin in the treatment of acute variceal bleeding. J Hepatol 2001;35:712–8.

    PubMed  CAS  Google Scholar 

  109. de Franchis R, Dell’Era A, Iannuzzi F. Diagnosis and treatment of portal hypertension. Dig Liver Dis 2004;36:787–98.

    PubMed  Google Scholar 

  110. Dagher L, Burroughs A. Variceal bleeding and portal hypertensive gastropathy. Eur J Gastroenterol Hepatol 2001;13:81–8.

    PubMed  CAS  Google Scholar 

  111. Sarin SK, Shastri HM, Jain M Jain AK, Issar SK, Murthy NS. The natural history of portal hypertensive gastropathy. Influence of variceal eradication. Am J Gastro 2000;95:2888–93.

    CAS  Google Scholar 

  112. Sarin SK, Govil A, Jain AK, Guptan RC, Issar SK, Jain M. Prospective trial of endoscopic sclerotherapy versus variceal band ligation for esophageal varices, influence on gastric varices and variceal recurrence. J Hepatol 1994;26:826–32.

    Google Scholar 

  113. De Franchis R, Primignani M. Natural history of portal hypertension in patients with cirrhosis. Clin Liver Dis 2001;5:645.

    PubMed  Google Scholar 

  114. Chawla YK, Bhushnurmath SR, Dilawari JB. Cruveilhier Baumgarten syndrome in idiopathic portal hypertension. Am J Gastro. 1987;82:12.

    Google Scholar 

  115. Lay CS, Tsai Y Te, Lee FY, Lai YL, Yu CJ, Chen CB et al. Endoscopic variceal ligation versus propranolol in prophylaxis of first variceal bleeding in patients with cirrhosis. Ann Gastro Hepatol J 2006;21:413–9.

    Google Scholar 

  116. Sarin SK, Wadhawan M, Agrawal SR, Tyagi P, Sharma BC. Endoscopic variceal ligation plus propranolol versus endoscopic variceal ligation alone in primary prophylaxis of variceal bleeding. Am J Gastro 2005;100:797–804.

    Google Scholar 

  117. Pal S, Radhakrishna P, Sahni P, Pande GK, Nundy S, Chattopadhyay TK. Prophylactic surgery in non-cirrhotic portal fibrosis: is it worthwhile? Indian J Gastroenterol 2005;24:239–42.

    PubMed  Google Scholar 

  118. Chawla YK, Dilawari JB, Dhiman RK, Goenka MK, Bhasin DK, Kochhar R. Sclerotherapy in noncirrhotic portal fibrosis. Dig Dis Sci 1997;42:1449–53.

    PubMed  CAS  Google Scholar 

  119. Bhargav DK. Dasarathy S, Sunderam KR, Ahuja RK. Efficacy of endoscopic sclerotherapy on long term management of esophageal varices: a comparative study of results in patients with cirrhosis beta, NCPF and EHPVO. J Gastroenterol Hepatol 1991;6:471–5.

    Google Scholar 

  120. Kochhar R, Goenka MK, Mehta SK. Outcome of infection sclerotherapy using absolute alcohol in patients with cirrhosis, NCPF and EHPVO. Gastrointest Endosc 1991;37:460–4.

    Article  PubMed  CAS  Google Scholar 

  121. Fakrly S, Omar M, Gannam AI. EST versus EVL in management of bleeding esophageal varices: a prospective randomized study in schiolosomal hepatic fibrosis. Endoscopic Arch Ed 2000;1:39–44.

    Google Scholar 

  122. Kire CF. Controlled trial of propranolol to prevent recurrent variceal bleeding in patients with non cirrhotic portal fibrosis. BMJ 1989;298:1363–5.

    Google Scholar 

  123. Jacobi D, de Muret A, Asbertle B, Perarnan TA. TIPS for treatment of portal hypertension secondary to noncirrhotic perisinusoidal hepatic fibrosis. Eur J Gastrohepatol 2006;18:549–51.

    Google Scholar 

  124. Sharma BC, Singh RP Chawla YK, Narasimhan KL, Rao KL, Mitra SK. Effect of shunt surgery on spleen size, portal pressure and esophageal varices in non cirrhotic portal fibrosis. J Gastroenterol Hepatol 1997;12:582–4.

    PubMed  CAS  Google Scholar 

  125. Mathur SK, Shah SR, Nagral SS, Soonawala ZF. Transabdominal extensive esophagogastric devascularization with gastroesophageal stapling for management of noncirrhotic portal hypertension: long-term results. World J Surg 1999;23:1168–74.

    PubMed  CAS  Google Scholar 

  126. Rosemurgy AS, Serafini FM, Ziverbel BR Black TJ, Kudryk BT, Nord HJ. TIPS vs small diameter porta caval shunt. Extended follow up of our expanded randomized prospective trial. J Gastrointest Surg 2000;4:589–97.

    PubMed  CAS  Google Scholar 

  127. Shozo H, Satoshi I, Shinichi M, Tomofumi M, Tetsuya F, Takeshi Y. Interventional radiologic treatment for idiopathic portal hypertension. Cardiovasc Intervent Radiol 1999;22:311–4.

    Google Scholar 

  128. Haskal ZJ, Louis M, Cardella JF, Cole PJ, Drooz A, Grassi CJ et al. Quality improvement guidelines for transjugular intrahepatic portosystemic shunts. J Vasc Interv Radiol 2003;14:S265–S270.

    PubMed  Google Scholar 

  129. Wolff M, Hirner A. Current state of portosystemic shunt surgery. Langenbeck’s Arch Surg 2003;388:141–9.

    Google Scholar 

  130. Rösch J, Keller FS. Transjugular intrahepatic portosystemic shunt: present status, comparison with endoscopic therapy and shunt surgery, and future perspectives. World J Surg 2001;25:337–46.

    PubMed  Google Scholar 

  131. Hausegger KA, Karnel F, Georgieva B, Tauss J, Portugaller H, Deutschmann H et al. Transjugular intrahepatic porto systemic shunt creation with the Viatorr expanded polytetrafluoroethylene-covered stent-graft. J Vasc Interv Radiol 2004;15:239–48.

    PubMed  Google Scholar 

  132. Freedman AM, Sanyal AJ, Tisnado J, Cole PE, Sbiffman M, Luketic VA et al. Complications of transjugular intrahepatic portosystemic shunt: a comprehensive review. Radiographics 1993;13:1185–210.

    PubMed  CAS  Google Scholar 

  133. Barton RE, Rösch J, Saxon RR, Lakin PC, Petersen BD, Keller FS. TIPS: short and long-term results: a survey of 1750 patients. Semin Interv Radiol 1995;12:364.

    Google Scholar 

  134. Orloff MJ. Prophylactic portasystemic shunt in non-cirrhotic portal fibrosis: is it worthwhile? Nobody knows. Indian J Gastroenterol 2005;24:233–5.

    PubMed  Google Scholar 

  135. Kanagawa H, Mima S, Kouyama H, Gotoh K, Uchida T, Okuda K. Treatment of gastric fundal varices by balloon-occluded retrograde transvenous obliteration. J Gastroenterol Hepatol 1996;11:51–8.

    PubMed  CAS  Google Scholar 

  136. Hirotaka A, Takehiko A, Hitoshi T, Masatomo M. Efficacy of balloon-occluded retrograde transvenous obliteration, percutaneous transhepatic obliteration and combined techniques for the management of gastric fundal varices. World J Gastroenterol 2006;12:3866–73.

    Google Scholar 

  137. Shimoda R, Horiuchi K, Hagiwara S, Suzuki H, Yamazaki Y, Kosone T et al. Short-term complications of retrograde transvenous obliteration of gastric varices in patients with portal hypertension: effects of obliteration of major portosystemic shunts. Abdom Imaging 2005;30:306–13.

    PubMed  CAS  Google Scholar 

  138. Sama SK, Bhargawa S, Nath NG, Talwar JR, Nayak NC, Tandon BN et al. Non-cirrhotic portal fibrosis. Am J Med 1971;51:160–9.

    PubMed  CAS  Google Scholar 

  139. Sarin SK. Non-cirrhotic portal fibrosis. Gut 1989;5:336–51.

    Google Scholar 

  140. Sarin SK, Kumar A. Non cirrhotic portal hypertension. Clin Liver Dis 2006;10:627–51.

    PubMed  Google Scholar 

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Correspondence to Shiv Kumar Sarin.

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Sarin, S.K., Kumar, A., Chawla, Y.K. et al. Noncirrhotic portal fibrosis/idiopathic portal hypertension: APASL recommendations for diagnosis and treatment. Hepatol Int 1, 398–413 (2007). https://doi.org/10.1007/s12072-007-9010-9

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