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  • 1995-1999  (3)
  • Phenylalanine  (2)
  • Ethanol
  • 1
    Digitale Medien
    Digitale Medien
    Springer
    Zeitschrift für Lebensmittel-Untersuchung und -Forschung 205 (1997), S. 158-164 
    ISSN: 1431-4630
    Schlagwort(e): Key words Adulteration ; Carbon-13 ; Citric acid ; Ethanol ; Malic acid ; Tartaric acid
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Werkstoffwissenschaften, Fertigungsverfahren, Fertigung
    Notizen: Abstract  The δ13C-values of organic acids and their correlations to those of the sugar and ethanol, respectively, from 57 EU data bank wines of the Rheinpfalz area (years 1991 – 1993) and from some of their corresponding musts have been determined. In addition to the well established difference between fermented sugar and ethanol (Δδ13C = –1.7±0.2‰), a new constant correlation was found in wine for ethanol and citric acid (Δδ13C = +2.4±0.4‰). From this result a fixed δ-value difference for citric acid in wine to the fermented sugar of +0.7±0.6‰ can be deduced. The δ13C-values of L-malic acid and L-tartaric acid in must were not altered by the alcoholic fermentation; they should therefore directly provide access to the δ13C-value of the natural sugar in must. However, in non-adulterated wines the expected δ13C-value differences between these acids and ethanol showed unsatisfactory correlation coefficients. For L-malate this is attributed to the secondary (partial) degradation of this acid by the malolactic fermentation; a corresponding correction is envisaged in order to make L-malate available as an internal standard. As a reason for the unsatisfactory correlation between L-tartaric acid and ethanol, it is supposed that the time of its maximum biosynthesis period does not coincide with that of glucose in the grape ripening period.
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 2
    Digitale Medien
    Digitale Medien
    Springer
    European journal of pediatrics 155 (1996), S. S97 
    ISSN: 1432-1076
    Schlagwort(e): Key words Phenylketonuria ; Intelligence ; Education ; Professional career ; Phenylalanine ; control
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract The intellectual status and professional careers of 51 young adults with phenylketonuria whose treatment started before 3 months of age are described. Their mean IQ was 97 (SD = 16). Of the IQs, 4% were more than 2 SD below the norm. The distribution of types of schooling of the patients was comparable to that in the German population. The professional careers of nearly all the patients were according to their educational level. Within the sample the outcome was significantly correlated with phenylalanine (Phe) control, even when the patients’ social background was statistically taken into account. The main influence of Phe on intelligence seems to occur during the first decade of life since IQ data remain stable even after Phe levels increased during adolescence.
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 3
    ISSN: 1432-1076
    Schlagwort(e): Key words Phenylketonuria ; Phenylalanine ; Magnetic resonance ; imaging ; Evoked potentials ; Myelination
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract White matter abnormalities on MRI have been observed in phenylketonuria (PKU) patients with late onset neurological symptoms as well as in neurologically inconspicious patients. We investigated 14 early treated adolescents at an age between 12 and 17 years (mean age 14.3 years) with classical PKU as well as one retarded patient with atypical PKU by cranial MRI with spinecho T1-, T2- and proton density sequences. Clinical examination was normal. Visual evoked potential (VEP) examination showed a prolonged latency of peak P100 (mean 122.6 ms; control mean 115.9) and IQ testing showed a mean IQ of 101.1. To investigate the influence of plasma phenylalanine (Phe) levels three approaches were used: Phe was determined for the day of MRI, for a period of 6 months prior to MRI and for lifetime up to 12 years. MRI scans revealed areas of abnormally increased signal intensity on T2-weighted and proton density images in 12 (86%) patients, preferably involving the parieto-occipital lobes. MRI of the patient with atypical PKU was normal. MRI findings correlated most strongly to long-term dietary control up to 12 years. We found no correlation with the other parameters of biochemical control, IQ or VEP latency. The nature and prognosis of MRI abnormalities in neurologically normal PKU patients remain unclear although abnormalities in VEPs which were not associated with the degree of MRI abnormalities in our sample indicate a disturbance in myelination along the visual pathways.
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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