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  • Friedreich's ataxia  (2)
  • 21.10.Ft Charge distribution  (1)
  • Childhood diabetes  (1)
  • Cyclosporine  (1)
  • 1
    Electronic Resource
    Electronic Resource
    Cyclosporin-induced endothelial dysfunction and hypertension: are nitric oxide system abnormality and oxidative stress involved? (2000)
    Springer
    Transplant international 13 (2000), S. S413 
    Details
    ISSN: 1432-2277
    Keywords: Key words ecNOS ; Nitric oxide ; Cyclosporine ; Hypertension ; Superoxide anions
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Hypertension is a major side effect of cyclosporin (CsA). While the mechanism(s) responsible are unclear, CsA-induced endothelial dysfunction and CsA-induced hypertension have been attributed to the CsA effect on the endothelial-derived factors controlling vasomotor tone. Endothelial nitric oxide (NO) is crucial in the maintenance of a state of basal vasodilation, and recent studies have suggested an NO-mediated counterregulatory mechanism protective from CsA-induced vasoconstriction. Our study evaluates endothelial nitric oxide synthase (ecNOS) gene status (PCR analysis) and plasma levels of NO metabolites (ELISA) in kidney and heart transplant patients under chronic CsA treatment with CsA-induced hypertension. Since CsA increases superoxide production, which metabolises NO, plasma hydroperoxides from cholesterol esters and from triglycerides and peroxynitrite were also evaluated (HPLC) as an index of the presence of superoxides and of “oxidative stress”. Quantification of monocyte ecNOS mRNA and NO metabolites plasma levels from patients and controls (C) demonstrated NO system upregulation in patients notwithstanding the hypertension. The mean ecNOS to β-actin ratio was 1.80 ± 0.85 in patients vs 0.40 ± 0.09 in C (P 〈 0.04). NO metabolites were 34.03 ± 14.32 μM in patients vs 11.53 ± 5.64 μM in C (P 〈 0.001). Hydroperoxides from cholesterol esters and from triglycerides were also increased in patients, 3.4 ± 1.4 vs 1.3 ± 0.6 integrated area units (i. a. u.), P 〈 0.007 and 10.6 ± 6.4 vs 1.3 ± 0.8 i. a. u., P 〈 0.008, respectively, as well as the peroxynitrite plasma level, 0.32 ± 0.11 μM/l vs undetectable in C. This study confirms a CsA-induced NO system upregulation in transplanted patients. However, the NO-mediated counterregulatory system to CsA-induced vasoconstriction, present in normals, could be canceled in patients by CsA-induced superoxide (O2 –) and free radical production which, by increasing NO metabolism, could contribute to CsA-induced vasoconstriction and hypertension and predispose to atherosclerosis.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s001470050374
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    Paper (German National Licenses)
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  • 2
    Electronic Resource
    Electronic Resource
    Disseminated intravascular coagulation and severe peripheral neuropathy complicating ketoacidosis in a newly diagnosed diabetic child (1994)
    Springer
    Acta diabetologica 31 (1994), S. 173-174 
    Details
    ISSN: 1432-5233
    Keywords: Disseminated intravascular coagulation ; Diabetic ketoacidosis ; Peripheral neuropathy ; Childhood diabetes
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Disseminated intravascular coagulation is a very rare complication of diabetic ketoacidosis. Central nervous system plasy but not peripheral neuropathy has been reported in these patients. On the other hand, signs of peripheral neuropathy may also be present at the onset of diabetes, but they are usually reversible within a few days after correction of the metabolic derangement. We describe an unusual case of mononeuritis multiplex syndrome still present after 2 months of follow-up in a child with diabetic ketoacidosis complicated by disseminated intravascular coagulation at the onset of insulin-dependent diabetes. These neurological impairments may be consistent with functional neural lesions due to vasa nervorum thrombosis and prolonged ischaemia.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00570376
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    Paper (German National Licenses)
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  • 3
    Electronic Resource
    Electronic Resource
    Search for spontaneous transition of nuclei to a superdense state (2005)
    Springer
    The European physical journal 23 (2005), S. 7-10 
    Details
    ISSN: 1434-601X
    Keywords: 21.10.Ft Charge distribution ; 21.10.-k Properties of nuclei; nuclear energy levels ; 21.65.+f Nuclear matter ; 29.40.Mc Scintillation detectors
    Source: Springer Online Journal Archives 1860-2000
    Topics: Physics
    Notes: Abstract. We report the results of an experimental search for spontaneous transition of nuclei from ordinary to superdense state in NaI(Tl). New limits on the superdense-state parameters are presented.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1140/epja/i2004-10072-2
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    Paper (German National Licenses)
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  • 4
    Electronic Resource
    Electronic Resource
    Friedreich's ataxia I. Clinical, Neurophysiological and in vivo biochemical studies (1979)
    Springer
    Neurological sciences 1 (1979), S. 231-238 
    Details
    ISSN: 1590-3478
    Keywords: Friedreich's ataxia ; Neuropathy ; Pyruvate test ; Lipoamide dehydrogenase ; Cardiomyopathy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: 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.
    Notes: 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.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02336703
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    Paper (German National Licenses)
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  • 5
    Electronic Resource
    Electronic Resource
    Friedreich's ataxia II. Biochemical studies in cultured cells (1979)
    Springer
    Neurological sciences 1 (1979), S. 239-243 
    Details
    ISSN: 1590-3478
    Keywords: Pyruvate-dehydrogenase ; Carnitine-acetyltransferase ; Glutamate-dehydrogenase ; Friedreich's ataxia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: 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.
    Notes: 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.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02336704
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    Paper (German National Licenses)
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