Search

Fabry's disease is an inherited lysosomal storage disorder characterized by the lack of enzyme alpha-galactosidase A (alpha-Gal A) which degrades globotriaosylceramides (Gb3) into products with lower molecular weight. The accumulation of Gb3 in different cell types is responsible for the variety of clinical manifestations. The renal function, estimated via proteinuria, hematuria and reduction of glomerular filtration rate (GRF), is heavily affected. Currently, substitution of alpha-Gal A remains the only therapeutic option for patients with Fabry's disease. Two products are approved for the treatment of Fabry's disease: agalsidase alfa and agalsidase beta. Both of these enzymes have shown a stabilization of renal function in various studies when evaluated by the creatinine clearance, estimated GFR, and serum creatinine. The pro gnosis has proven to be significantly better in cases of mild or moderate renal insufficiency from the baseline. For this reason, an early substitution of the lacking enzyme is necessary. Furthermore, enzyme replacement therapy (ERT) has proven efficient in reducing the amount of intracellular Gb3 and Gb3 in urine. Without treatment, an eGFR reduction of approximately 12 ml/min/year has been reported. After diverse studies of ERT, no significant correlation between enzyme substitution and improvement of patients' proteinuria could be shown. Furthermore, renoprotective drugs have not been consistently applied so far in the ERT trials. In any case, further studies to evaluate the long-term effect of ERT on the morbidity and mortality of patients with Fabry's disease are necessary.

There are indications of a link between right-wing extremist orientation and attitudes towards people with mental illnesses. Social distance is used to investigate this.In 2002, a representative survey was conducted among the German adult population (n = 2089). Social distance was measured using a question from the European values surveys. Right-wing extremist attitudes were measured with a scale developed by Niedermayer and Faller.Only a marginal link between right-wing extremism and social distance towards people with mental illnesses was found which, moreover, showed an inconsistent pattern.It would be premature to conclude from this "negative" finding that a right-wing extremist orientation has no influence on the attitude towards people with mental illnesses, especially in the light of only one form of discrimination being investigated here.

Citizens of Novosibirsk were questioned regarding their ideas about schizophrenia and depressive disorders, with one of the main concerns being a comparison with the ideas and attitudes of the German population.In 2002, a representative survey (N = 745) was conducted among the adult population of Novosibirsk, using a fully structured interview that has been used in previous surveys in Germany.Symptoms of schizophrenia were more frequently identified as an indication of mental disorder than were depressive symptoms. Psychosocial stress and biological influences were similarly frequently seen as the cause of schizophrenia, with the former prevailing among the respondents in the case of depressive symptoms. Psychotherapy was most frequently recommended as a treatment measure, also for schizophrenia. Supposing that no treatment measures will be taken, respondents assessed the prognosis for both mental disorders negatively. Conversely, interviewees gave a positive treatment prognosis.Respondents from Germany and Novosibirsk showed similar beliefs, especially as regards the importance of biological influences and psychosocial stress and the assessment of psychotherapy as compared to psychopharmacology. However, German and Russian respondents also held differing views: a comparatively strong tendency of the Russian population to consider mental disorders as self-inflicted - an important finding that could shape future anti-stigma programs.

The goal of this study is to investigate the stigma of mental illness from the perspective of the persons directly affected by it.210 patients with schizophrenia or major depression were questioned about anticipated and concrete stigmatization experiences, using a questionnaire especially developed for this study.Most of the patients expected negative reactions from the environment. Three quarters were convinced that their job application would be rejected when it became known that they are mentally ill. Almost two thirds felt apprehensive that others would avoid them due to their illness. There is hardly any difference between schizophrenia and depressive patients' assessment of stigmatization of mentally ill people. Concrete stigmatization experiences were most frequently reported in the domain of interpersonal interaction. Second comes the distorted picture of mentally ill people that is depicted in the media and experienced as hurtful by the patients. The obstacles to access social roles (partnership, work, etc.) perceived by the patients come third. Participants least frequently mentioned structural discrimination, i. e. disadvantages regarding psychiatric treatment or rehabilitation measures. Contrary to anticipated stigmatization, there are differences between the two diagnostic groups when it comes to concrete stigmatization experiences. Schizophrenia patients more frequently report that others would avoid contact with them and that the access to social roles was especially complicated for them. They also seemed to be more exposed to structural discrimination than depressive patients.Based on the results of this study, ways are discussed of how stigmatization and discrimination of mentally ill people can be reduced.