Cirrhosis, primary biliary

Primary biliary cirrhosis (PBC). Autoimmime liver disease that results in the destruction of bile ducts, leading to fibrosis and cirrhosis. Primary biliary cirrhosis-specific are antimitochondrial antibodies directed against proteins of the pyruvate dehydrogenase complex (mainly the E2 subunit). 

The conditions for which hepatic transplantation has been applied differ in pediatric and adult cases and are shown in Tables 1 and 2. 50% of the pediatric cases have been transplanted for biliary atresia.The majority of the other pediatric recipients have been transplanted for alpha 1 antitrypsin deficiency or chronic liver disease of unknown ethiology. In contrast, the leading conditions for which hepatic transplantation has been applied in adult recipients are postnecrotic cirrhosis, primary biliary cirrhosis and primary hepatic malignancy. 

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... 

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. 

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

Cholestatic liver diseases Primary biliary cirrhosis... 

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. 

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. 

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

UCDA may also be useful in primary biliary cirrhosis. 

Hepatic or severe renal dysfunction, including primary biliary cirrhosis preexisting gallbladder disease hypersensitivity to gemfibrozil. 

Colchicine [Antigout Agent/Colchicum Alkaloid] Uses Acute gouty arthritis prevention of recurrences familial Mediterranean fever primary biliary cirrhosis Action -1- Migration of leukocytes X leukocyte lactic acid production Dose Initial 0.6—1.2 mg PO, then 0.6 mg ql-2h until rehef or GI SE develop (max 8 mg/d) do not r eat for 3 d /U 1-3 mg, then 0.5 mg q6h until rehef (max 4 mg/d) do not rqjeat for 7 d Prophylaxis PO 0.6 mg/d or 3-4 d/wk ... 

Uses Hypertriglyceridemia, coronary heart Dz Action Fibric acid Dose 1200 mg/d PO bid 30 min ac am pm Caution [C, ] t Warfarin effect, sulfony-lureas t risk of myopathy w/ HMG-CoA reductase inhibitors X effects w/ cyclosporine Contra Renal/hepatic impair (SCr >2.0 mg/dL), gallbladder Dz, primary biliary cirrhosis, use w/ repaglinide (i glucose) Disp Tabs 600 mg SE Cholelithiasis, GI upset Interactions t Effects OF anticoagulants, sulfonylureas t risk of rhabdomyolysis W/ HMG-CoA reductase inhibitors X effects W/ rifampin X effects OF cyclosporine EMS t Effects of anticoagulants OD May... 

We (K1) attempted to develop a noncompetitive assay based on the anti-idiotype antibodies for a conjugated bile acid metabolite, ursodeoxycholic acid 7-A-acetyl-glucosaminide (UDCA 7-NAG), which is expected to serve as a diagnostic index for an autoimmune disease, primary biliary cirrhosis. In our assay, the hapten UDCA 7-NAG, a /3-type antibody, and a biotin-labeled a-type antibody were simultaneously added to a microtiter plate coated with an F(ab )2 fragment of a specific anti-UDCA 7-NAG antibody, then incubated at room temperature for 8 h. Bound biotin was then detected with HRP-labeled streptavidin, whose enzyme activity was measured using o-phenylenediamine/H202 as a substrate. This noncompetitive assay system provided a subfemtomole-order sensitivity (detection limit 118 amol) that was 7 times lower than the competitive immunoassay using the same anti-hapten antibody (K2), even with a common colorimetric detection (Fig. 13). Somewhat improved specificity was also obtained namely, better... 

Jorgensen R, Angnlo P, Dickson ER, Lindor KD. Re-snlts of long-term nrsodiol treatment for patients with primary biliary cirrhosis. Am J Gastroenterol 2002 97 2647-50. 

Primary biliary cirrhosis the underlying cause is unknown. The disease is particularly common in older women. Associated itching is helped by... 

Gluud C, Christensen E. Ursodeoxycholic acid for primary biliary cirrhosis. Cochrane Database Syst Rev 2001. 

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