[Primary ciliary dyskinesia (Pcd) in Austria]

INTRODUCTION: Primary ciliary dyskinesia (PCD) is a rare hereditary recessive disease with symptoms of recurrent pneumonia, chronic bronchitis, bronchiectasis, and chronic sinusitis. Chronic rhinitis is often the presenting symptom in newborns and infants. Approximately half of the patients show visceral mirror image arrangements (situs inversus). In this study, we aimed 1) to determine the number of paediatric PCD patients in Austria, 2) to show the diagnostic and therapeutic modalities used in the clinical centres and 3) to describe symptoms of children with PCD. PATIENTS, MATERIAL AND METHODS: For the first two aims, we analysed data from a questionnaire survey of the European Respiratory Society (ERS) task force on Primary Ciliary Dyskinesia in children. All paediatric respiratory units in Austria received a questionnaire. Symptoms of PCD patients from Vienna Children's University Hospital (aim 3) were extracted from case histories. RESULTS: In 13 Austrian clinics 48 patients with PCD (36 aged from 0-19 years) were identified. The prevalence of reported cases (aged 0-19 yrs) in Austria was 1:48000. Median age at diagnosis was 4.8 years (IQR 0.3-8.2), lower in children with situs inversus compared to those without (3.1 vs. 8.1 yrs, p = 0.067). In 2005-2006, the saccharine test was still the most commonly used screening test for PCD in Austria (45%). Confirmation of the diagnosis was usually by electron microscopy (73%). All clinics treated exacerbations immediately with antibiotics, 73% prescribed airway clearance therapy routinely to all patients. Other therapies and diagnostic tests were applied very inconsistently across Austrian hospitals. All PCD patients from Vienna (n = 13) had increased upper and lower respiratory secretions, most had recurring airway infections (n = 12), bronchiectasis (n = 7) and bronchitis (n = 7). CONCLUSION: Diagnosis and therapy of PCD in Austria are inhomogeneous. Prospective studies are needed to learn more about the course of the disease and to evaluate benefits and harms of different treatment strategies.

Immunosuppressive treatment for acquired haemophilia: current practice and future directions in Germany, Austria and Switzerland

Acquired haemophilia is an autoimmune disorder characterised by autoantibody formation against coagulation factor VIII. Immunosuppressive treatments including steroids, cytotoxic drugs, rituximab or combinations thereof have been used to eradicate autoantibodies. Very few prospective studies exist evaluating the use of these treatments. Here, we performed a survey among 73 physicians from 57 haemophilia treatment centres in order to describe current practice patterns and critical issues for future research in acquired haemophilia. The results demonstrate a high diversity of first- and second-line treatments. Factors influencing treatment decision were underlying disorder, severity of bleeding and inhibitor titre. Frequently used first-line treatments were steroids plus cyclophosphamide (44%) and steroids alone (11%). Second-line treatment was most often rituximab (30%), with or without steroids and/or cyclophosphamide. Most participants indicated to change from first- to second-line treatment after 4 weeks in case of failure to obtain partial remission (31%), continued bleeding (40%) or continued severe bleeding requiring bypass treatment (59%). Immunoadsorption was preferred for first- and second-line treatment by 10% and 9% of participants, respectively. These results highlight critical issues in the field. Open questions and directions for future research are discussed.

Appearance of red mark syndrome / cold water strawberry disease in Switzerland and Austria

Red mark syndrome (RMS) or cold water strawberry disease (CWSD) is a non-lethal skin disease of rainbow trout Oncorhynchus mykiss that is of high economic importance in the UK. The disease is temperature-dependent, with up to 60% morbidity at water temperatures below 15 degrees C. Although CWSD is horizontally transmissible, the aetiology is still unknown. Here we describe the first cases of RMS on the European mainland in the alpine regions of Switzerland and Austria. In Switzerland, morbidity remained around 1% after the first outbreak, whereas in Austria no further cases were diagnosed.

Floods of the Upper Danube River and Its Tributaries and Their Impact on Urban Economies (c. 1350–1600): The Examples of the Towns of Krems/Stein and Wels (Austria)

This paper examines the impact of disastrous and ‘ordinary’ floods on human societies in what is now Austria. The focus is on urban areas and their neighbourhoods. Examining institutional sources such as accounts of the bridge masters, charters, statutes and official petitions, it can be shown that city communities were well acquainted with this permanent risk: in fact, an office was established for the restoration of bridges and the maintenance of water defences and large depots for timber and water pipes ensured that the reconstruction of bridges and the system of water supply could start immediately after the floods had subsided. Carpenters and similar groups gained 10 to 20 per cent of their income from the repair of bridges and other flood damage. The construction of houses in endangered zones was adapted in order to survive the worst case experiences. Thus, we may describe those communities living along the central European rivers as ‘cultures of flood management’. This special knowledge vanished, however, from the mid-nineteenth century onwards, when river regulations gave the people a false feeling of security.

Subnational Patterns of Democracy in Austria, Germany and Switzerland

This article investigates the main political institutions in the sub-national democracies of Austria, Germany and Switzerland. It applies Lijphart’s approach to these German-speaking countries in Western Europe and expands it – following recent advances – by direct democracy. The main finding of the sub-national analysis is that, similar to Lijphart, two dimensions of democracy can be distinguished. While the first can be considered as the ‘consensual dimension’ of democracy, the second represents the ‘rules of the game’. Moreover, and in contrast to analyses at the national level, direct democracy does not constitute a dimension on its own, but forms an important element of consensus decision-making in the sub-national units at hand. Finally, based on cluster analysis three homogenous national clusters were found, but also one cluster with sub-national democracies from Germany and Austria that are more similar to one another than to other Länder within their respective federal states.