Decreased serum antioxidant capacity in patients with Wilson disease is associated with neurological symptoms.

Background & Aims: Wilson disease (WD) is an inherited disorder of copper disposition caused by an ATP7B transporter gene mutation, leading to copper accumulation in predisposed tissues. In addition to a genetic predisposition, other factors are likely to contribute to its clinical manifestation. The aim of the study was to assess whether oxidative stress affects the phenotypic manifestation of WD.

Long-term follow-up of Wilson disease: natural history, treatment, mutations analysis and phenotypic correlation.

Background And Aims: Wilson disease (WD) is an inherited disorder of copper metabolism. When treated, the outcome can be excellent, although the long-term survival has yet to be well documented. The aim of this study was to describe the long-term outcome of a cohort of patients with WD and to assess those factors affecting the phenotypic manifestation of WD.

Double-blind randomized multicenter study comparing the efficacy and safety of 10-day to 5-day terlipressin treatment of bleeding esophageal varices.

Background/aims: The aim of the study was to compare the efficacy and safety of 5-day terlipressin treatment of bleeding esophageal varices to 10-day treatment period.

Methodology: Of 25 patients with variceal bleeding, 15 were randomized to receive terlipressin for day 1-5 and placebo for day 6-10 (Group A), and 10 to receive terlipressin for day 1-10 (Group B).

Results: The bleeding was stopped in 24 patients (96%).

The efficacy of terlipressin in comparison with albumin in the prevention of circulatory changes after the paracentesis of tense ascites--a randomized multicentric study.

Background/aims: Postparacentesis circulatory dysfunction is the most severe complication of ascites paracentesis. The aim of our study was to compare the standard treatment with the administration of a vasoconstrictor terlipressin.

Methodology: Forty-nine patients treated by paracentesis due to tense ascites were randomized for the treatment with albumin (8g/L of removed ascites) or terlipressin (1 mg every four hours for 48 hours).

Estrogen-induced cholestasis results in a dramatic increase of b-series gangliosides in the rat liver.

Hepatic ganglioside composition was investigated in normal and cholestatic Wistar rats. Cholestasis was induced by 17alpha-ethinylestradiol (EE; 5 mg/kg body weight s.c.

Changes in GM1 ganglioside content and localization in cholestatic rat liver.

(Glyco)sphingolipids (GSL) are believed to protect the cell against harmful environmental factors by increasing the rigidity of plasma membrane. Marked decrease of membrane fluidity in cholestatic hepatocytes was described but the role of GSL therein has not been investigated so far. In this study, localization in hepatocytes of a representative of GSL, the GM1 ganglioside, was compared between of rats with cholestasis induced by 17alpha-ethinylestradiol (EE) and vehicle propanediol treated or untreated animals.

Effect of carvedilol on portal hypertension depends on the degree of endothelial activation and inflammatory changes.

Objective: Bleeding from esophageal varices is a major complication of liver cirrhosis. Non-selective beta-blockers exert an influence on the functional part of portal hypertension, thereby reducing the risk of bleeding. Direct measurement of this functional part is not possible; nevertheless, pro-inflammatory markers as well as parameters of endothelial dysfunction might serve as surrogate markers.

Hepatic vein stenosis and inferior vena cava stenosis in a patient with chronic obstructive pulmonary disease.

The case is presented of a patient with stenosis of the intrahepatic portion of the inferior vena cava and the terminal part of hepatic veins, a state manifesting itself in ascites resistant to treatment. The case was originally seen as decompensated cirrhosis of the liver. Once properly diagnosed, it was successfully treated with angioplasty and subsequent anticoagulation.

Effect of drugs on cholesterol crystallization in an artificial bile model and relation of this effect to drug binding to albumin.

Substances that can affect the crystallization of cholesterol from human bile and consequently the gallstone formation have been given considerable attention. We improved the model system for testing cholesterol crystallization-affecting activity (promoting or inhibiting) of substances and used it for some drugs that are excreted into bile. Besides other factors natural lipid-protein complexes isolated from the native human bile have been shown to be responsible for nucleation and fast crystal growth in cholesterol supersaturated model bile.

Gilbert syndrome and ischemic heart disease: a protective effect of elevated bilirubin levels.

Background: Oxidation processes play an important role in atherogenesis. Bilirubin IXalpha is recognised as a potent antioxidant. In the present study, we assessed the role of elevated serum bilirubin levels in the prevention of ischemic heart disease (IHD).