Cirrhosis, biliary

Unlabeled Uses Prophylaxis of liver transplant rejection, treatment of alcoholic cirrhosis, biliary atresia, chronic hepatitis, gallstone formation, sclerosing cholangitis... 

Trade names Destolit (Norgine) Urso (Forte) (Axcan) Indications The dissolution of radiolucent (i.e. non-radio opaque) cholesterol gallstones in patients with a functioning gallbladder, primary biliary cirrhosis, biliary calculus. 

Approximately 11% of patients with ulcerative colitis are reported to have hepatobiliary complications with frequencies ranging from 5% to 95% in IBD patients overall. Hepatic complications include fatty liver, pericholangitis, chronic active hepatitis, and cirrhosis. Biliary complications include sclerosing cholangitis, cholangio-carcinoma, and gallstones. 

After hepatoblastoma, hepatocellular carcinoma (HCC) account for the majority of malignant tumors of the liver in patients between 5 and 15 years. Males are affected more than females, and 25% of these patients will usually have an underlying liver condition such as cirrhosis, biliary atresia, hereditary... 

There is very little evidence relating to the role of ROMs in cholestatic liver disease. Serum selenium and glutathione peroxidase activity are decreased in humans with intrahepatic cholestasis of pregnancy (Kauppila et al., 1987). Low levels of vitamin E have been reported in patients with primary biliary cirrhosis, and in children with Alagille s syndrome or biliary atresia (Knight et al., 1986 Jeffrey etal., 1987 Lemonnier etal., 1987 Babin etal., 1988 Kaplan et al., 1988 Sokol etal., 1989). Serum levels of Mn-SOD are increased in patients with all stages of primary biliary cirrhosis compared with patients with other forms of chronic liver disease, although whether this causes or results from the disease process is unclear (Ono etal., 1991). 

Jeffrey, G.P., Muller, D.P.R., Burroughs, A.K., Matthews, S., Kemp, C., Epstein, O., Metcalfe, T.A., Southam, E., Tazir-Melbourcy, M., Thomas, P.K. and McIntyre, N. (1987). Vitamin E deficiency and its clinical significance in adults with primary biliary cirrhosis and other forms of chronic liver disease. J. Hepatol. 4, 307-317. 

Knight, RE., Bourne, A.J., Newton, M., Black, A., Wilson, P. and Lawson, M.J. (1986). Neurologic syndrome associated with low levels of vitamin E in primary biliary cirrhosis. Gastroenterology 91, 209-211. 

Ono, M., Sekiya, C., Ohhira, M., Ohhiia, M., Namiki, M., Endo, Y., Susuki, K., Matsuda, Y. andTaniguchi, N. (1991). Elevated level of serum Mn-superoxide dismutase in patients with primary biliary cirrhosis possible involvement of free radicals in the pathogenesis of primary biliary cirrhosis. J. Lab. Clin. Med. 118, 476-483. 

PAS Periodic acid-SchiflF reagent PBA Polyclonal B cell activators PBC Primary biliary cirrhosis PBL Peripheral blood lymphocytes PBMC Peripheral blood mononuclear cells PBN N- e f-butyl-a-phenylnitrone PBS Phosphate-buffered saline PC Phosphatidylcholine... 

Biliary cirrhosis may be asymptomatic or evidenced by hepatomegaly, splenomegaly, or prolonged bleeding. 

Primary biliary cirrhosis is characterized by progressive inflammatory destruction of the bile ducts. Immune-mediated inflammation of intrahepatic bile ducts results in remodeling and scarring, causing retention of bile within the liver and subsequent hepatocellular damage and cirrhosis. The number of patients affected with primary biliary cirrhosis is difficult to estimate because many people are asymptomatic and incidental diagnosis during routine health care visits is common. 

Chronic obstruction of the common bile duct by the inflamed pancreas can cause icterus, cholangitis, and biliary cirrhosis.36... 

Cholestatic liver disease/cirrhosis (e.g., primary biliary cirrhosis)... 

Cholestatic liver diseases Primary biliary cirrhosis... 

Secondary biliary cirrhosis (possible causes gallstones, strictures, parasitic infection)... 

Alkaline phosphatase levels and GGT are elevated in plasma with obstructive disorders that disrupt the flow of bile from hepatocytes to the bile ducts or from the biliary tree to the intestines in condition such as primary biliary cirrhosis, sclerosing cholangitis, drug-induced cholestasis, gallstone disease, and autoimmune cholestatic liver disease. 

Malabsorption of protein and fat occurs when the capacity for enzyme secretion is reduced by 90%. A minority of patients develop complications including pancreatic pseudocyst, abscess, and ascites or common bile duct obstruction leading to cholangitis or secondary biliary cirrhosis. 

Reddy A, Prince M, James OF, Jain S, Bassendine MF (2004) Tamoxifen a novel treatment for primary biliary cirrhosis Liver Int 24 194-197... 

A recent report by the National Institutes of Health estimated that at 14 to 22 million people in the United States are affected by an autoimmune disease.1 As a group, these diseases represent a leading cause of death among women under age 65, with systemic lupus erythematosus, multiple sclerosis, and type 1 diabetes being the major sources of this impact on mortality.2 The autoimmune thyroid diseases, type 1 diabetes and rheumatoid arthritis are the most common of the autoimmune diseases (Table 25.1).3-5 Most autoimmune diseases disproportionately affect women. In the thyroid diseases, primary biliary cirrhosis, scleroderma, systemic lupus erythematosus, and Sjogren s syndrome, more than 85% of patients are female, but it is not known why the female predominance is so high in these specific diseases. 

Kojima, H., Nies, A.T., Konig,., Hagmann, W., Spring, H., Uemura, M., Fukui, H. and Keppler, D. (2003) Changes in the expression and localization of hepatocellular transporters and radixin in primary biliary cirrhosis. Journal of Hepatology, 39, 693-702. 

Occlusion of the bile duct (gallstone, primary biliary cirrhosis, pancreatic cancer) prevents conjugated bihrubin from leaving the liver. Conjt ated bihrubin increases in blood and may also appear in urine. Feces are light-colored. 

L. Serfaty, A. De Leusse, O. Rosmorduc, B. Desaint, J.-F. Flejou, O. Chaouilleres, R. E. Poupon and R. Poupon, Ursodeoxycholic acid therapy and the risk of colorectal adenoma inpatients with primary biliary cirrhosis An observational study, Hepatology, 2003, 38, 203. 

Questran contains colestyramine, a lipid-regulating drug that acts as a bile acid sequestrant it is also used in pruritus associated with partial biliary obstruction and primary biliary cirrhosis. 

Cirrhosis is among the top 10 causes of death in the Western World. This is largely the result of alcohol abuse, viral hepatitis and biliary diseases [75]. The causes for cirrhosis can be roughly divided into six categories ... 

Autoimmune diseases such as primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC) and autoimmune hepatitis,... 

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