Library

X
feed icon rss

Your email was sent successfully. Check your inbox.

An error occurred while sending the email. Please try again.

Proceed reservation?

Export
  • 1
    Electronic Resource
    Electronic Resource
    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
    Keywords: Mucopolysaccharidosis I Scheie phenotype ; Bone marrow transplantation ; Fibroblasts ; Stereologic analysis ; Ultrastructure
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: 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.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00454894
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
    Paper (German National Licenses)
    Fulltext
  • 2
    Electronic Resource
    Electronic Resource
    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
    Keywords: Tangle ; Nodule ; Cortical development ; Ischemia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: 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.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00315009
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
    Paper (German National Licenses)
    Fulltext
  • 3
    Electronic Resource
    Electronic Resource
    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
    Keywords: Key words Tangle ; Nodule ; Cortical development ; Ischemia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: 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.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00315009
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
    Paper (German National Licenses)
    Fulltext
Close ⊗
This website uses cookies and the analysis tool Matomo. More information can be found here...