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  • 1985-1989  (4)
  • 1986  (4)
  • 1
    ISSN: 1432-0533
    Keywords: Severe sialidosis ; α-Neuraminidase deficiency ; Neuropathology ; Congenital ascites ; Nephrosialidosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A 56-day-old infant with α-neuraminidase deficiency, whose clinical features included severe edema of extremities and ascites which resembled those in severe infantile sialidosis, was autopsied. Perforation, whose pathogenesis was unclear, was found on the descending portion of the duodenum. Light and electron microscope studies showed that neurons in the cerebral and cerebellar corticies, and the thoracic spinal cord contained membrane-bound vacuoles but no membranous cytoplasmic bodies. Zebra bodies were found only in the neurons of the spinal cord. The neurons in the paraganglion and in the Auerbach's myenteric plexus were also distended with numerous membrane-bound vacuoles. Hepatocytes, endothelial cells and Kupffer cells in the liver and glomerular and tubular epithelial cells in the kidney were swollen with a number of vacuoles although the patient showed none of the clinical features of renal involvement. These pathological changes were similar to those in nephrosialidosis reported by Le Sec et al. [Arch Fr Pediatr 35:819–829 (1978)].
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1089-7623
    Source: AIP Digital Archive
    Topics: Physics , Electrical Engineering, Measurement and Control Technology
    Notes: The soft x-ray continuum radiation in tokamak fusion test reactor (TFTR) low-density neutral-beam discharges can be much lower than its theoretical value obtained by assuming a corona equilibrium. This reduced continuum radiation is caused by an ionization equilibrium shift toward lower states, which strongly changes the value of the average recombination coefficient of metallic impurities γ¯, even for only slight changes in the average charge Z¯. The primary agent for this shift is the charge exchange between the highly ionized impurity ions and the neutral hydrogen, rather than impurity transport, because the central density of the neutral hydrogen is strongly enhanced at lower plasma densities with intense beam injection. In the extreme case of low-density, high neutral-beam power TFTR operation (energetic ion mode) the reduction in γ¯ can be as much as one-half to two-thirds. We calculate the parametric dependence of γ¯ and Z¯ for Ti, Cr, Fe, and Ni impurities on neutral density (equivalent to beam power), electron temperature, and electron density. These values are obtained by using either a one-dimensional impurity transport code or a zero-dimensional code with a finite particle confinement time. As an example, we show the variation of γ¯ and Z¯ in different TFTR discharges.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    [S.l.] : American Institute of Physics (AIP)
    Review of Scientific Instruments 57 (1986), S. 2151-2153 
    ISSN: 1089-7623
    Source: AIP Digital Archive
    Topics: Physics , Electrical Engineering, Measurement and Control Technology
    Notes: Metal impurity concentrations are measured by the pulse-height-analyzer (PHA) diagnostic from Kα x-ray peak intensities by use of an averaged excitation rate 〈σv〉. Low-Z impurity concentrations are inferred from the continuum enhancement (relative to a pure plasma) minus the enhancement due to metals. Since the PHA does not resolve lines from different charge states, 〈σv〉 is a weighted sum of rates; coronal equilibrium is usually assumed. The 〈σv〉 used earlier omitted the intercombination and forbidden lines from the dominant heliumlike state. The result was an overestimate of metals and an underestimate of low-Z impurities in cases where metals were significant. Improved values of 〈σv〉 using recent calculations for H-, He-, and Li-like Fe range from 10% to 50% larger than the earlier rates and yield metal concentrations in better agreement with those from vacuum ultraviolet (VUV) spectroscopy.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Journal of materials science 5 (1986), S. 1099-1100 
    ISSN: 1573-4811
    Source: Springer Online Journal Archives 1860-2000
    Topics: Mechanical Engineering, Materials Science, Production Engineering, Mining and Metallurgy, Traffic Engineering, Precision Mechanics
    Type of Medium: Electronic Resource
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