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  • 1
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    International journal of dermatology 26 (1987), S. 0 
    ISSN: 1365-4632
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: : We compared morphologic features of Keratohyalin granules (KHG) that were directly related to keratinization in oral mucosa (tongue, cheek, gums, palate; n = 4) with those in parakeratotic epidermis (psoriasis, n = 2; pityriasis rubra pilaris, n = 1; acute dermatitis, n = 1) and normal or-thokeratotic epidermis. Among others, the ultrastructural features of globular KHG were observed in the cheek, nonspecialized tongue mucosa, and parakeratotic epidermis occurring in psoriasis, pityriasis rubra pilaris, and acute dermatitis, whereas gums and palate showed a mixture of characteristics, also resembling stellate KHG as seen in normal skin.From literature as well as from our studies, the impression was gained that globular KHG were found especially in quickly dividing epithelia and could easily be distinguished from the irregular or stellate KHG that were found in slowly dividing normal epidermis.Therefore, we studied keratinization features on days 3, 7, and 14 after autografting normal human skin (n = 4), thus inducing high cell turnover. Stellate KHG, present in granular cells of normal skin, were almost absent on the third day. Active cell division on the seventh day resulted in sparse keratohyalin formation inside globular granules of low electron density, whereas numerous, rather electron-translucent lipid droplets occurred in upper spinous and horny cells. These two phenomena seemed to be interrelated. After 14 days, round and increasingly electron-dense KHG were noted. In more differentiated areas, KHG became irregular. The morphogenesis of KHG could be studied in the subsequent phases of differentiation in autologous skin grafts.We conclude that the ultrastructural features of KHG formation are related to the speed of cell turnover in the epidermis as well as in the oral mucosa.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Publishing Ltd
    Journal of cutaneous pathology 13 (1986), S. 0 
    ISSN: 1600-0560
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: In quickly dividing epithelia such as that of the tongue, keratohyalin formation takes place in globular keratohyalin granules (KHG). This is in contrast with the irregular KHG as seen in normal, slowly dividing epidermis. The morphogenesis of the globular KHG is explained in this study. In small KHG, dense aggregates of ribosomes can be seen at the site of blebs. It is suggested that these blebs framed with ribosomes are internalized giving rise to “dense homogeneous deposits” or “single granules”. Lipid droplets occur in the upper spinous and horny layer. Globular KHG also contain variable amounts of lipids, and the lipid content seems to be inversely related to the protein content, dependent on the degree of cell differentiation or on the rate of cell turnover. It is suggested that in epithelia with a high cell turnover few rigid keratohyalin components are dispersed in lipids, which maintain a globular shape due to the surface tension.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Virchows Archiv 382 (1979), S. 139-150 
    ISSN: 1432-2307
    Keywords: Intimal fibrosis of pulmonary arteries ; Ultrastructure of intima ; Intimal smooth muscle cells
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Intimal fibrosis of muscular pulmonary arteries may present in various forms and in varying degrees of severity according to the underlying condition. In patients with pulmonary hypertension, the type of intimal fibrosis is often significant with regard to prognosis and reversibility. For these reasons we have studied the ultrastructure of the thickened intimal layer in aged individuals, where intimal fibrosis occurs as a normal age change, and in patients with pulmonary hypertension associated with fibrosis of the lungs, mitral stenosis, chronic pulmonary thromboembolism and plexogenic pulmonary arteriopathy (either primary or secondary to congenital cardiac defects). In all these forms of intimal fibrosis, the cellular component of the subendothelial intimal layer was apparently almost exclusively the smooth muscle cell. These cells usually had a haphazard arrangement. In primary and secondary plexogenic pulmonary arteriopathy, however, there was a more regular circumferential arrangement. The ultrastructural evidence suggested that the intimal cells were derived from medial smooth muscle cells.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Journal of neurology 216 (1977), S. 163-172 
    ISSN: 1432-1459
    Keywords: Cross striations loss of ; Myopathy congenital
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Es werden zwei Patienten beschrieben, Bruder und Schwester, beide an einer kongenitalen, generalisierten, progressiven Muskelschwäche leidend. Die Muskelbiopsie zeigte Zonen mit Verlust der Querstreifung in den Muskelfasern, und bei elektronenmikroskopischer Untersuchung zeigte sich eine totale Unordnung der Myofibrillen mit Strömung der Z-Scheiben und Fehlen von Mitochondrien. In diesen Bezirken fanden sich blasige Kerne mit prominenten Nukleolen. In beiden Fällen zeigte sich ein starkes Überwiegen der Typ-I-Fasern. Der mittlere Durchmesser der Typ-I-Muskelfasern war in einem der Fälle zu gering. Veränderungen in der motorischen Endplatte in diesem Fall zeigten keine Denervierung, sondern deuteten auf eine verzögerte Entwicklung der motorischen Nerven.
    Notes: Summary Two patients, a brother and sister, both suffering from congenital generalized muscle weakness with a progressive course are reported. Muscle biopsy revealed areas with loss of cross striations in the muscle fibers, electronmicroscopically presenting complete disorganization of the myofibrils with streaming of the Z discs and absence of mitochondria. Vesicular nuclei with prominent nucleoli were present in these areas. There was a type I fiber prodominance in both cases. The mean diameter of the type I muscle fibers in one of the cases was too small. Motor endplate alterations in this patient gave no evidence of denervation but were suggestive of a delayed development of motor nerves.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 80 (1990), S. 103-107 
    ISSN: 1432-0533
    Keywords: Brain neoplasms ; Medulloepithelioma ; Primitive neuro-ectodermal tumor ; Immunohistochemistry ; Electron microscopy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A case is reported of a boy, 3 years of age, with a large medulloepithelioma in the left cerebral hemisphere. Medulloepitheliomas are rare tumors of the primitive medullar epithelium. Histological, immunohistochemical and electron microscopical findings are presented. We discuss previously reported cases, the ontogeny of this type of tumor and the relation to the socalled primitive neuro-ectodermal tumors (PNET).
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 88 (1994), S. 485-489 
    ISSN: 1432-0533
    Keywords: Key words Glioma ; Astrocytoma ; Lipoma ; Lipidization ; Cerebellum
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report the case of an elderly woman with a history of headache, vomiting and dizziness while walking. On CT scans a mass was identified in the right cerebellar hemisphere exhibiting radiological characteristics of lipomatous tissue. Surgery revealed a compact lesion consisting of whitish-yellow tissue with a fatty aspect and texture. Smears of tissue samples and paraffin sections showed features suggestive of tissue mainly composed of fully differentiated lipocytes. Lipid-specific stainings on fresh frozen material confirmed univacuolar intracytoplasmic fat accumulation. However, immunohistochemistry for glial fibrillary acidic protein and electron microscopy clearly demonstrated the glial lineage of these lipid-laden cells. Therefore, the tumor was diagnosed as a highly lipidized astrocytoma. In our view, this case represents a variant of lipidized gliomas that has not been described previously and that differs phenotypically from the entities documented earlier.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 88 (1994), S. 485-489 
    ISSN: 1432-0533
    Keywords: Glioma ; Astrocytoma ; Lipoma ; Lipidization ; Cerebellum
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report the case of an elderly woman with a history of headache, vomiting and dizziness while walking. On CT scans a mass was identified in the right cerebellar hemisphere exhibiting radiological characteristics of lipomatous tissue. Surgery revealed a compact lesion consisting of whitish-yellow tissue with a fatty aspect and texture. Smears of tissue samples and paraffin sections showed features suggestive of tissue mainly composed of fully differentiated lipocytes. Lipid-specific stainings on fresh frozen material confirmed univacuolar intracytoplasmic fat accumulation. However, immunohistochemistry for glial fibrillary acidic protein and electron microscopy clearly demonstrated the glial lineage of these lipid-laden cells. Therefore, the tumor was diagnosed as a highly lipidized astrocytoma. In our view, this case represents a variant of lipidized gliomas that has not been described previously and that differs phenotypically from the entities documented earlier.
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 81 (1990), S. 228-232 
    ISSN: 1432-0533
    Keywords: Cerebral tumor ; Polar spongioblastoma ; Astrocytoma ; Immunohistochemistry ; Electron microscopy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A case is reported of a 9-year-old boy with a cerebral polar spongioblastoma. This neoplasm, first described by Russell and Cairns in 1947, is morphologically a distinct entity characterized by bipolar tumor cells with palisading nuclei. In the case under study immunoreactivity for neuron-specific enolase was found and ultrastructural features of developing neuronal elements were present. A neuro-endocrine nature was suggested by de Chadarévian et al. (1984) in a morphologically similar case. These findings are in contrast with the longheld view that the polar spongioblastoma is cytogenetically related to the embryonal radial glial cells.
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 144 (1985), S. 338-342 
    ISSN: 1432-1076
    Keywords: Zellweger syndrome ; Autosomal recessive inheritance ; Cerebro-hepato-renal syndrome ; Peroxisomal disease ; Mental retardation ; Epilepsy ; Hepatic fibrosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A 4.5-year-old male patient is described with chorioretinopathy, minor facial anomalies, delayed closure of the fontanel, mental retardation, moderate hypotonia, epilepsy and hepatic fibrosis. Postural control, intentional vocalising and manual dexterity were superior to the performance of patients with classical Zellweger syndrome (ZS). Morphologically distinct peroxisomes were absent in the liver. In blood elevated pipecolic acid levels and abnormal levels of bile acid intermediates were found. The plasmalogen content of erythrocytes was normal. In fibroblasts we found an accumulation of very long chain fatty acids, decreased activity of acyl CoA: dihydroxyacetone phosphate acyltransferase, and impaired de novo biosynthesis of plasmalogens. On the basis of these clinical, ultrastructural and biochemical characteristics we assume that this patient represents a milder variant of the classical cerebro-hepato-renal syndrome of Zellweger.
    Type of Medium: Electronic Resource
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  • 10
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 150 (1991), S. 656-660 
    ISSN: 1432-1076
    Keywords: Arthrogryposis ; Congenital ; X-linked ; Degenerative myopathy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Five males are reported with severe X-linked arthrogryposis. Main findings are marked respiratory insufficiency and feeding problems, multiple contractures, deformities of chest and vertebral column, and typical facies. Most of these findings can be explained by a pronounced prenatal and postnatal muscle weakness. The sole living child has severe psychomotor retardation. Several female carriers show mild features (clubfeet, contractures, hyperkyphosis, and slight muscle weakness). One manifesting carrier is affected more severely (multiple contractures, mental retardation, and various dysmorphic features). Additional investigations including muscle biopsy revealed none of the usual signs of denervation, and pointed to the presence of a degenerative muscle disorder.
    Type of Medium: Electronic Resource
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