Treatment with ursodeoxycholic acid is associated with weight gain in patients with primary biliary cirrhosis.

Background: Ursodeoxycholic acid (UDCA) is the established treatment of primary biliary cirrhosis (PBC) and is a safe and well-tolerated medication. Nevertheless, patients often anecdotally complain of weight gain while on this drug.

Goals: We compared weight changes in patients treated with UDCA and those on placebo to characterize this potential side effect.

Primary biliary cirrhosis.

Primary biliary cirrhosis is a chronic cholestatic liver disease of adults. This disorder is characterised histologically by chronic non-suppurative destruction of interlobular bile ducts leading to advanced fibrosis, cirrhosis, and liver failure. The precise aetiopathogenesis of primary biliary cirrhosis remains unknown, although dysregulation of the immune system and genetic susceptibility both seem to be important.

Primary sclerosing cholangitis in children: a long-term follow-up study.

Primary sclerosing cholangitis (PSC) is increasingly diagnosed in children and adolescents, but its long-term prognosis remains uncertain. The aim of this longitudinal, cohort study was to determine the long-term outcome of children with PSC. Fifty-two children with cholangiography-proven PSC (34 boys and 18 girls; mean age 13.

Combined analysis of the effect of treatment with ursodeoxycholic acid on histologic progression in primary biliary cirrhosis.

Background/aims: This study aimed at evaluating the effect of ursodeoxycholic acid (UDCA) treatment on histologic progression in primary biliary cirrhosis (PBC).

Methods: Using combined individual histologic findings from four clinical trials, we selected the patients in whom paired liver-biopsy specimens were available with a time interval of about 36 months between biopsies. A total of 367 patients were selected (UDCA: 200 vs.

Is there a role for liver biopsy in primary sclerosing cholangitis?

Objective: Liver biopsies are performed in patients with primary sclerosing cholangitis (PSC) to stage disease and to rule out coexisting liver disease. The purpose of this study was to examine how often routine liver biopsies provide important information in patients with PSC.

Methods: We reviewed the charts of 138 patients with a cholangiographic diagnosis of PSC to determine whether information from liver biopsy had an impact on clinical management.

Safety and efficacy of estrogen therapy in preventing bone loss in primary biliary cirrhosis.

Objective: Estrogen therapy has been found to be useful in the treatment of postmenopausal osteoporosis. However, concern about its potential for worsening cholestasis has limited its use in postmenopausal women with primary biliary cirrhosis. The aim of the present study was to determine the safety as well as the efficacy of estrogen replacement therapy with respect to bone mass in postmenopausal women with primary biliary cirrhosis.

Ursodeoxycholic acid as a chemopreventive agent in patients with ulcerative colitis and primary sclerosing cholangitis.

Background & Aims: Ursodeoxycholic acid (UDCA) has shown effectiveness as a colon cancer chemopreventive agent in preclinical studies. In addition, a recent report suggests that it also may decrease the risk for developing colorectal dysplasia in patients with ulcerative colitis (UC) and primary sclerosing cholangitis (PSC). We sought to evaluate the effect of UDCA on colorectal neoplasia in a group of patients with UC and PSC enrolled in a randomized, placebo-controlled trial.

Treatment Options for Primary Biliary Cirrhosis and Primary Sclerosing Cholangitis.

Primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) are chronic cholestatic liver diseases that affect 0.5 to 40 per 100,000 and 1 to 6 per 100,000 Americans, respectively. Prompt recognition and management of the clinical manifestations of these diseases is essential for the patients' well-being and ultimate outcome.

Methotrexate therapy for primary biliary cirrhosis.

Objective: Preliminary data suggested possible benefits of methotrexate in primary biliary cirrhosis. We assessed the effectiveness of methotrexate use in primary biliary cirrhosis and its tolerance in patients with this disease.

Methods: A total of 110 primary biliary cirrhosis patients began methotrexate 15 mg/wk; for most, ursodeoxycholic acid was added during the study.

A novel incentive system for faculty in an academic medical center.

The need to contain health care costs has led some physicians to become salaried employees of health care organizations. However, the use of nonfinancial incentives for physicians in such an environment has not been broadly explored. The authors describe a novel incentive system that is designed to promote continuing high-quality care and to increase patient access to health care while enhancing clinical and academic productivity and physician satisfaction.

Treatment of non-alcoholic steatohepatitis.

Treatment of patients with non-alcoholic steatohepatitis (NASH) has typically been focused on the management of associated conditions such as obesity, diabetes mellitus and hyperlipidaemia. NASH associated with obesity may resolve with weight reduction, although the benefits of weight loss have been inconsistent. Appropriate control of glucose and lipid levels is always recommended, but is not always effective in reversing the liver condition.

Results of long-term ursodiol treatment for patients with primary biliary cirrhosis.

Objective: When ursodeoxycholic acid (UDCA) is used for the treatment of primary biliary cirrhosis, it has been associated with biochemical improvement, histological stability, reduced risk of esophageal varices, and increased survival free of transplantation. There is limited information available about the long-term outcome of these patients with primary biliary cirrhosis on UDCA treatment. To address this, we reviewed the long-term results from patients enrolled in our original randomized study with up to 12 yr of follow-up.

Pregnancy in a patient with primary sclerosing cholangitis.

Primary sclerosing cholangitis (PSC) is a chronic cholestatic syndrome that is characterized by fibrosing inflammatory destruction of intra- and/or extrahepatic biliary ducts. The etiology is unknown and there is no known effective treatment. The course of PSC is quite variable; however, the disease is typically slowly progressive.

Small-duct primary sclerosing cholangitis: a long-term follow-up study.

Some patients with inflammatory bowel disease (IBD) have chronic cholestasis and hepatic histology compatible with primary sclerosing cholangitis (PSC) but normal findings on cholangiography. These patients with small-duct PSC have remained largely unstudied. Our aim was to determine the prevalence and long-term outcomes of patients with small-duct PSC.

Overlap of autoimmune hepatitis and primary biliary cirrhosis: an evaluation of a modified scoring system.

Objective: The coexistence of autoimmune hepatitis (AIH) with primary biliary cirrhosis (PBC) as an overlap syndrome has been previously described. The ability to detect AIH overlap with a revised version of the International Autoimmune Hepatitis Group (IAHG) scoring system, however, remains unknown. Our specific aim was to evaluate the revised IAHG scoring system and its ability to identify AIH overlap in PBC.

In primary sclerosing cholangitis, gallbladder polyps are frequently malignant.

Objective: The management of gallbladder polyps/masses in patients with primary sclerosing cholangitis (PSC) (i.e., cholecystectomy vs observation) remains problematic.

Non-alcoholic fatty liver disease.

Non-alcoholic fatty liver disease (NAFLD) is a chronic liver disease that affects a high proportion of the world's population. Insulin resistance and oxidative stress play a critical role in the pathogenesis of NAFLD. Clinical, biochemical and imaging studies are of value in the diagnostic evaluation of patients with NAFLD, but liver biopsy remains the most sensitive and specific means of providing important diagnostic and prognostic information.

Pirfenidone in the treatment of primary sclerosing cholangitis.

Our aim was to evaluate the safety and assess the efficacy of pirfenidone, an antifibrotic drug, in patients with primary sclerosing cholangitis (PSC). Twenty-four patients with PSC were enrolled in this pilot study. Oral pirfenidone, 2400 mg daily, was given for one year.

Primary biliary cirrhosis with additional features of autoimmune hepatitis: response to therapy with ursodeoxycholic acid.

Patients with primary biliary cirrhosis (PBC) may have additional features of autoimmune hepatitis (AIH). Corticosteroids usually contraindicated in PBC have been advocated for these patients. Patients with antimitochondrial antibody (AMA)-positive PBC from two previous randomized, controlled trials were assessed for features of AIH.

Human leukocyte antigen Class II associations in serum antimitochondrial antibodies (AMA)-positive and AMA-negative primary biliary cirrhosis.

Background/aims: An association of Class II HLA-DR8 antigen is reported in patients with serum antimitochondrial antibodies (AMA)-positive primary biliary cirrhosis (PBC); no information exists as to an association with AMA-negative PBC. We compared the frequency of HLA Class II genes in AMA-positive and AMA-negative PBC patients and healthy controls.

Methods: Genomic DNA was extracted from the blood of 154 AMA-positive and 26 AMA-negative Caucasian PBC patients and from 216 healthy Caucasian controls and tested for the alleles at two HLA Class II loci, DRbeta1 and DQbeta1.