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  • Artikel: DFG Deutsche Nationallizenzen  (8)
  • Phenylketonuria  (4)
  • spirooxazine  (4)
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  • Artikel: DFG Deutsche Nationallizenzen  (8)
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Erscheinungszeitraum
  • 1
    Digitale Medien
    Digitale Medien
    Springer
    European journal of pediatrics 147 (1988), S. 361-367 
    ISSN: 1432-1076
    Schlagwort(e): CNS development ; Dietary treatment ; EEG ; IQ ; Phenylketonuria
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract In 34 children with phenylketonuria (PKU) treated early the prognostic value of the age on institution of the diet (within the first 3 months of life) and of the quality of dietary treatment was determined in two different ways: 1) following intelligence closely (IQ) and (2) evaluating the EEG development up to their 12th (n=34) and 15th (n=18) years of life as appropriate. In general, IQ scores were found to be normal from the 4th–15th years of life. In our group of patients there was no effect on the IQ of the timing of diet onset. Children with “strict” dietary control showed a significantly higher IQ than those with “loose” control. One hundred and fifty-four EEGs (10/20 system, awake with eyes closed) were recorded at intervals of 2 years and conventionally evaluated. The development of alpha-activity was found to be normal. Beta-activity was enhanced. Abnormal EEG findings like general slowing and generalized paroxysmal activity (GPA) with or without spikes were more frequent in children with PKU than in controls, with the exception of focal abnormalities. EEG abnormalities increased with advancing age independently of IQ development and showed no relation to either the age at the onset nor the quality of dietary treatment.
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 2
    Digitale Medien
    Digitale Medien
    Springer
    European journal of pediatrics 146 (1987), S. A17 
    ISSN: 1432-1076
    Schlagwort(e): Phenylketonuria ; Adolescents ; Dietary control ; Intelligence ; Psychological problems
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 3
    ISSN: 1432-1076
    Schlagwort(e): Phenylketonuria ; Biopterin ; Phosphate-eliminating enzyme ; 6-Pyruvoyl tetrahydropterin synthase ; Amniotic fluid ; Erythrocytes
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract Amniocentesis was performed at 19 weeks gestation in a mother who had previously delivered a boy with “dihydrobiopterin synthetase” (DHBS) deficiency. The amniotic fluid contained neopterin in high (136 nmol/l) and biopterin in very low concentrations (1.8 nmol/l). The activity of the phosphate-eliminating enzyme (PEE, also called 6-pyruvoyl tetrahydropterin synthase, substrate: 7,8-dihydroneoptein triphosphate) which is present in liver and erythrocytes and defective in DHBS deficiency, was measured in the erythrocytes of the family members. The fetal sample showed only 2% of the activity of healthy adult controls and was comparable with that of the affected sibling. Obligate heterozygotes had activities around 20% of the controls. Two fetal control samples showed even higher activities than adult erythrocytes. Sepiapterin reductase activities were normal in all cases. At autopsy, PEE deficiency was confirmed in the liver of the fetus. We concluded that DHBS deficiency (and most probably also GTP cyclohydrolase I deficiency) can be diagnosed by metabolite measurements in amniotic fluid. PEE activity is measurable in erythrocytes, although the assay needs to be improved. Since maternal tetrahydrobiopterin does not cross the placenta, treatment of a tetrahydrobiopterin-deficient fetus with tetrahydrobiopterin in utero is not possible.
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 4
    Digitale Medien
    Digitale Medien
    Springer
    European journal of pediatrics 157 (1998), S. 824-830 
    ISSN: 1432-1076
    Schlagwort(e): Key words Intelligence ; Neurology ; Neuropsychology ; Phenylketonuria
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Medizin
    Notizen: Abstract Due to the observation of severe neurological symptoms in single patients as well as brain imaging, neuropsychological and neurophysiological abnormalities, the long-term prognosis of treated phenylketonuria is still under discussion. We investigated the neurological outcome of 57 (24 male, 33 female) patients with phenylketonuria (diet onset 〈3 months) at a mean age of 23.6 (17–33) years in comparison to control subjects. Methods used were a clinical-neurological examination, tests for fine motor abilities, IQ test (WAIS-R), a neuropsychological attention task and MRI (30 patients only). Tremor was increased in the patients (28%) compared to controls (15%). Fine motor abilities were significantly reduced in three areas: hand-wrist steadiness, finger-hand dexterity and hand-wrist speed. Tremor as well as reduced fine motor skills were not associated with treatment-related variables, e.g. diet onset, strictness of biochemical control or amount of MRI white matter change. IQ was lower in patients (mean 97.6) compared to matched control subjects (mean 105.5). IQ at 12 years was correlated with biochemical control from birth up to the age of 12 and remained stable up to adult age, independent of biochemical control after 12 years of age. In contrast to the other outcome parameters, the performance in a neuropsychological attention task was influenced by the concurrent plasma phenylalanine concentration. Specific late-onset neurological impairment was not identified in this sample of early-treated adults with phenylketonuria. Conclusion Careful neurological investigation revealed subtle symptoms of brain damage even after early-initiated treatment in adult patients with phenylketonuria. At present it cannot be excluded that further neurological deterioration could emerge later in life. Thus, patients with phenylketonuria – either on or off diet – should be monitored throughout life.
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 5
    ISSN: 1573-4846
    Schlagwort(e): photochromism ; photochromic coatings ; spirooxazine ; photochromic properties ; mechanical properties
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Chemie und Pharmazie
    Notizen: Abstract The experimental results on the photochromic and mechanical properties of coatings containing 1,3-dihydro-1,3,3-trimethylspiro[2H-indole-2,3′-(3H)-naphth(2,1-b)(1,4) oxazine] (SO) derived from 3-glycidyloxypropyltrimethoxysilane (GPTMS), bisphenol A (BPA) and 1-methylimidazole (MI) by sol-gel processing are presented. It is shown that heat treatment temperature is a conflicting factor to the photochromic intensity (Δ A 0), photostability and abrasion resistance of the photochromic coatings. With increasing densification temperature the matrix rigidity increases leading to a decrease of Δ A 0 and at the same time an enhancement of both abrasion resistance and photostability, the optimum heat treatment temperature is 110°C under our experimental conditions. By the use of certain additives, e.g., fluorosilanes (FAS), not only Δ A 0 but also the photostability and the abrasion resistance of the photochromic coatings have been further improved.
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 6
    Digitale Medien
    Digitale Medien
    Springer
    Journal of sol gel science and technology 8 (1997), S. 927-929 
    ISSN: 1573-4846
    Schlagwort(e): organic-inorganic hybrids ; sol-gel ; photochromic performance ; spirooxazine
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Chemie und Pharmazie
    Notizen: Abstract Organic-inorganic hybrid materials synthesized via sol-gel processing are excellent solid matrices for photochromic dyes like spirooxazine, and the photochromic performance can be further enhanced by introducing suitable additives. This work describes the effects of additives on the photochromic intensity (Δ A 0), decolouration rate (k) and photostability of spirooxazine in sol-gel derived organic-inorganic hybrid matrices. They include fluoro-alkylsilane (FAS), bisphenol A (BPA) and methyl-imidazole (MI). FAS enhances both Δ A 0 and photostability, but has little effect on k. The higher the content of BPA, the higher the Δ A 0 and the higher the photostability, but the lower the k. The effect of MI on Δ A 0 and k is not so considerable as that of BPA and is a little complicated, while significant improvement in photostability is achieved by the addition of MI at an optimum concentration with the presence of BPA at a higher content.
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 7
    ISSN: 1573-4846
    Schlagwort(e): photochromism ; photochromic coatings ; spirooxazine ; photochromic properties ; mechanical properties
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Chemie und Pharmazie
    Notizen: Abstract The experimental results on the photochromic and mechanical properties of coatings containing 1,3-dihydro-1,3,3-trimethylspiro[2H-indole-2,3′-(3H)-naphth(2,1-b)(1,4) oxazine] (SO) derived from 3-glycidyl-oxypropyltrimethoxysilane (GPTMS), bisphenolA (BPA) and 1-methylimidazole (MI) by sol-gel processing are presented. It is shown that heat treatment temperature is a conflicting factor to the photochromic intensity (ΔA 0), photostability and abrasion resistance of the photochromic coatings. With increasing densification temperature the matrix rigidity increases leading to a decrease of ΔA 0 and at the same time an enhancement of both abrasion resistance and photostability, the optimum heat treatment temperature is 110°C under our experimental conditions. By the use of certain additives, e.g., fluorosilanes (FAS), not only ΔA 0 but also the photostability and the abrasion resistance of the photochromic coatings have been further improved.
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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  • 8
    Digitale Medien
    Digitale Medien
    Springer
    Journal of sol gel science and technology 8 (1997), S. 927-929 
    ISSN: 1573-4846
    Schlagwort(e): organic-inorganic hybrids ; sol-gel ; photochromic performance ; spirooxazine
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Chemie und Pharmazie
    Notizen: Abstract Organic-inorganic hybrid materials synthesized via sol-gel processing are excellent solid matrices for photochromic dyes like spirooxazine, and the photochromic performance can be further enhanced by introducing suitable additives. This work describes the effects of additives on the photochromic intensity (ΔA 0), decolouration rate (k) and photostability of spirooxazine in sol-gel derived organic-inorganic hybrid matrices. They include fluoroalkylsilane (FAS), bisphenolA (BPA) and methyl-imidazole (MI). FAS enhances both ΔA 0 and photostability, but has little effect onk. The higher the content of BPA, the higher the ΔA 0 and the higher the photostability, but the lower thek. The effect of MI on ΔA 0 andk is not so considerable as that of BPA and is a little complicated, while significant improvement in photostability is achieved by the addition of MI at an optimum concentration with the presence of BPA at a higher content.
    Materialart: Digitale Medien
    Bibliothek Standort Signatur Band/Heft/Jahr Verfügbarkeit
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