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  • 1
    ISSN: 1432-1076
    Keywords: Key words     X-chromosomal adrenoleukodystrophy ; Adrenomyeloneuropathy ; Very long-chain fatty acids ; Therapy ; Peroxisomal disorder
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract      We have investigated the effect of glyceroltrioleate/glyceroltrierucate (GTO/GTE) therapy on X-chromosomal adrenoleukodystrophy in 16 patients with adrenoleukodystrophy (n = 6), adrenomyeloneuropathy (n = 3), Addison disease without neurological involvement (n = 2), and neurologically and endocrinologically asymptomatic patients (n = 5). Therapy was carried out for 19.4 ± 10 months. All patients showed a normalization of C 26:0 plasma fatty acid concentrations. None of the seven neurologically asymptomatic patients developed neurological symptoms. Somatosensory evoked potentials of the tibialis nerve was the most sensitive electrophysiological parameter, showing a slight improvement in neurologically asymptomatic patients during therapy. In none of the patients with normal cranial MRI at start of therapy (n = 6) has MRI deterioration been observed whilst on therapy. Follow up of the neurologically asymptomatic children supports the hypothesis that GTO/GTE therapy might prevent the development of neurological symptoms. Six of the nine neurologically symptomatic patients deteriorated to varying degrees whilst on therapy. MRI alterations have worsened in all patients with clinical deterioration. Conclusion     GTO/GTE treatment should be initiated in all neurological asymptomatic boys before first neurological symptoms develop. To discover these patients very long-chain fatty acid determination should be performed in all family members at risk when adrenoleukodystrophy or adrenomyeloneuropathy is diagnosed.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-1076
    Keywords: X-chromosomal adrenoleukodystrophy ; Adrenomyeloneuropathy ; Very long-chain fatty acids Therapy ; Peroxisomal disorder
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We have investigated the effect of glyceroltrioleate/glyceroltrierucate (GTO/GTE) therapy on X-chromosomal adrenoleukodystrophy in 16 patients with adrenoleukodystrophy (n=6), adrenomyeloneuropathy (n=3), Addison disease without neurological involvement (n=2), and neurologically and endocrinologically asymptomatic patients (n=5). Therapy was carried out for 19.4±10 months. All patients showed a normalization of C 26:0 plasma fatty acid concentrations. None of the seven neurologically asymptomatic patients developed neurological symptoms. Somatosensory evoked potentials of the tibialis nerve was the most sensitive electrophysiological parameter, showing a slight improvement in neurologically asymptomatic patients during therapy. In none of the patients with normal cranial MRI at start of therapy (n=6) has MRI deterioration been observed whilst on therapy. Follow up of the neurologically asymptomatic children supports the hypothesis that GTO/GTE therapy might prevent the development of neurological symptoms. Six of the nine neurologically symptomatic patients deteriorated to varying degrees whilst on therapy. MRI alterations have worsened in all patients with clinical deterioration. Conclusion GTO/GTE treatment should be initiated in all neurological asymptomatic boys before first neurological symptoms develop. To discover these patients very long-chain fatty acid determination should be performed in all family members at risk when adrenoleukodystrophy or adrenomyeloneuropathy is diagnosed.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    European journal of clinical pharmacology 11 (1977), S. 33-36 
    ISSN: 1432-1041
    Keywords: Sulphadimidine ; acetylator phenotype ; chronic renal failure ; serum estimation ; urine estimation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Chemistry and Pharmacology , Medicine
    Notes: Summary After oral administration of sulphadimidine (mean dose 3.33 g) to 21 volunteers it was possible to distinguish fast and slow acetylators by calculating the acetylated fraction (%acSDD) in a single serum sample obtained at any time between 1/2–24 h. There was a close correlation between %acSDD in serum and in urine collected from 0–8 h. Two groups of patients with chronic renal failure were studied. Four of the first 8 patients studied would have been designated as slow acetylators from their low %acSDD in 0–8 h urine, but as fast acetylators from their %acSDD in serum 6 h after drug administration. The next 18 patients were given a smaller dose of SDD (2 g) and they showed complete intra-individual correlation between %acSDD in 0–24 h urine and in a serum sample obtained at 24 h. The patients could be divided into 2 sub-groups on the basis of %acSDD in serum and urine, thus demonstrating the ability of this procedure to distinguish fast and slow acetylators, even in advanced chronic renal failure.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    European journal of clinical pharmacology 19 (1981), S. 187-192 
    ISSN: 1432-1041
    Keywords: disopyramide ; cardiac failure ; pharmacokinetics
    Source: Springer Online Journal Archives 1860-2000
    Topics: Chemistry and Pharmacology , Medicine
    Notes: Summary The parmacokinetics of disopyramide (DP) in 10 patients with imminent to moderate cardiac failure has been studied and compared with the results in normal volunteers. The biological half life of rapid distribution (T1/2 α) and of elimination (T1/2 β) were increased (11.1±4.4 min and 9.7±4.2 h, respectively). Total body clearance (Clt) was decreased (0.467±0.215 ml · min−1 · kg−1), and the volume of distribution (Vd) was slightly reduced (0.610±0.1361 · kg−1), probably due to the lower cardiac index. After oral administration, the time to peak serum concentration was increased (139±89 min), and the mean peak serum concentration (2.4±0.8% dose · 1−1) was also higher than reported in normal subjects. Comparison of the areas under the concentration versus time curves after intravenous and oral administration (AUC i. v. and AUC oral) showed that DP was almost completely absorbed, its bioavailability being 97.5±15.0%.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1432-1041
    Keywords: Procainamide ; sustained release tablets ; ventricular arrhythmias ; maintenance therapy ; plasma levels
    Source: Springer Online Journal Archives 1860-2000
    Topics: Chemistry and Pharmacology , Medicine
    Notes: Summary A new sustained-release tablet formulation of procainamide has been tested in 11 patients with chronic ventricular arrhythmias. At 6 h dosing intervals the fluctuations in plasma procainamide concentrations were equal to or less than those found after treatment at the 3 hourly intervals necessary with conventional tablets of Pronestyl®. It was concluded that the sustained release preparation ought to be more convenient for maintenance therapy with procainamide, as it permitted treatment at intervals of 6 h.
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1573-7241
    Keywords: hospital mortality ; coronary mortality ; calcium antagonists ; myocardial infarction
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We investigated whether prior use of calcium antagonists in 80 (16.8%) out of 477 patients (64% males) admitted with acute myocardial infarction (MI) had any impact on in-hospital mortality. Patients using calcium antagonists were slightly older (74 years vs. 72 years, 2P = 0.039) than those not taking them and fewer were male patients. Previous MI, diabetes mellitus, and prior use of aspirin, beta-blockers, and long-acting nitrates were more frequent in patients on calcium antagonists. In contrast, fewer patients on calcium antagonists prior to symptoms received thrombolytic treatment (21.3% vs. 34.8%, 2P = 0.018). The study had an observational exposed/nonexposed design, and we looked for both crude and adjusted effects. Of the 83 patients (17.4%) who died during hospitalization, 18 patients were in the calcium antagonist group (22.5%). The odds ratio (OR) for these patients to die in the hospital was 1.48 and the 95% confidence interval (CI) 0.78–2.78; 2P = 0.19. When adjusting for confounders (gender, age, smoking habit, previous MI, and diabetes mellitus, as well as prior use of aspirin, beta-blockers, long-acting nitrates, and thrombolytic treatment at entry) OR was 1.08 and 95% CI 0.57–2.05; 2P = 0.85. Thus, we found no excess in-hospital mortality in patients with acute MI using calcium antagonists prior to the onset of symptoms.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    Chromatographia 31 (1991), S. 535-538 
    ISSN: 1612-1112
    Keywords: Supercritical fluid chromatography ; Sulphur dioxide mobile phase ; Capillary and packed columns ; Column degradation
    Source: Springer Online Journal Archives 1860-2000
    Topics: Chemistry and Pharmacology
    Notes: Summary The properties of sulphur dioxide as mobile phase in supercritical fluid chromatography have been examined. With neat supercritical sulphur dioxide polysiloxane and silica stationary phases were stripped from the columns and clogged the restrictors. The injector rotors needed frequent replacement and epoxy glued fused silica columns went to pieces. With a mixture of 20% sulphur dioxide in carbon dioxide, the problems were significantly reduced, but not completely. A series of columns were examined and all were eventually degraded, with the exception of a porous graphitic carbon column. The adsorptive properties of this material was too high, however, to be of any practical use with the SO2−CO2 mixture. The retention of polycyclic aromatic hydrocarbons as well as several other compounds was significantly reduced in 20% SO2 in CO2, compared to the retention in neat carbon dioxide, and good peak shapes were obtained before the columns became degraded. Before further progress can be made, however, capillary columns with stationary phases which can better withstand mixtures of sulphur dioxide and carbon dioxide are needed.
    Type of Medium: Electronic Resource
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