Antioxidant Capacity Is Decreased in Wilson's Disease and Correlates to Liver Function.

The metabolic disorder Wilson's disease (WD) is caused by copper accumulation in the tissues due to a biallelic pathogenic mutation of the gene ATP7B, encoding intracellular copper transporter ATPase-7B. As copper is a redox active metal; aberrations in its homeostasis may create favourable conditions for superoxide-yielding redox cycling and oxidative damage to the cells. We tried to characterise antioxidant defence in WD patients and to evaluate whether it is related to liver function. The blood glutathione concentration, the activity of manganese-SOD (MnSOD), catalase (Cat), glutathione peroxidase, and glutathione S-transferase glutathione (GST), and serum antioxidant potential (AOP-450) were measured in WD treatment-naive patients and healthy controls and correlated with clinical data. The blood glutathione concentration, the activity of MnSOD, Cat, glutathione peroxidase, and GST and AOP-450 are significantly decreased in WD patients. There was a positive correlation of AOP-450 with AST. Moreover, the Cat and GST activity as well as AOP-450 strongly correlated with parameters of synthetic liver function. MnSOD activity correlated positively with ALT and AST.The blood glutathione concentration, the activity of MnSOD, Cat, glutathione peroxidase, and GST and AOP-450 are significantly decreased in WD patients. There was a positive correlation of AOP-450 with AST. Moreover, the Cat and GST activity as well as AOP-450 strongly correlated with parameters of synthetic liver function. MnSOD activity correlated positively with ALT and AST. Liver injury in course of WD is linked with decreased antioxidant capacity.

Coping strategies observed in women with rheumatoid arthritis.

When faced with a chronic disease such as rheumatoid arthritis, the patient attempts to cope with the stressful situation by applying coping strategies. The main aim of rheumatoid treatment is not only improving health but also increasing the quality of life. The research objective was to determine the relationship among socio demographic factors, duration of the disease and its associated ailments, attitude to the disease, self-assessment of one's knowledge of RA, and the application of coping strategies in stressful situations by women with rheumatoid arthritis. The study involved 193 patients of the Clinic of Rheumatology and Systemic Connective Tissue Diseases, and the Rheumatology Unit of the Specialist Outpatient Clinic of the Independent Public Teaching Hospital No. 4 in Lublin, from November 2016 - June 2017. The Coping Orientations to Problems Experienced Inventory (COPE) Questionnaire and an author's Original Questionnaire were used in the study. Analysis of variance (ANOVA) and Tukey's range test were applied for statistical analysis. A p-value<0.05 defined the statistical differences. Analysis was performed using the commercial SPSS Statistics 19 software (IBM Corp., Armonk, NY, USA). The respondents usually use instrumental social support (11.5±1.7), focus on and vent emotions (11.4±1.9), use emotional social support (11.4±1.8), employ active coping (11.4±2.1) and positive reinterpretation and growth (11.2±1.8), and least often rely on their sense of humour (5.5±1.4) or use alcohol or drugs (5±1.1). The factors which determine the types of strategy used are age, education, the duration of the disease, ailments experienced, and attitude towards the disease. Those respondents who declared a greater knowledge of RA more often applied positive reinterpretation and growth, and more rarely used alcohol or drugs. In the treatment and rehabilitation processes it is important to reinforce in the patient positive expectations for the treatment, seek advantages and benefits in one's present health status, and educate patients about the disease, its therapy and appropriate coping strategies.

Treatment with D-penicillamine or zinc sulphate affects copper metabolism and improves but not normalizes antioxidant capacity parameters in Wilson disease.

Copper accumulation in tissues due to a biallelic pathogenic mutation of the gene: ATP7B results in a clinical phenotype known as Wilson disease (WD). Aberrations in copper homeostasis can create favourable conditions for superoxide-yielding redox cycling and oxidative tissue damage. Drugs used in WD treatment aim to remove accumulated copper and normalise the free copper concentration in the blood. In the current study the effect of decoppering treatment on copper metabolism and systemic antioxidant capacity parameters was analyzed. Treatment naïve WD patients (TNWD) (n = 33), those treated with anti-copper drugs (TWD) (n = 99), and healthy controls (n = 99) were studied. Both TNWD and TWD patients characterised with decreased copper metabolism parameters, as well as decreased total antioxidant potential (AOP), glutathione (GSH) level, activity of catalase, glutathione peroxidase (GPx), and S-transferase glutathione, compared to controls. TWD patients had significantly lower copper metabolism parameters, higher total AOP and higher levels of GSH than TWD individuals; however, no difference was observed between these two patient groups with respect to the rest of the antioxidant capacity parameters. Patients who had undergone treatment with D-penicillamine or zinc sulphate did not differ with respect to copper metabolism or antioxidant capacity parameters, with the exception of GPx that was lower in D-penicillamine treated individuals. These data suggest that anti-copper treatment affects copper metabolism as well as improves, but does not normalize, natural antioxidant capacity in patients with WD. We propose to undertake studies aimed to evaluate the usefulness of antioxidants as well as selenium as a supplemental therapy in WD.

[Oxidative stress and natural antioxidant mechanisms: the role in neurodegeneration. From molecular mechanisms to therapeutic strategies]. / Stres oksydacyjny i naturalne mechanizmy antyoksydacyjne ­ znaczenie w procesie neurodegeneracji. Od mechanizmów molekularnych do strategii terapeutycznych.

A lot of evidence exists that oxidative stress is the primary cause of neurodegeneration. Neurons are more susceptible to oxidative damage than other cells due to their high oxygen consumption, low activity of antioxidant enzymes, elevated concentration of polyunsaturated fatty acids in the cell membrane, high number of mitochondria, unfavorable space/volume ratio and vicinity of microglia cells which are likely to produce increased amounts of superoxide radical. Moreover, the tendency to accumulate transition metals in the brain creates a higher probability of Fenton's reaction occurring, a product of which is a hydroxyl radical. Lower activities of natural antioxidants as well as higher concentrations of markers of oxidative damage to proteins, lipids and DNA were observed in patients with neurodegenerative diseases in relation to healthy individuals. There is a lot of research being conducted to develop effective and safe antioxidants that would be useful in the therapy or prevention of neurodegenerative diseases.