Non-cirrhotic portal

Various infectious, toxic or immunological lesions lead to a presinusoidal block in adults. From a primary endothelial lesion, endophlebitis ensues. Rich in fibres and deficient in cells, it is ultimately responsible for the obliteration and even disappearance of the portal branches. Obliterative portal venopathy (N.C. Nayak et al., 1969) with portal and periportal fibrosis and subsequent perisinusoidal sclerosis is referred to as hepatoportal sclerosis (W P. Mikkelsen et al., 1965). This is a complex disorder involving splenomegaly, hypersplenism and portal hypertension, which has also been described as non-cirrhotic portal fibrosis (XL. Boyer et al., 1967) or idiopathic portal hypertension (K. Okuda et al., 1982). (I2l, 127) Band s syndrome (7) probably fell into this group ... 

Chawla, ., Dllawarl, JJ. Anorectal varices-their frequency in dr-rhotic and non-cirrhotic portal hypertension. Gut 1991 32 309 - 311... 

Vaklll, C., Farahvash, M.J., Bynum, T.E. Endemic idiopathic portal hypertension report on 32 patients with non-cirrhotic portal fibrosis. World J. Surg. 1992 16 118-125... 

Sarin, S.K., Nundy, S. Subclinical encephalopathy after portosystemic shunts in patients with non-cirrhotic portal fibrosis. Liver 1985 5 142-146... 

Sarin, S.K., Kapoor, D. Non-cirrhotic portal fibrosis current concepts and management, (review) J. Gastroenterol. Hepatol. 2002 17 526-534... 

Non-cirrhotic portal hypertension in congenital cytomegalovirus infection. Hepatology 1984 4 684- 686... 

Trevisani, F., Merli, M., Savelli, F., Valeriano, V., Zambruni, A., Riggio, O., Caraceni, R, Domenicali, M., Bernard , IVL QT interval in patients with non-cirrhotic portal hypertension and in cirrhotic patients treated with transjugular intrahepatic portosystemic shunt. J. Hepatol. 2003 38 461-467... 

Woolf, D., Voigt, MJ)., Jasklewicz, K., Kalla, A.A. Pulmonary hypertension associated with non-cirrhotic portal hypertension in systemic lupus erythematosus. Postgrad. Med. X 1994 70 41 -43... 

The United Kingdom Medical Research Council Chronic Myeloid Leukemia Group reported 18 cases of tioguanine-induced non-cirrhotic portal hypertension, commonly associated with deterioration in liver function (5). 

Shepherd PC, Fooks J, Gray R, Allan NC. Thioguanine used in maintenance therapy of chronic myeloid leukaemia causes non-cirrhotic portal hypertension. Results from MRC CML. II. Trial comparing busulphan with busulphan and thioguanine. Br I Haematol 1991 79(2) 185-92. 

Liver Long-term didanosine therapy was associated with non-cirrhotic portal hypertension in three HIV-positive individuals with chronic hepatitis C, mild liver fibrosis, and... 

In a nested case-control study conducted by the Swiss HIV Cohort there was a strong association between prolonged exposure to didanosine and non-cirrhotic portal hypertension [107 ]. In 15 patients with non-cirrhotic portal hypertension and 75 controls matched for duration of HIV infection, absence of viral hepatitis, and duration of follow-up, cumulative exposure to antiretroviral drug therapy (OR per year = 1.3 95% Cl = 1.0,1.6), nucleoside reverse transcriptase inhibitors (OR = 1.3 95% Cl = 1.1, 1.7), didanosine (OR = 3.4 95% Cl = 1.5, 8.1), ritonavir (OR = 1.4 95% Cl = 1.0, 1.9), and nelfinavir (OR = 1.4 95% Cl = 1.0,1.9) were longer in the patients with portal hypertension. Exposure to non-nucleoside reverse transcriptase inhibitors and other protease inhibitors were not different. 

Liver A further case of non-cirrhotic portal hypertension has been reported with didanosine [152 ]. 

A case report described mercaptopurine-induced hepatoportal sclerosis leading to non-cirrhotic portal hypertension in a patient with Crohn disease [76 ]. 

Again several reports stress the tri c consequences of the use of arsenic for medical purposes (Le. Fowler s solution). Angiosarcoma of the liver (7 ), multiple basal cell carcinoma (S -, 9 -), polyneuropathy (10 ). and non-cirrhotic portal hypertension (11 , 12) have been reported. Further study is required to assess the evidence that arsenic can also induce internal malignant tumours (8,9). 

Datta, D. V. (1976) Arsenic and non-cirrhotic portal hypertension. Lancet, Febr.,A iZ. 

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