Aeolian sedimentation in the Rhine and Main area from the Late Glacial until the Mid-Holocene

Äolische Sedimente (sandiger Löss, Flugsand) wurden durch pedologische und geochronologische Methoden (OSL) mit dem Ziel untersucht, hieraus Aussagen zur stratigraphischen Abfolge und zum Ablagerungsalter zu gewinnen und diese Ergebnisse auf die Ergebnisse der archäologischen Ausgrabung zu beziehen. Die Ergebnisse zeigen, dass die Ablagerung des Lösses im Spätglazial auf einer älteren Lage von Windkantern erfolgte und dieser am Ende des Pleistozäns von äolischem Sand überdeckt wurde. Untersuchungen der fundführenden Schichten ergaben eine mittelholozäne Datierung (6.9 ka), die mit einer lokalen Störung durch äolische Sedimentumlagerungen in Folge anthropogener Landschaftsveränderungen während des Neolithikums erklärt werden. Diese Untersuchungsergebnisse werden vor dem Hintergrund vergleichbarer äolischer Stratigraphien und Fundplätz im Rhein-Main-Gebiet diskutiert.researc

Ein technisches Informationssystem zur verbesserten betrieblichen Überwachung des Kernkraftwerkes Saporoshje/Ukraine

In order to improve the operational surveillance of a VVER-1000 unit of the Ukrainian nuclear power plant Zaporosh´ye a technical monitoring system has been specified. The system will enable the state regulatory and supervisory bodies to survey the unit operation independently of operators to assess its safety status, and to impose appropriate conditions. Due to its up-to-date configuration the system provides early indication of any operational incident and emission of radioactive materials connected. Based on the system an immediate warning in mergency situations is possible as well as an effective emergency management. For this purpose 49 different operational parameters of the unit, 18 radiological parameters of the unit and the plant site and 6 meteorological parameters are monitored. The monitoring concept and its technical realization are described

Clinical and morphological phenotype of the filamin myopathy: a study of 31 German patients

Mutations in the filamin C gene (FLNC) cause a myofibrillar myopathy (MFM), morphologically characterized by focal myofibrillar destruction and abnormal accumulation of several proteins within skeletal muscle fibres. We studied 31 patients from four German families to evaluate the phenotype of filaminopathy. All patients harboured the same p.W2710X mutation in FLNC. Haplotype analysis suggested a founder mutation in these German filaminopathy families. The mean age at onset of clinical symptoms was 44 +/− 6 years (range, 24-57 years). Slowly progressive muscle weakness was mostly pronounced proximally, initially affecting the lower extremities and involving the upper extremities in the course of disease progression, similar to the distribution of weakness seen in limb-girdle muscular dystrophies (LGMD). Patients frequently developed respiratory muscle weakness. About one-third of the patients showed cardiac abnormalities comprising conduction blocks, tachycardia, diastolic dysfunction and left ventricular hypertrophy indicating a cardiac involvement in filaminopathy. Serum creatine kinase levels varied from normal up to 10-fold of the upper limit. Magnetic resonance imaging studies showed a rather homogenous pattern of muscle involvement in the lower extremities differing from that in other types of MFM. Myopathological features included perturbation of myofibrillar alignment, accumulation of granulofilamentous material similar to that seen in primary desminopathies and abnormal intracellular protein deposits typical of MFM. Decreased activities of oxidative enzymes and fibre hypertrophy seem to be early features, whereas dystrophic changes were present in advanced stages of filaminopathy. Rimmed vacuoles were detected in only a few cases. The intracellular aggregates were composed of a variety of proteins including filamin C, desmin, myotilin, Xin, dystrophin and sarcoglycans. Therapy is so far limited to symptomatic treatment. The German filaminopathy cohort, the largest group of patients studied so far, shares phenotypic features with LGMD and presents with characteristic histopathological findings of MF