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  • 1
    ISSN: 1432-0533
    Keywords: Germanium intoxication ; Vacuolar myopathy ; Mitochondrial abnormality
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Pathological examinations were carried out on the skeletal muscle of a patient with germanium intoxication. The prominent histochemical finding was vacuolar myopathy with lipid excess, increased acid phosphatase activity and decreased cytochrome c oxidase activity. Ultrastructural lesions revealed a mitochondrial abnormality, autophagic vacuoles and accumulation of high electron-dense materials in deformed mitochondria and at the periphery of lipid droplets. Furthermore, the toxic effect of germanium on skeletal muscle was confirmed by the experimentally induced germanium myopathy, which showed autophagic degeneration, decreased cytochrome c oxidase activity and a mitochondrial abnormality with high electron-dense materials.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-0533
    Keywords: Abetalipoproteinemia ; Muscle pathology ; Giant lysosome ; Concentric laminated body
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Pathological examination was carried out of the skeletal muscle of an 8-year-old boy with abetalipoproteinemia. The patient complained of diarrhea, and showed a deficiency of betalipoprotein, decreased fatsoluble vitamins, acanthocytosis and a mild incrase in serum creatine kinase. The prominent histochemical finding was punctate deposits of acid phosphatase activity in most fibers. Ultrastructural lesions revealed a number of giant lysosomes. Although these pathological findings seemed to be related to vitamin E deficiency, other pathological findings such as concentric laminated bodies of filamentous bodies were also observed. The clinical course and the changes in the pathological findings in our patient after long-term vitamin E therapy need to be observed.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 149 (1990), S. 659-660 
    ISSN: 1432-1076
    Keywords: Acute infantile hemiplegia ; Propionic acidaemia ; Carnitine ; Methylcitric acid
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A 10-month-old girl with mild developmental delay became hemiplegic after seizures. Cranial CT scan and magnetic resonance imaging (MRI) revealed no lesions related to vascular diseases, but brain atrophy on the right side was remarkable. Digital subtraction angiography showed slightly decreased visualization of peripheral branches of the right medial cerebral artery. Propionic acidaemia was diagnosed on the basis of high plasma levels of propionic acid and its metabolites and the elevated urinary excretion of these acids. With therapy, the levels of these acids fell, and her left hemiplegia disappeared 3 months later.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    [S.l.] : International Union of Crystallography (IUCr)
    Acta crystallographica 25 (1969), S. 682-693 
    ISSN: 1600-5724
    Source: Crystallography Journals Online : IUCR Backfile Archive 1948-2001
    Topics: Chemistry and Pharmacology , Geosciences , Physics
    Notes: The X-ray scattering amplitude of a crystal containing isolated imperfections is expressed in terms of the Fourier transform of the atomic displacement vectors. The amplitude contains only the local properties of the imperfections which are `seen' by the incident beam of a finite size. Disruption of the Borrmann transmitted (or diffracted) beam, narrowing of the dynamical diffraction range and `diffuse' scattering caused by dynamically diffracted X-rays are some of the results obtained from the calculated amplitude. Black, white and black-white images in topographs are explained by the present theory. Image contrast is also discussed in terms of the thickness of the crystal.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    [S.l.] : International Union of Crystallography (IUCr)
    Acta crystallographica 26 (1970), S. 667-673 
    ISSN: 1600-5724
    Source: Crystallography Journals Online : IUCR Backfile Archive 1948-2001
    Topics: Chemistry and Pharmacology , Geosciences , Physics
    Notes: Primary and secondary extinction are studied using the dynamical theory of X-rays diffracted by imperfect crystals. The transition from dynamical to kinematical scattering is explained in terms of fundamental processes in diffraction. Contrary to existing extinction theories, where the intensities diffracted dynamically by single coherent domains of a mosaic are combined using an ad hoc assumption of mosaic distributions, the present theory permits the dynamical amplitudes to change in response to disturbances of the dynamical interactions by imperfections. Neither the mosaic block model nor the statistical treatment of imperfections is used. The extinction of diffracted intensities is thereby treated as caused solely by inhomogeneous strains in a single coherent domain.
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    [S.l.] : International Union of Crystallography (IUCr)
    Acta crystallographica 27 (1971), S. 273-280 
    ISSN: 1600-5724
    Source: Crystallography Journals Online : IUCR Backfile Archive 1948-2001
    Topics: Chemistry and Pharmacology , Geosciences , Physics
    Notes: The scattering amplitude of an X-ray interferometer is calculated using a general theory of X-ray diffraction. Analytic expressions are given for the intensities of diffracted beams in interferometry; these expressions include the effect of the positions of each crystal lattice in addition to ordinary dynamical effects. It is concluded that the fringes observed in X-ray interferometry of a lattice spacing are accounted for by an optical (Moiré) effect rather than by dynamical effects of X-ray diffraction.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    [S.l.] : International Union of Crystallography (IUCr)
    Acta crystallographica 27 (1971), S. 634-647 
    ISSN: 1600-5724
    Source: Crystallography Journals Online : IUCR Backfile Archive 1948-2001
    Topics: Chemistry and Pharmacology , Geosciences , Physics
    Notes: X-ray Compton-Raman scattering is reviewed from an original point of view. The differential cross section for X-ray inelastic scattering from crystals is derived from first principles to explain the coexisting Compton and Raman scattering in solids. This derivation makes it possible to express the cross section in terms of the current correlation of electrons in a crystal. All the electrons, including the core electrons, are treated on an equal basis in this formulation. The relationship between the X-ray absorption spectrum and the inelastic scattering spectrum is discussed in detail. This formulation provides a theoretical justification to the importance of X-ray inelastic scattering experiments in connection with an experimental determination of the two-particle Green's function. In the Appendices the theoretical formulations are given in detail, which apply not only to the ordinary inelastic scattering process, but also to the processes involving Bragg diffraction.
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    [S.l.] : International Union of Crystallography (IUCr)
    Acta crystallographica 30 (1974), S. 525-535 
    ISSN: 1600-5724
    Source: Crystallography Journals Online : IUCR Backfile Archive 1948-2001
    Topics: Chemistry and Pharmacology , Geosciences , Physics
    Notes: Based on a general dynamical theory of diffraction, an integral equation for dynamical diffraction in imperfect crystals is obtained in the spatial coordinate representation. This equation is derived for diffraction topography in the symmetrical Laue geometry from the basic dynamical equation of diffraction previously derived in the momentum representation. Discussion is presented on an interpretation of this integral equation beyond the given diffraction geometry. This equation can be considered as a basic equation for diffraction topography.
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    [S.l.] : International Union of Crystallography (IUCr)
    Acta crystallographica 42 (1986), S. 156-164 
    ISSN: 1600-5724
    Source: Crystallography Journals Online : IUCR Backfile Archive 1948-2001
    Topics: Chemistry and Pharmacology , Geosciences , Physics
    Notes: Crystallographic analysis is applied to a set of electron diffraction patterns taken from a rapidly cooled Al-Mn alloy to construct reciprocal-lattice patterns in agreement with the observed icosahedral results. The analysis leads to a proposed atomic scale model which is derived from two sets of experimental modulations, each of which has six independent modulation vectors. The underlying structure has a lattice, the unit cell of which involves 32 atomic sites with the required symmetry properties. The appearance of the experimental electron diffraction patterns is explained either by the coherent arrangement of this lattice with an irrational sublattice or by an independent set of modulations. The relationship of this structure to three-dimensional nonperiodic Penrose tilings is explored.
    Type of Medium: Electronic Resource
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  • 10
    Electronic Resource
    Electronic Resource
    [S.l.] : International Union of Crystallography (IUCr)
    Acta crystallographica 31 (1975), S. 774-779 
    ISSN: 1600-5724
    Source: Crystallography Journals Online : IUCR Backfile Archive 1948-2001
    Topics: Chemistry and Pharmacology , Geosciences , Physics
    Notes: The basic equation of dynamical diffraction for imperfect crystals, which has been derived previously by a general dynamical theory of diffraction, is rederived classically based on the Ewald–Laue–Bethe concept, thus completing the formulation of a 'scattering matrix' theory. It is shown in this classical derivation that a series of assumptions is required at each stage of the mathematical formulation to allow it to proceed further. These assumptions are then viewed in terms of the general dynamical theory of diffraction, and found unnecessary in the rigouous formulation. This classical formulation provides a conceptual relation between the traditional Ewald–Laue–Bethe dynamical theory and the general dynamical theory, one that has been derived with the aid of quantum-field theory.
    Type of Medium: Electronic Resource
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