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  • Digitale Medien  (5)
  • 1980-1984  (2)
  • 1975-1979  (3)
  • 1
    Digitale Medien
    Digitale Medien
    Structure elucidation with lanthanide-induced shifts. The use of bound shifts and high-symmetry substrates (1976)
    s.l. : American Chemical Society
    Journal of the American Chemical Society 98 (1976), S. 6042-6044 
    Details
    ISSN: 1520-5126
    Quelle: ACS Legacy Archives
    Thema: Chemie und Pharmazie
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1021/ja00435a047
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 2
    Digitale Medien
    Digitale Medien
    Abnormal auditory evoked potentials in Déjérine-Sottas disease (1984)
    Springer
    Journal of neurology 231 (1984), S. 46-49 
    Details
    ISSN: 1432-1459
    Schlagwort(e): Hereditary motor sensory neuropathy ; Déjérine-Sottas disease ; Auditory evoked potentials
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Beschreibung / Inhaltsverzeichnis: Zusammenfassung Es wird über zwei Fälle von hereditärer motorischer und sensorischer Neuropathie vom Typ-III (Déjérine-Sottas-Krankheit) berichtet, welche otoneurologisch und elektrophysiologische eingehend untersucht wurden. In beiden Fällen fand man Hinweise dafür, daß die vestibulären und akkustischen zentralen Bahnen mitbetroffen sind. Dies läßt darauf schließen, daß bei dieser Erkrankung die Myelinstrukturen nicht nur in den peripheren Nerven sondern auch im Zentralorgan betroffen sein können.
    Notizen: Summary Two cases of hereditary motor sensory neuropathy type III (Déjérine-Sottas disease) examined by audiological, vestibular and electrophysiological methods are reported. In both cases there were signs of vestibular and acoustic central pathway involvement, shown by vestibular examination and by the study of auditory evoked potentials. The presence of central involvement in this hereditary neuropathy suggests central as well as peripheral myelin alteration.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/BF00313652
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  • 3
    Digitale Medien
    Digitale Medien
    Friedreich's ataxia I. Clinical, Neurophysiological and in vivo biochemical studies (1979)
    Springer
    Neurological sciences 1 (1979), S. 231-238 
    Details
    ISSN: 1590-3478
    Schlagwort(e): Friedreich's ataxia ; Neuropathy ; Pyruvate test ; Lipoamide dehydrogenase ; Cardiomyopathy
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Beschreibung / Inhaltsverzeichnis: Sommario Si presenta uno studio clinico, neurofisiologico e biochimico di 18 pazienti con diagnosi presuntiva di atassia di Friedreich. I dati portano ad inquadrare nella malattia di Friedreich tipica solo 14 pazienti, gli altri 4 si differenziano per diversi aspetti clinici di cui il più rilevante è la normalità cardiaca. Si conclude quindi che per giungere alla diagnosi di malattia di Friedreich che permetta in seguito uno studio biochimico della malattia, sia necessario un approccio multidisciplinare.
    Notizen: Abstract Eighteen patients with the presumptive diagnosis of Friedreich's ataxia were studied. Clinical, neurophysiological and biochemical data were concordant in 14 patients and led to the diagnosis of typical Friedreich's ataxia in this group of patients: the remaining 4 patients differed from the typical patients in several respects but mainly in the cardiological findings. It is concluded that so far no single clinical or laboratory finding is typical of F.A.. Multidisciplinary approaches are essential to the diagnosis of Friedreich's ataxia.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/BF02336703
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  • 4
    Digitale Medien
    Digitale Medien
    Friedreich's ataxia II. Biochemical studies in cultured cells (1979)
    Springer
    Neurological sciences 1 (1979), S. 239-243 
    Details
    ISSN: 1590-3478
    Schlagwort(e): Pyruvate-dehydrogenase ; Carnitine-acetyltransferase ; Glutamate-dehydrogenase ; Friedreich's ataxia
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Beschreibung / Inhaltsverzeichnis: Sommario L'ossidazione del piruvato e del palmitato misurata nei fibroblasti in cultura di pazienti affetti da malattia di Friedreich è risultata normale. Il dosaggio del complesso enzimatico piruvico deidrogenasi è invece risultato significativamente diminuito rispetto ai controlli nelle cellule di questi pazienti quando i fibroblasti venivano omogenizzati in glicerolo. Questa diminuzione però non era più evidente se le cellule venivano sonicate. Questi dati suggeriscono che il deficit del complesso PDH nei pazienti con atassia di Freidreich non sia un difetto primario ma rifletta piuttosto anomalie di membrana.
    Notizen: Abstract Pyruvate and palmitate oxidations by cultured fibroblast suspensions were measured in optimized conditions and proved to be with normal range in the cells from Friedreich's patients. But when pyruvate oxidation was measured by direct assay of the pyruvate dehydrogenase complex, this enzyme activity proved to be significantly lower in Friedreich's than in controls' cells. These abnormalities were not observed when the cells were sonicated. Moreover, lipoamide dehydrogenase activity Km and Vmax were within the normal range in Friedreich's cells. These data suggest that the low activities of the PDH complex are not a primary defect in Friedreich's ataxia but are more likely to be related to membrane abnormalities in Friedreich's cells.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/BF02336704
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  • 5
    Digitale Medien
    Digitale Medien
    Influenza B-associated Reye syndrome: report of a case with isolation of a coxsackie A5 virus from CSF (1983)
    Springer
    Neurological sciences 4 (1983), S. 329-334 
    Details
    ISSN: 1590-3478
    Schlagwort(e): Reye syndrome ; influenza B virus ; Coxsackievirus A5 ; mixed infection
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Beschreibung / Inhaltsverzeichnis: Sommario Abbiamo studiato da un punto di vista clinico e virologico un caso di sindrome di Reye verificatosi in una bambina di 16 mesi pochi giorni dopo un episodio di influenza. La diagnosi di sindrome di Reye è stata posta sulla base di dati clinici e di laboratorio. Le indagini virologiche sono state condotte su campioni di feci, tampone faringeo, liquor e su una coppia di sieri. Dal liquor è stato isolato un virus coxsackie A5. Nella coppia di sieri è stata stata evidenziata una sieroconversione verso la variante del virus influenzale di tipo B in circolazione nella regione a quell'epoca e verso il ceppo di virus coxsackie A5 isolato. L'isolamento dal SNC del virus coxsackie A5 e la dimostrazione di una concomitante infezione da virus influenzale B nel caso da noi studiato depongono a favore dell'ipotesi che una infezione virale mista possa scatenare la sindrome di Reye.
    Notizen: Abstract A 16 month old girl developed Reye syndrome a few days after an episode of influenza. The diagnosis of RS was made on clinical and laboratory data. Virological examinations were done on specimens of stools, throat swab, spinal fluid and two samples of serum. A coxsackievirus A5 was isolated from the spinal fluid. A seroconversion was found against the variant of influenza type B virus circulating in our region at that time and the isolated coxsackie A5 strain. The involvement in the CNS of coxsackie A5 and the demonstration of a simultaneous infection with influenza B virus supports the hypothesis that a mixed viral infection could trigger the Reye syndrome.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/BF02043487
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