Nephrosis in two siblings with infantile sialic acid storage disease

The diagnosis of infantile sialic acid storage disease (ISSD) was established in two siblings on the basis of typical clinical signs and the biochemical findings of hyperexcretion and intracellular storage of free sialic acid. A severe, steroid resistant nephrosis occurred in both siblings. The activities of lysosomal enzymes, including sialidase, were normal. A combined detection method for sialic acids with Limax flavus agglutinin labelling and phosphotungstic acid staining showed severely alterated sialic acid components in epithelial kidney cells and indicate a causal relationship between the nephrosis and the underlying biochemical defect. Further observations of ISSD patients with ren…

Poster contributions

Mössbauer Spectroscopic Evidence of Angle-Dependent Intersystem Crossing in LiNbO3:Fe3+

M\"ossbauer emission spectra of LiNb${\mathrm{O}}_{3}$:$^{57}\mathrm{Co}$ single crystals in magnetic fields up to 5 T at 4.2 K show initial populations of the $^{6}A_{1\mathrm{g}}$ Zeeman substates of ${\mathrm{Fe}}^{3+}$ which depend strongly on the angle between the crystallographic $c$ axis and the magnetic field. This is interpreted in terms of a crystal-field effect on excited states which influences the initial populations of the Zeeman sublevels of the $^{6}A_{1\mathrm{g}}$ ground term after the electron-capture decay of $^{57}\mathrm{Co}$. An intersystem crossing process due to orbit-lattice interaction can fairly well explain the angular dependence.

Large underground, liquid based detectors for astro-particle physics in Europe: scientific case and prospects

This document reports on a series of experimental and theoretical studies conducted to assess the astro-particle physics potential of three future large-scale particle detectors proposed in Europe as next generation underground observatories. The proposed apparatus employ three different and, to some extent, complementary detection techniques: GLACIER (liquid Argon TPC), LENA (liquid scintillator) and MEMPHYS (\WC), based on the use of large mass of liquids as active detection media. The results of these studies are presented along with a critical discussion of the performance attainable by the three proposed approaches coupled to existing or planned underground laboratories, in relation to…