Subscribe to RSS
Please copy the URL and add it into your RSS Feed Reader.
https://www.thieme-connect.de/rss/thieme/en/10.1055-s-00000069.xml
Download PDF
Semin Liver Dis 2008; 28(3): 270-281
DOI: 10.1055/s-0028-1085095
© Thieme Medical PublishersDOI: 10.1055/s-0028-1085095
Intrahepatic Portal Venopathy and Related Disorders of the Liver
Further Information
Publication History
Publication Date:
23 September 2008 (online)
ABSTRACT
Intrahepatic portal venopathy leads to various entities that are important causes of portal hypertension. Noncirrhotic portal fibrosis (NCPF) occurs in the Indian subcontinent, whereas idiopathic portal hypertension (IPH) occurs in Japan although the pathogenesis and presentation of both are similar. NCPF presents mainly with upper gastrointestinal bleeding; IPH presents with massive splenomegaly. The liver functions are preserved. Wedged hepatic venous pressure is normal, but portal venous pressure is high indicating a presinusoidal block. Patients are best managed with endoscopic therapy or surgery, with better results than in patients with cirrhosis. Nodular regenerative hyperplasia is a histological diagnosis characterized by development of nodules in the liver due to uneven perfusion of the portal venous blood. These patients may develop portal hypertension and if they bleed would require treatment as in NCPF/IPH. Schistosomiasis produces portal hypertension by the development of fibrous tissue around the portal veins as a response to schistosome eggs. Gratifying results have been reported with praziquantel therapy. Rarely sarcoidosis and chronic biliary obstruction may also produce portal venopathy.
KEYWORDS
Hepatoportal sclerosis - schistosomiasis - idiopathic portal hypertension - noncirrhotic portal fibrosis - nodular regenerative hyperplasia - incomplete septal cirrhosis - noncirrhotic portal hypertension
REFERENCES
- 1 Mikkelsen W P, Edmoandson H A, Peters R L et al.. Extra and intrahepatic portal hypertension without cirrhosis (hepatoportal sclerosis). Ann Surg. 1965; 162 602-620
- 2 Kingham J G, Levison D A, Stansfeld A G, Dawson A M. Non-cirrhotic intrahepatic portal hypertension: a long term follow-up study. Q J Med. 1981; 50 259-268
- 3 Levison D A, Kingham J G, Dawson A M, Stansfeld A G. Slow cirrhosis or no cirrhosis? A lesion causing benign intrahepatic portal hypertension. J Pathol. 1982; 137 253-272
- 4 Polish E, Christie J, Cohen A, Sullivan Jr B. Idiopathic presinusoidal portal hypertension (Banti's syndrome). Ann Intern Med. 1962; 56 624-627
- 5 LeBail B, Bernand P H, Balaband C. Bioulac-Sage . Hepatoportal sclerosis. Semin Liver Dis. 1995; 15 329-339
- 6 Hillaire S, Bonte E, Deninger M H et al.. Idiopathic noncirrhotic intrahepatic portal hypertension in the West: a re-evaluation in 28 patients. Gut. 2002; 51 275-280
- 7 Dhiman R K, Chawla Y, Vashista R K et al.. Noncirrhotic portal fibrosis (idiopathic portal hypertension) experience with 151 patients and a review of the literature. J Gastroenterol Hepatol. 2002; 17 6-16
- 8 Pande C, Kumar A, Sarin S K. Noncirrhotic portal fibrosis a clinical profile of 366 patients. Am J Gasteroenterol. 2006; 101 S19
- 9 Sarin S K, Kumar A, Chawla Y K et al.. Noncirrhotic portal fibrosis/idiopathic portal hypertension. APASL recommendations for diagnosis and treatment. Hepatol Int. 2007; 1 398-413
- 10 Mall M L, Rai R R, Philip M et al.. Seroepidemiology of hepatitis A infection in India: changing pattern. Indian J Gastroenterol. 2001; 20 132-135
- 11 Okudaira M, Ohbu M, Okuda K. Idiopathic portal hypertension and its pathology. Semin Liver Dis. 2002; 22 59-72
- 12 Imanaga H, Yamamoto S, Kuroyanagi Y. Surgical treatment of portal hypertension according to state of Intrahepatic circulation. Ann Surg. 1962; 155 42-50
- 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, Japan; Tokyo Ministry of Health and Welfare 1975: 10-11
MissingFormLabel
- 14 Iwata H, Nishikawa A, Tanaka H et al..
National survey on idiopathic portal hypertension . In: Kameda H 1985 Report of the Research Committee on Aberrant Portal Hemodynamics (in Japanese). Tokyo, Japan; Tokyo Ministry of Health and Welfare 1986: 117-129MissingFormLabel - 15 Iman F, Kuge K, Komaba M et al..
Interim report on IPH survey . In: Futagawa S 1992 Report of the Research Committee on Aberrant Portal Hemodynamics (in Japanese). Tokyo, Japan; Tokyo Ministry of Health & Welfare 1993: 107-110MissingFormLabel - 16 Okuda K. Noncirrhotic portal hypertension: why it is so common in India. J Gastroenterol Hepatol. 2002; 17 1-5
- 17 Datta D V, Mitra S K, Chhutani P N, Chakravarti R N. Chronic oral arsenic intoxication as a possible etiological factor in idiopathic portal hypertension (Non cirrhotic portal fibrosis) in India. Gut. 1979; 20 378-384
- 18 Mazumdar D N, Das Gupta J, Santra A et al.. Chronic assenic toxicity in West Bengal – the worst calamity in the world. J Indian Med Assoc. 1998; 96 4-7
- 19 Santra A, Das Gupta J D, De B K, Roy B, Guha Mazumdar D N. Hepatic manifestations in chronic arsenic toxicity. Indian J Gastroenterol. 1999; 18 152-155
- 20 Thomas L B, Popper H, Berk P D, Selikoff I, Falk H. Vinyl-chloride-induced liver disease. From idiopathic portal hypertension (Banti's syndrome) to angiosarcomas. N Engl J Med. 1975; 292 17-22
- 21 Blendis L M, Smith P M, Lawrie B W, Stephens M R, Evan N D. Portal hypertension in vinyl chloride monomer workers. A hemodynamic study. Gastroenterology. 1978; 75 206-211
- 22 Pimentel J C, Menezes A P. Liver disease in vineyard sprayers. Gastroenterology. 1977; 72 275-283
- 23 Podurgiel B J, McGill D B, Ludwig J, Taylor W F, Muller S A. Liver injury associated with methotrexate therapy for psoriasis. Mayo Clin Proc. 1973; 48 787-792
- 24 Nataf C, Feldmann G, Lebrec D et al.. Idiopathic portal hypertension (perisinusoidal fibrosis) after renal transplantation. Gut. 1979; 20 531-537
- 25 Barnard J A, Marshall G S, Neblett W W, Gray G, Ghishan F K. Noncirrhotic portal fibrosis after Wilms' tumor therapy. Gastroenterology. 1986; 90 1054-1056
- 26 Geubel A P, 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 1701-1709
- 27 Nevens F, Fevery J, Van Steenbergen W et al.. Arsenic and non-cirrhotic portal hypertension. A report of eight cases. J Hepatol. 1990; 11 80-85
- 28 Das S, Santra A, Lahiri S, Guha Mazumder D N. Implications of oxidative stress and hepatic cytokine (TNF-alpha and IL-6) response in the pathogenesis of hepatic collagenesis in chronic arsenic toxicity. Toxicol Appl Pharmacol. 2005; 204 18-26
- 29 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(11) 1312-1317
- 30 Nayyar A K, Sharma B K, Sarin S K et al.. Characterization of peripheral blood lymphocytes in patients with non-cirrhotic portal fibrosis. A comparison with cirrhotic and healthy controls. J Gastroenterol Hepatol. 1990; 5 554-559
- 31 Sarin S K, Mehra N K, Agarwal A et al.. Familial aggregation in noncirrhotic portal fibrosis: a report of four families. Am J Gastroenterol. 1987; 82 1130-1133
- 32 Yamaguchi N, Tokushige K, Harcuta I, Yamauchi K, Hayashi N. Analysis of adhesion molecules in patients with idiopathic portal hypertension. J Gastroenterol Hepatol. 1999; 14 364-369
- 33 Sato Y, Sawada S, Nakanuma Y. Fibulin 5 is involved in phlebosclerosis of major portal vein branches associated with elastic fiber deposition in idiopathic portal hypertension. Hepatol Res. 2008; 38 166-173
- 34 Yamagulin E, Yamanoi A, Ono T, Nagasue N. Experimental investigation of the role of endothelin-1 in idiopathic portal hypertension. J Gasteroenterol Hepatol. 2007; 22 1134-1140
- 35 Kamath P S, Carpenter H A, Lloyd R V et al.. Hepatic localization of endothelin-1 in patients with idiopathic portal hypertension and cirrhosis of the liver. Liver Transpl. 2000; 6 596-602
- 36 Paradis V, Dargere D, Vidaud M et al.. Expression of connective tissue growth factor in experimental rat and human liver fibrosis. Hepatology. 1999; 30 968-976
- 37 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
- 38 Morikawa H, Tamori A, Nishiguchi S et al.. Expression of connective tissue growth factor in the human liver with idiopathic portal hypertension. Mol Med. 2007; 13 240-245
- 39 Inagaki H, Nonami T, Kawagoe T et al.. Idiopathic portal hypertension associated with systemic lupus erythematosus. J Gastroenterol. 2000; 35 235-239
- 40 Nakanuma Y, Nonomura A, Hayashi M 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; 39 586-592
- 41 Saito K, Nakanuma Y, Takegoshi K et al.. Non-specific immunological abnormalities and association of autoimmune diseases in idiopathic portal hypertension. A study by questionnaire. Hepatogastroenterology. 1993; 40 163-166
- 42 Sharma B C, Bhasin D K, Nada R. Association of celiac disease with noncirrhotic portal fibrosis. J Gasteroenterol Hepatol. 2006; 21 332-334
- 43 Makharia G, Dhiman R K, Chawla Y K, Vasistha R K. Non-cirrhotic portal fibrosis in a patient with rheumatoid arthritis. Indian J Gastroenterol. 2001; 20 197-198
- 44 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; 7 270-273
- 45 Shirai T, Hirose S, Futagawa S et al..
Abnormalities in T cells in idiopathic portal hypertension . In: Futagawa S Report on the Research Committee on Aberrant Portal Hemodynamics (in Japanese). Tokyo, Japan; Ministry of Health and Welfare 1993: 67-69MissingFormLabel - 46 Schiano T D, Kotler D P, Ferian E, Fiel M I. Hepatoportal sclerosis as a cause of noncirrhotic portal hypertension in patients with HIV. Am J Gastroenterol. 2007; 102 2536-2540
- 47 Boyer J L.
Non-cirrhotic portal hypertension . In: Hoofnagle JH, Goodman Z Liver Biopsy: Interpretation for the 1990s. Clinicopathological Correlation in Liver Disease. Thorofare, NJ; Slack/AASLD 1991: 428-439MissingFormLabel - 48 Wanless I R.
Non-cirrhotic portal hypertension and nodular transformation (nodular regenerative hyperplasia) . In: Hoofnagle JH, Goodman Z Liver Biopsy: Interpretation for the 1990s. Clinicopathological Correlation in Liver Disease. Thorofare, NJ; Slack/AASLD 1991: 440-455MissingFormLabel - 49 Albini B, Ito S, Brentjens J, Aandres G. Splenomegaly and immune complex splenitis in rabbits with experimentally induced chronic serum sickness immunopathological findings. J Reticuloendothel Soc. 1983; 34 485-500
- 50 Kono K, Ohnishi K, Omata M et al.. Experimental portal fibrosis produced by intraportal injection of killed nonpathogenic Escherichia coli in rabbits. Gastroenterology. 1988; 94 787-796
- 51 Shekhar K C, Pathmanathan R. Hepatosplenic lesions induced by experimental Schistosoma malayensis in rabbits. Southeast Asian J Trop Med Public Health. 1993; 24 333-339
- 52 Okuda K, Nakashima T, Okudaira 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; 2 176-192
- 53 Nayak N C, Ramalingaswami V. Obliterative portal venopathy of the liver. Arch Pathol. 1969; 87 359-369
- 54 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; 35 885-897
- 55 Tandon B N, Lakshminarayanan R, Bhargava S, Nayak N C, Sama S K. Ultrastructure of the liver in non-cirrhotic portal fibrosis with portal hypertension. Gut. 1970; 11 905-910
- 56 Sciot R, Staessen D, Van Damme B et al.. Incomplete septal cirrhosis: histopathological aspects. Histopathology. 1988; 13 593-603
- 57 Hidaka H, Ohbu M, Kokubu S et al.. Hepatocellular carcinoma associated with idiopathic portal hypertension: review of large nodules in seven non-cirrhotic portal hypertensive livers. J Gastroenterol Hepatol. 2005; 20 493-494
- 58 Schinoni M I, Andrade Z, de Freitas L A, Oliveira R, Paraná R. Incomplete septal cirrhosis: an enigmatic disease. Liver Int. 2004; 24 452-456
- 59 Nakanuma Y, Hoso M, Sasaki M et al.. Histopathology of the liver in non-cirrhotic portal hipertensión of unknown aetiology. Histopathology. 1996; 28 195-204
- 60 Bernard P H, Le Bail B, Cransac M et al.. Progression from idiopathic portal hypertension to incomplete septal cirrhosis with liver failure requiring liver transplantation. J Hepatol. 1995; 22 495-499
- 61 Okuda K, Nakashima T, Kameda H for the Japan Ministry of Health & Welfare Research Committee on Idiopathic Portal
Hypertension et al..
Idiopathic noncirrhotic portal hypertension: a natural study . In: Brunner H, Thalei H Hepatology a festschrift for Hans Popper. New York; Raven Press 1985: 95-108MissingFormLabel - 62 Sama S K, Bhargava S, Nath N G et al.. Noncirrhotic portal fibrosis. Am J Med. 1971; 51 160-169
- 63 Boyer J L, Sen Gupta K P, Biswas S K et al.. Idiopathic portal hypertension. Comparison with the portal hypertension of cirrhosis and extrahepatic portal vein obstruction. Ann Intern Med. 1967; 66 41-68
- 64 Matsutani S, Maruyama H, Akiike T et al.. Study of portal vein thrombosis in patients with idiopathic portal hypertension in Japan. Liver Int. 2005; 25 978-983
- 65 Warren W D, Henderson J M, Millikan W J, Galambos J T, Bryan F C. Management of variceal bleeding in patients with noncirrhotic portal vein thrombosis. Ann Surg. 1988; 207 623-634
- 66 Sarin S K, Kumar A. Noncirrhotic portal hypertension. Clin Liver Dis. 2006; 10 627-651
- 67 Mathur S K, Shah S R, Nagral S S, Soonawala Z F. Transabdominal extensive esophagogastric devascularization with gastroesophageal stapling for management of noncirrhotic portal hypertension: long-term results. World J Surg. 1999; 23 1168-1174
- 68 Ishii M, Katada Y. Idiopathic portal hypertension in a systemic sclerosis patient heterozygous for factor V Leiden mutation. Rheumatol Int. 2003; 23 44-46
- 69 Nevens F, Staessen D, Sciot R et al.. Clinical aspects of incomplete septal cirrhosis in comparison with macronodular cirrhosis. Gastroenterology. 1994; 106 459-463
- 70 Rangari M, Sinha S, Kapoor D, Mohan J C, Sarin S K. Prevalence of autonomic dysfunction in cirrhotic and noncirrhotic portal hypertension. Am J Gastroenterol. 2002; 97 707-713
- 71 Chawla Y, Dilawari J B. Anorectal varices, their frequency in cirrhotic and Noncirrhotic portal hypertension. Gut. 1991; 32 309-311
- 72 Futagawa S, Fukazawa M, Horisawa M et al.. Portographic liver changes in idiopathic noncirrhotic portal hypertension. AJR Am J Roentgenol. 1980; 134 917-923
- 73 Okuda K, Kono K, Ohnishi K et al.. Clinical study of eighty-six cases of idiopathic portal hypertension and comparison with cirrhosis with splenomegaly. Gastroenterology. 1984; 86 600-610
- 74 Villeneuve J P, Huet P M, Joly J G et al.. Idiopathic portal hypertension. Am J Med. 1976; 61 459-464
- 75 Okuda K, Nakashima T, Okudaira M et al.. Anatomical basis of hepatic venographic alterations in idiopathic portal hypertension. Liver. 1981; 1 255-263
- 76 Dilawari J B, Chawla Y K, Chari S T. Natural/spontaneous shunts in non-cirrhotic portal fibrosis. J Gastroenterol Hepatol. 1989; 4 215-219
- 77 Ohnishi K, Nakayama T, Koen H et al.. Interrelationship between type of spontaneous portal systemic shunt and portal vein pressure in patients with liver disease. Am J Gastroenterol. 1985; 80 561-564
- 78 Okuda K, Obata H.
Idiopathic portal hypertension (hepato portal sclerosis) . In: Okuda K, Benhamoci JP Portal Hypertension. Clinical and Physiological Aspects. Tokyo; Springer 1992: 271-287MissingFormLabel - 79 Sinha R. Grayscale and pulsed Doppler characteristics of non-cirrhotic portal fibrosis: a preliminary report. Clin Radiol. 1999; 54 156-159
- 80 Fukuzawa T, Matsutani H, Akiike T, Saisho H, Hatlon T. Magnetic resonance images of the globus pallidus in patients with idiopathic portal hypertension: a qualitative analysis of the relationship between signal intensity and grade of portosystemic shunt. J Gastroenterol Hepatol. 2006; 21 902-907
- 81 Qureshi H, Kamal S, Khan R A, Zuberi S J, Alam S E. Differentiation of cirrhotic vs idiopathic portal hypertension using 99mTc-Sn colloid dynamic and static scintigraphy. J Pak Med Assoc. 1991; 41 126-129
- 82 Ohnishi K, Saito M, Sato S et al.. Portal hemodynamics in idiopathic portal hypertension (Banti's syndrome). Comparison with chronic persistent hepatitis and normal subjects. Gastroenterology. 1987; 92 751-758
- 83 Sarin S K, Sethi K K, 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; 28 260-266
- 84 Pal S, Radhakrishna P, Sahni P et al.. Prophylactic surgery in non-cirrhotic portal fibrosis: is it worthwhile?. Indian J Gastroenterol. 2005; 24 239-242
- 85 Chawla Y K, Dilawari J B, Dhiman R K et al.. Sclerotherapy in noncirrhotic portal fibrosis. Dig Dis Sci. 1997; 42 1449-1453
- 86 Bhargava D K, Dasarathy S, Sundaram K R, Ahuja R K. 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. 1991; 6 471-475
- 87 Kochhar R, Goenka M K, Mehta S K. Outcome of injection sclerotherapy using absolute alcohol in patients with cirrhosis, non-cirrhotic portal fibrosis, and extrahepatic portal venous obstruction. Gastrointest Endosc. 1991; 37 460-464
- 88 Jacobi D, de Muret A, Arbeille B, Perarnau J M. Transjugular intrahepatic portosystemic shunt for the treatment of portal hypertension secondary to non-cirrhotic perisinusoidal hepatic fibrosis. Eur J Gastroenterol Hepatol. 2006; 18 549-551
- 89 Arai H, Abe T, Takagi H, Mori 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 3866-3873
- 90 Romano M, Giojelli A, Capuano G, Pomponi D, Salvatore M. Partial splenic embolization in patients with idiopathic portal hypertension. Eur J Radiol. 2004; 49 268-273
- 91 Sharma B C, Singh R P, Chawla Y K et al.. Effect of shunt surgery on spleen size, portal pressure and oesophageal varices in patients with non-cirrhotic portal hypertension. J Gastroenterol Hepatol. 1997; 12 582-584
- 92 Mathur S K, Shah S R, Nagral S S, Soonawala Z F. Transabdominal extensive esophagogastric devascularization with gastroesophageal stapling for management of noncirrhotic portal hypertension: long-term results. World J Surg. 1999; 23 1168-1174
- 93 Fukazawa M, Sugiura M.
Portal hypertension . In: Oka H, Sugiura M Rinsho Kanzobyogaku. Tokyo; Asakura Shoten 1989: 221-222MissingFormLabel - 94 Eguchi A, Hashizume M, Kitano S et al.. High rate of portal thrombosis after splenectomy in patients with esophageal varices and idiopathic portal hypertension. Arch Surg. 1991; 126 752-755
- 95 Belli L, Romani F, Riolo F et al.. Thrombosis of portal vein in absence of hepatic disease. Surg Gynecol Obstet. 1989; 169 46-49
- 96 Wanless I R. Micronodular transformation (nodular regenerative hyperplasia) of the liver: a report of 64 cases among 2,500 autopsies and a new classification of benign hepatocellular nodules. Hepatology. 1990; 11 787-797
- 97 Wanless I R. Noncirrhotic portal hypertension: recent concepts. Prog Liver Dis. 1996; 14 265-278
- 98 Devarbhavi H, Abraham S, Kamath P S. Significance of nodular regenerative hyperplasia occurring de novo following liver transplantation. Liver Transpl. 2007; 13 1552-1556
- 99 Wanless I R, Godwin T A, Allen F, Feder A. Nodular regenerative hyperplasia of the liver in hematologic disorders: a possible response to obliterative portal venopathy. A morphometric study of nine cases with an hypothesis on the pathogenesis. Medicine (Baltimore). 1980; 59 367-379
- 100 Stromeyer F W, Ishak K G. Nodular transformation (nodular “regenerative” hyperplasia) of the liver. A clinicopathologic study of 30 cases. Hum Pathol. 1981; 12 60-71
- 101 Wanless I R, Solt L C, Kortan P et al.. Nodular regenerative hyperplasia of the liver associated with macroglobulinemia. A clue to the pathogenesis. Am J Med. 1981; 70 1203-1209
- 102 Young I D, Segura J, Ford P M, Ford S E. The pathogenesis of nodular regenerative hyperplasia of the liver associated with rheumatoid vasculitis. J Clin Gastroenterol. 1992; 14 127-131
- 103 Nakanuma Y. Non-neoplastic nodular lesions in the liver. Pathol Int. 1995; 45 703-714
- 104 Nakanuma Y, Ohta G. Nodular hyperplasia of the liver in primary biliary cirrhosis of early histological stages. Am J Gastroenterol. 1987; 82 8-10
- 105 Wanless I R, Gryfe A. Nodular transformation of the liver in hereditary hemorrhagic telangiectasia. Arch Pathol Lab Med. 1986; 110 331-335
- 106 Ferlitsch A, Teml A, Reinisch W et al.. 6-thioguanine associated nodular regenerative hyperplasia in patients with inflammatory bowel disease may induce portal hypertension. Am J Gastroenterol. 2007; 102 2495-2503
- 107 Hubert C, Sempoux C, Horsmans Y et al.. Nodular regenerative hyperplasia: a deleterious consequence of chemotherapy for colorectal liver metastases?. Liver Int. 2007; 27 938-943
- 108 Rougier P, Degott C, Rueff B, Benhamou J P. Nodular regenerative hyperplasia of the liver. Report of six cases and review of the literature. Gastroenterology. 1978; 75 169-172
- 109 Horita T, Tsutsumi A, Takeda T et al.. Significance of magnetic resonance imaging in the diagnosis of nodular regenerative hyperplasia of the liver complicated with systemic lupus erythematosus: a case report and review of the literature. Lupus. 2002; 11 193-196
- 110 Naber A H, Van Haelst U, Yap S H. Nodular regenerative hyperplasia of the liver: an important cause of portal hypertension in non-cirrhotic patients. J Hepatol. 1991; 12 94-99
- 111 Samuel D, Smadja C, Degos F et al.. Encephalopathy following portal shunting in nodular regenerative hyperplasia of the liver. Gastroenterol Clin Biol. 1985; 9 162-165
- 112 Dunn M A, Kamel R. Hepatic schistosomiasis. Hepatology. 1981; 1 653-661
- 113 Symmers W SC. Note on a new form of liver cirrhosis due to the presence of ova of Bilharzia hematobium. J Pathol Bartenol. 1904; 9 237-239
- 114 Boros D L, Pelley R P, Warren K S. Spontaneous modulation of granulomatous hypersensitivity in Schistosoma mansoni. J Immunol. 1975; 114 1437-1441
- 115 Pearce E J, Caspar P, Grzych J M, Lewis F A, Sher A. Downregulation of Th1 cytokine production accompanies induction of Th2 responses by a parasitic helminth, Schistosoma mansoni. J Exp Med. 1991; 173 159-166
- 116 Salam E A, Ishaac S, Mahmoud A A. Histocompatibility-linked susceptibility for hepatosplenomegaly in human schistosomiasis mansoni. J Immunol. 1979; 123 1829-1831
- 117 Dessein A J, Hillaire D, Elwali N E et al.. Severe hepatic fibrosis in Schistosoma mansoni infection is controlled by a major locus that is closely linked to the interferon-gamma receptor gene. Am J Hum Genet. 1999; 65 709-721
- 118 Shahin M, Schuppan D, Waldherr R et al.. Serum procollagen peptides and collagen type VI for the assessment of activity and degree of hepatic fibrosis in schistosomiasis and alcoholic liver disease. Hepatology. 1992; 15 637-644
- 119 Wyler D J. Fibrosin, a novel fibrogenic protein: discovery, cloning and implications for fibrotic disorders. Int Arch Allergy Immunol. 1996; 111 326-329
- 120 Andrade Z A, Guerret S, Fernandes A L. Myofibroblasts in schistosomal portal fibrosis of man. Mem Inst Oswaldo Cruz. 1999; 94 87-93
- 121 Morcos S H, Khayyal M T, Mansour M M et al.. Reversal of hepatic fibrosis after praziquental therapy of murine Schistosomiasis . Am J Trop Med Hyg. 1985; 34 314-321
- 122 Andrade Z A, Cox T M, Cheever A M. Regression of hepatic lesions after treatment of Schistosoma mansoni or Schistosoma japonicum infection in mice: a comparative study. Am J Trop Med Hyg. 1993; 49 1-9
- 123 Dunn M A, Cheever A W, Paglia L M et al.. Reversal of advanced liver fibrosis in rabbits with Schistosomiasis japonica. Am J Trop Med Hyg. 1994; 50 499-505
- 124 Li Y S, Sleigh A C, Ross A G et al.. Two-year impact of praziquantel treatment for Schistosoma japonicum infection in China: re-infection, subclinical disease and fibrosis marker measurements. Trans R Soc Trop Med Hyg. 2000; 94 191-197
- 125 Boisier P, Ramarokoto C E, Ravaoalimalala V E et al.. Reversibility of Schistosoma mansoni-associated morbidity after yearly mass praziquantel therapy: ultrasonographic assessment. Trans R Soc Trop Med Hyg. 1998; 92 451-453
- 126 Ruiz-Guevara R, de Noya B A, Valero S K et al.. Clinical and ultrasound findings before and after praziquantel treatment among Venezuelan schistosomiasis patients. Rev Soc Bras Med Trop. 2007; 40 505-511
- 127 Manzella A, Ohtomo K, Monzawa S, Lim J H. Schistosomiasis of the liver. Abdom Imaging. 2008; 33(2) 144-150
- 128 Nash T E, Cheever A W, Ottesen E A, Cook J A. Schistosome infections in humans: perspectives and recent findings. NIH conference. Ann Intern Med. 1982; 97 740-754
- 129 Mudawi H, Ali Y, El Tahir M. Prevalence of gastric varices and portal hypertensive gastropathy in patients with Symmers periportal fibrosis. Ann Saudi Med. 2008; 28 42-44
- 130 Coutinho A. Hemodynamic studies of portal hypertension in schistosomiasis. Am J Med. 1968; 44 547-556
- 131 Ramos O L, Saad F, Leser W P. Portal hemodynamics and liver cell function in hepatic schistosomiasis. Gastroenterology. 1964; 47 241-247
- 132 Grimaud J A, Borojevic R. Chronic human schistosomiasis mansoni. Pathology of the Disse's space. Lab Invest. 1977; 36 268-273
- 133 Alves C A, Alves A R, Abreu W N, Andrade Z A. Hepatic artery hypertrophy and sinusoidal hypertension in advanced schistosomiasis. Gastroenterology. 1977; 72 126-128
- 134 Siqueira E S, Rohr M R, Libera E D, Castro R R, Ferrari A P. Band ligation or sclerotherapy as endoscopic treatment for oesophageal varices in schistosomotic patients: results of a randomized study. HPB Surg. 1998; 11 27-32
- 135 el Tourabi H, el Amin A A, Shaheen M et al.. Propranolol reduces mortality in patients with portal hypertension secondary to schistosomiasis. Ann Trop Med Parasitol. 1994; 88 493-500
- 136 Conn H O. A randomized comparison of three types of surgery in schistosomal portal hypertension: many fewer answers than questions. Hepatology. 1994; 20 526-528
- 137 Valla D, Pessegueiro-Miranda H, Degott C et al.. Hepatic sarcoidosis with portal hypertension. A report of seven cases with a review of the literature. Q J Med. 1987; 63 531-544
- 138 Moreno-Merlo F, Wanless I R, Shimamatsu K et al.. The role of granulomatous phlebitis and thrombosis in the pathogenesis of cirrhosis and portal hypertension in sarcoidosis. Hepatology. 1997; 26 554-560
- 139 Tekeste H, Latour F, Levitt R E. Portal hypertension complicating sarcoid liver disease: case report and review of the literature. Am J Gastroenterol. 1984; 79 389-396
- 140 Mistilis S P, Green J R, Schiff L. Hepatic sarcoidosis with portal hypertension. Am J Med. 1964; 36 470-475
- 141 Devaney K, Goodman Z D, Epstein M S, Zimmerman H J, Ishak K G. Hepatic sarcoidosis. Clinicopathologic features in 100 patients. Am J Surg Pathol. 1993; 17 1272-1280
- 142 Sharma S K, Mohan A. Uncommon manifestations of sarcoidosis. J Assoc Physicians India. 2004; 52 210-214
- 143 Maddrey W C, Johns C J, Boitnott J K, Iber F L. Sarcoidosis and chronic hepatic disease: a clinical and pathologic study of 20 patients. Medicine (Baltimore). 1970; 49 375-395
- 144 Case Records of Massachusetts General Hospital. Weekly clinicopathological exercises. Case 1-1979. N Engl J Med. 1979; 300 28-37
- 145 Rudzki C, Ishak K G, Zimmerman H J. Chronic intrahepatic cholestasis of sarcoidosis. Am J Med. 1975; 59 373-387
- 146 Nakanuma Y, Ohta G, Kobayashi K, Kato Y. Histological and histometric examination of the intrahepatic portal vein branches in primary biliary cirrhosis without regenerative nodules. Am J Gastroenterol. 1982; 77 405-413
- 147 Kew M C, Varma R R, Dos Santos H A. Portal hypertension in primary biliary cirrhosis. Gut. 1971; 12 830-834
- 148 Abraham S C, Kamath P S, Eghtesad B, Demetris A J, Krasinskas A M. Liver transplantation in precirrhotic biliary tract disease: Portal hypertension is frequently associated with nodular regenerative hyperplasia and obliterative portal venopathy. Am J Surg Pathol. 2006; 30 1454-1461
- 149 Akhan O, Karaosmanoglu A D, Ergen B. Imaging findings in congenital hepatic firbrosis. Eur J Radiol. 2007; 61 18-24
- 150 Desmet V J. What is congenital hepatic fibrosis?. Histopathology. 1992; 20 465-467
- 151 Kerr D N, Okonkwo S, Choa R G. Congenital hepatic fibrosis: the long-term prognosis. Gut. 1978; 19 514-520
Yogesh ChawlaM.D.
Professor and Head, Department of Hepatology, Postgraduate Institute of Medical Education
and Research
Chandigarh 160 012, India
Email: ykchawla@gmail.com