Serum selenium concentration in patients with Wilson's disease.

BACKGROUND/AIMS: Wilson's disease is a genetically determined disorder of copper metabolism in the liver. Due to the toxic accumulation of this trace element, body organs are damaged by free radical generation, lipid peroxidase and inhibition of synthesis of some proteins. Behavior of anti-oxidative factors in Wilson's disease has not been completely evaluated yet. The aim of the study was to assess blood serum concentrations of selenium in patients with Wilson's disease. METHODOLOGY: Twenty-five patients with Wilson's disease and 30 healthy volunteers, constituting a control group were included in the study. The patients were in good clinical condition. In all the subjects blood serum concentrations of selenium were tested using the atomic absorption spectroscopy, hydride generation method. RESULTS: Selenium concentrations in the blood serum of the patients and healthy controls did not show statistical differences between both groups. Correlations between selenium concentrations and biochemical parameters: activity of alanine and aspartate aminotransferase, alkaline phosphatase, gamma-glutamyltranspeptidase, concentration of bilirubin, albumin and gamma globulin, international normalized prothrombin index as well as serum copper, ceruloplasmine and 24-h urine copper excretion were assessed. Statistically significant correlation was found only between selenium concentration and aspartate aminotransferase activity. No statistically significant differences between selenium concentrations in the serum of patients with different forms of Wilson's disease were found. CONCLUSIONS: On the basis of the results obtained in the study it can be assumed that in treated patients with Wilson's disease the antioxidant status measured as serum selenium concentration is comparable to healthy controls.

Treatment of Wilson's disease.

On the basis of literature review and own experience we presented the method of treatment of Wilson's disease. Causative treatment has been impossible so far, although gene therapy could be real in the future. Nowadays the principle of treatment is the elimination of the excess of easily mobilized copper by chelating agents or blocking the intestinal absorption of copper. Chelation therapy, aimed at mobilizing copper from the affected organs and promoting its excretion in the urine or stool is the most important. The major chelating agent is d-penicillamine, which is quite effective but not without some side effects. Alternative chelating agents such as trientine and tetrathiomolybdate have also been successfully employed. Zinc salts are also of therapeutic value. They promote copper excretion by inducing the synthesis of metallothionein in the intestine, thereby blocking copper absorption from the gut. Zinc salts have almost no side effects. They cannot be used as an initial treatment, but are very effective for maintenance therapy. The chelation therapy is ineffective in patients with acute liver failure with encephalopathy and hemolysis. In these cases, liver transplantation is the only hope for survival. Liver transplantations in patients with dominating psychoneurological symptoms are open to discussion.

Results of treatment of Wilson's disease--own observations.

BACKGROUND: Causative treatment of genetically determined Wilson's disease (WD) has been impossible so far, although gene therapy could be real in the future. Nowadays the principle of treatment is the elimination of the excess of easily mobilized copper, bound by chelating agents, the most important of which is d-penicillamine, through the kidneys. Blocking of the intestinal absorption of copper by administration of zinc preparations, which additionally induce hepatic metallothionein synthesis, is also possible. The aim of our study was to present own observations and results of treatment of Wilson's disease. MATERIAL/METHODS: During the last 16 years, we have observed 33 patients aged 13-60 (mean age 27 years) with various forms of WD. The studied group consisted of 11 females and 21 males, admitted to hospital or seen at the Specialistic Outpatient Department of Hepatology with various diagnoses. In addition to standard laboratory tests, the levels of ceruloplasmin, serum and urine copper, as well as the activity of some hepatic enzymes, proteins and HBV/HCV infection markers were determined. The patients were also examined by a neurologist and an ophthalmologist, with psychiatric consultation if necessary. Taking into account the overall clinical presentation, the patients were divided into the following groups according to the form of the disease: fulminant, acute, hepatic, hepatic with neurological and psychiatric symptoms, neuropsychiatric, asymptomatic. RESULTS: All the patients were initially treated with d-penicillamine. In most of them, no side effects were observed. The treatment was continued according to the levels of copper excreted with urine (for 10 years at the longest). After obtaining clinical improvement with reduced amount of copper excreted with 24-h urine, we tapered d-penicillamine doses or even discontinued the drug, introducing zinc preparations. In asymptomatic carriers, zinc preparations were used throughout the period of treatment. CONCLUSIONS: Early institution of chelation treatment is associated with good prognosis both in hepatic and neurological forms of WD. Zinc preparations are effective and safe in neurological and oligosymptomatic forms of the disease.

Serum selenium levels in alcoholic liver disease.

BACKGROUND: The pathomechanism of liver damage in chronic alcoholic liver disease has not been fully elucidated yet. It seems undoubted that one of the mechanisms of alcohol-induced liver damage involves free radical reactions leading to peroxidation of proteins and lipids. The most important defense mechanisms are associated with the activity of antioxidative enzymes, among which glutathione peroxidase (GSH-Px), belonging to so-called 'free radical scavengers' should be mentioned. Selenium, regarded as a bioelement, is present in GSH-Px. Involved in numerous redox reactions, it belongs to the factors protecting the organism from oxidative shock. The aim of the study was to determine the selenium levels in blood serum of chronic alcohol abusers and to find potential correlations with the parameters of liver damage. MATERIAL/METHODS: The study was carried out in a group of 25 subjects (21 males, 4 females), treated in the Clinic or Outpatient Department of Hepatology for chronic alcoholic liver disease. At the time of the study, the patients had abstained from drinking for a period from one month to a year. Selenium was determined with atomic absorption spectroscopy method, using the hydride generation technique. The control group consisted of 11 males and 7 females. RESULTS: Statistical analyses of the control group indicated a significantly higher blood serum selenium level in males than in females. The patients demonstrated elevated aminotransferase activity, normal Falk, markedly increased GGTP. Mean INR was 1.4 and albumin concentration 3.3 g/l. Blood serum selenium levels in male patients were significantly lower in comparison with normal values. The analysis of correlations between some liver function parameters and selenium levels demonstrated a positive correlation between the levels of albumin and selenium. Serum selenium level was inversely proportional to ALAT activity. No correlations between selenium levels and INR levels, or GGTP activity were found. CONCLUSIONS: 1. Serum selenium levels differ in male and female populations. In healthy men, the level of Se in the serum is higher. 2. Antioxidative activity measured by serum Se level is low in men with chronic alcoholic liver disease (during the abstinence period). 3. Increased selenium level in the hair may indicate the presence of certain antioxidative reserve, which requires further studies.