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  • 1
    Digitale Medien
    Digitale Medien
    Hyperamino-acidaemia and hyperammonaemia in epileptic children treated with valproic acid (1988)
    Springer
    European journal of pediatrics 148 (1988), S. 267-269 
    Details
    ISSN: 1432-1076
    Schlagwort(e): Valproic acid ; Hyperammonaemia ; Aminoacidaemia ; Mitochondrial dysfunction
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract Serum amino acid pattern, blood ammonia, and serum valproic acid (VPA) levels were estimated in 75 epileptic children with and without VPA therapy. The incidence of patients with hyperammonaemia (〉60 μmol/l) in patients treated with only VPA or with a combination of VPA and other antiepileptic drugs was 19% and 20% respectively. Hyperammonaemia was not observed in patients receiving other drugs. Elevated levels of serum glycine, alanine and serine were more common in epileptic patients treated with VPA alone and with VPA in combination with other drugs than in patients receiving other drugs. A significant positive correlation was found between the serum levels of glycine and alanine, of glycine and serine, and of serine and alanine. These findings may indicate a mitochondrial dysfunction caused by VPA.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/BF00441418
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 2
    Digitale Medien
    Digitale Medien
    Dietary therapy in two patients with vitamin B12-unresponsive methylmalonic acidemia (1981)
    Springer
    European journal of pediatrics 135 (1981), S. 305-312 
    Details
    ISSN: 1432-1076
    Schlagwort(e): Vitamin B12 unresponsive methylmalonic acidemia ; Protein intolerance ; Dietary therapy
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract The biochemical and therapeutic responses to dietary therapy were studied in a 25-month-old girl and a 1-month-old girl with methylmalonic acidemia (MMA-emia), which was unresponsive to vitamin B12. The minimum daily intake of protein which patients could tolerate and display a good development was between 1.0 and 1.2 g per kg body weight. Supplementation with amino acid mixture devoid of toxic amino acids was required to prevent protein malnutrition when daily protein intake was restricted to 0.6 g per kg body weight. Caloric intake should be sufficient, not only to promote growth but also to prevent a rise in MMA level, especially when a patient has ketoacidosis. It was found that MMA excretion per mg creatinine in random urine specimens correlated significantly with serum MMA and twenty four-hour output of MMA per kg body weight. Therefore measurement of MMA in a single urine specimen is useful for evaluating the in vivo accumulation of MMA.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/BF00442109
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  • 3
    Digitale Medien
    Digitale Medien
    Observation of laser-cooled Be+ -ion clouds in a Penning trap (1995)
    Springer
    Applied physics 61 (1995), S. 285-289 
    Details
    ISSN: 1432-0649
    Schlagwort(e): 32.80.Pj ; 05.70.Fh
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Physik
    Notizen: Abstract Be+ ions trapped in a Penning trap are laser-cooled to about 10 mK. The excitation spectra of ion clouds containing about 500 ions are obtained by scanning the frequency of the cooling laser and discontinuities in these spectra are observed because of phase transitions. When the cooled ions are heated electrically by applying an rf voltage, no phase transition occurs and the spectra become continuous. Two-dimensional measurement of the ion clouds is carried out and the abrupt change in the shape of the ion cloud due to the phase transition is observed. When many ions are trapped and cooled, the phase transition occurs partially and a transient state where two states are mixed can be observed. The static properties of the ions are also measured by using an additional probe laser and the results of experimental measurements are compared with theoretical predictions.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/BF01082048
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  • 4
    Digitale Medien
    Digitale Medien
    Laser cooling of a small number of Be+ ions in a penning trap (1993)
    Springer
    Applied physics 57 (1993), S. 141-144 
    Details
    ISSN: 1432-0649
    Schlagwort(e): 32.80.Pj ; 05.70.Fh ; 34.40.+n
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Physik
    Notizen: Abstract Be+ ions stored in a Penning trap were cooled by a laser beam perpendicular to the magnetic field. The cooled ions are strongly coupled and phase transitions of up to 100 ions were observed. In experiments with only a few ions stored in the trap, a stepwise decrease in fluorescence intensity was observed. All steps are of the same size and so every step is attributed to a single ion. The discrete changes in fluorescence occurred more frequently when the background pressure was increased, caused by collisions between stored ions and background neutral molecules.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/BF00425998
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