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  • Cortical development  (2)
  • Engineering General  (2)
  • Ultrastructure  (2)
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  • 1
    Digitale Medien
    Digitale Medien
    Stereological and morphometric analysis of dermal fibroblasts before and after bone marrow transplantation in a case of mucopolysaccharidosis I Scheie phenotype (1993)
    Springer
    Acta neuropathologica 86 (1993), S. 21-28 
    Details
    ISSN: 1432-0533
    Schlagwort(e): Mucopolysaccharidosis I Scheie phenotype ; Bone marrow transplantation ; Fibroblasts ; Stereologic analysis ; Ultrastructure
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Summary Bone marrow transplantation (BMT) has been used therapeutically in several types of mucopoly-saccharidoses (MPS) and other inherited metabolic disorders. Fibroblasts are severely affected in MPS due to the intralysosomal accumulation of glycosaminoglycans. We report a stereological and morphometric study at light and electron microscopy levels of dermal fibroblasts before and 21 months after BMT in a young girl with MPS I Scheie phenotype (MPS I-S). Dermal fibroblasts showed remarkable morphological changes although their density, expressed as number of fibroblasts per unit volume of dermis (number density), was not modified in the post-BMT samples as compared to pre-BMT ones. Stereological and morphometric parameters referring to cell characteristics of post-BMT fibroblasts (nuclear and cell surface densities, and both nucleus/cell and cell/nucleus volume densities) showed significant differences when compared with pre-BMT fibroblasts, and non-significant differences regarding control cells. On the other hand, quantitative parameters of the lysosomal compartment from post-BMT fibroblasts showed intermediate values between pre-BMT and control fibroblasts. These results, at cellular level, are in agreement with previous biochemical and clinical results, and clearly showed a progressive course to a non-pathological state. All parameters estimated may be considered useful tools in evaluating the success of BMT. These parameters provide quantitative data which can be statistically compared, showing the changes due to the reduction of storage material after BMT. Cell/nucleus volume density is especially interesting since not only is it easy to estimate, even by automatic procedures, but it could also constitute a numerical expression of skin anatomopathological analyses performed post-BMT.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/BF00454894
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  • 2
    Digitale Medien
    Digitale Medien
    Alzheimer's disease-type neurofibrillary degeneration in verrucose dysplasias of the cerebral cortex (1995)
    Springer
    Acta neuropathologica 90 (1995), S. 356-365 
    Details
    ISSN: 1432-0533
    Schlagwort(e): Key words Tangle ; Nodule ; Cortical development ; Ischemia
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract Verrucose dysplasias, found at autopsy in the cerebral cortex of three elderly individuals (two without neurological disorders and one with motor neuron disease), are shown to present neurofibrillary degeneration of Alzheimer's disease type. This neurofibrillary degeneration immunoreacted with antibodies against abnormally phosphorylated tau (5E2 and AT8), disclosed acetyl- and butyrylcholinesterase activity, and was consistently stained with thioflavin-S. Cortical dysplasias, found either as isolated verrucose nodules or comprising multiple nodules, contained cell-sparse areas around which a peak of neurofibrillary changes was seen. Cell-sparse areas were sometimes bridged by stripes of neurons and fibers arranged in a radial fashion, and many of these neurons showed neurofibrillary degeneration. Cytoskeletal abnormalities were conspicuous in layers II and III at the external borders of the dysplasias, as well as in neurons located in layers V and VI, and in the white matter beneath layer VI in central zones of each lesion. The morphology of cells undergoing neurofibrillary changes (from early non-fibrillar stages to late extracellular ones) suggests that neurons disturbed in their migration toward the site to which they had been committed may become vulnerable to cytoskeletal changes. Micro-environmental disturbances related to hypoxia-ischemia in the affected cortex are proposed as likely contributing factors for the long-term production of this neurofibrillary degeneration.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/BF00315009
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  • 3
    Digitale Medien
    Digitale Medien
    Bone marrow transplant in a case of mucopolysaccharidosis I Scheie phenotype: skin ultrastructure before and after transplantation (1991)
    Springer
    Acta neuropathologica 82 (1991), S. 33-38 
    Details
    ISSN: 1432-0533
    Schlagwort(e): MPS I-S ; Skin biopsy ; Ultrastructure ; Bone marrow transplant
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Summary An 11-year-old girl with mucopolysaccharidosis I Scheie phenotype (MPS I-S) received a bone marrow transplant (BMT) from her heterozygous HLA-identical LMC-non-reactive mother. Multidisciplinary studies were carried out and results evaluated 21 months after transplantation. Herein we report the ultrastructural findings pre-and post-BMT in skin. Multidisciplinary studies are commonly used to evaluate the benefits of metabolic correction following BMT in some MPS and other inherited metabolic disorders, and changes in morphology have been described in liver and few other tissues. In this case, we elected skin, since connective tissue is universally involved in MPS and is safely and easily obtainable. Comparison of skin biopsy specimens taken before and after BMT showed a considerable change in dermal fibroblast morphology, with marked reduction in cell size and the number and size of abnormal lysosomes, thus indicating the clearance of storage. Our results demonstrate that dermal cells respond to enzyme replacement therapy in MPS I-S, with the clearance of glycosaminoglycan lysosomal accumulation in connective tissue fibroblasts, which had near-normal morphology 21 months after BMT. Therefore, the practice of skin biopsy after BMT in MPS and other metabolic disorders in which dermal cells are involved should be encouraged.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/BF00310920
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  • 4
    Digitale Medien
    Digitale Medien
    Alzheimer's disease-type neurofibrillary degeneration in verrucose dysplasias of the cerebral cortex (1995)
    Springer
    Acta neuropathologica 90 (1995), S. 356-365 
    Details
    ISSN: 1432-0533
    Schlagwort(e): Tangle ; Nodule ; Cortical development ; Ischemia
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract Verrucose dysplasias, found at autopsy in the cerebral cortex of three elderly individuals (two without neurological disorders and one with motor neuron disease), are shown to present neurofibrillary degeneration of Alzheimer's disease type. This neurofibrillary degeneration immunoreacted with antibodies against abnormally phosphorylated tau (5E2 and AT8), disclosed acetyland butyrylcholinesterase activity, and was consistently stained with thioflavin-S. Cortical dysplasias, found either as isolated verrucose nodules or comprising multiple nodules, contained cell-sparse areas around which a peak of neurofibrillary changes was seen. Cell-sparse areas were sometimes bridged by stripes of neurons and fibers arranged in a radial fashion, and many of these neurons showed neurofibrillary degeneration. Cytoskeletal abnormalities were conspicuous in layers II and III at the external borders of the dysplasias, as well as in neurons located in layers V and VI, and in the white matter beneath layer VI in central zones of each lesion. The morphology of cells undergoing neurofibrillary changes (from early nonfibrillar stages to late extracellular ones) suggests that neurons disturbed in their migration toward the site to which they had been committed may become vulnerable to cytoskeletal changes. Micro-environmental disturbances related to hypoxia-ischemia in the affected cortex are proposed as likely contributing factors for the longterm production of this neurofibrillary degeneration.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/BF00315009
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 5
    Digitale Medien
    Digitale Medien
    Seismic earth thrust against massive building walls (1989)
    New York, NY [u.a.] : Wiley-Blackwell
    International Journal for Numerical and Analytical Methods in Geomechanics 13 (1989), S. 347-357 
    Details
    ISSN: 0363-9061
    Schlagwort(e): Engineering ; Engineering General
    Quelle: Wiley InterScience Backfile Collection 1832-2000
    Thema: Architektur, Bauingenieurwesen, Vermessung , Geologie und Paläontologie
    Notizen: The problem of earth thrust for a massive building in a seismic environment is analysed. Intertial and kinematic soil-structure interactions are considered by modelling the soil and the structure together. The problem is solved in the frequency domain by using the computer code FLUSH. Results show that the horizontal component of the seismic earth thrust is much greater than that obtained when applying the Mononobe-Okabe theory. This study establishes a set of conclusicns and recommendations for both design and practical purposes, and proposes future lines of investigation.
    Zusätzliches Material: 10 Ill.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1002/nag.1610130402
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  • 6
    Digitale Medien
    Digitale Medien
    Effect of adjoining structures on seismic response of tunnels (1992)
    New York, NY [u.a.] : Wiley-Blackwell
    International Journal for Numerical and Analytical Methods in Geomechanics 16 (1992), S. 797-814 
    Details
    ISSN: 0363-9061
    Schlagwort(e): Engineering ; Engineering General
    Quelle: Wiley InterScience Backfile Collection 1832-2000
    Thema: Architektur, Bauingenieurwesen, Vermessung , Geologie und Paläontologie
    Notizen: In this paper the seismic behaviour of buried concrete tunnels is studied using the finite element method. The numerical tool employed is the well-known soil-structure interaction computer program FLUSH and thus analyses are carried out in the frequency domain. The seismic environment is supposed to consist of vertically propagating shear and compression waves that reproduce artificial accelerograms of a broad frequency content at selected control points. The effect of near or adjacent massive structures, such as those of nuclear power plants, is evaluated showing their influence on tunnel response. The results calculated for acceleration levels, internal structural loads, response spectra, etc., are analysed and compared either with those obtained from soil free-field response or with others computed using an alternative simplified methodology, providing useful information for preliminary design purposes.
    Zusätzliches Material: 12 Ill.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1002/nag.1610161103
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