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  • Polymer and Materials Science  (23)
  • Phenylketonuria  (4)
  • 1
    Electronic Resource
    Electronic Resource
    Long-term development of intelligence (IQ) and EEG in 34 children with phenylketonuria treated early (1988)
    Springer
    European journal of pediatrics 147 (1988), S. 361-367 
    Details
    ISSN: 1432-1076
    Keywords: CNS development ; Dietary treatment ; EEG ; IQ ; Phenylketonuria
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: 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.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00496411
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  • 2
    Electronic Resource
    Electronic Resource
    Prenatal diagnosis of “dihydrobiopterin synthetase” deficiency, a variant form of phenylketonuria (1986)
    Springer
    European journal of pediatrics 145 (1986), S. 176-178 
    Details
    ISSN: 1432-1076
    Keywords: Phenylketonuria ; Biopterin ; Phosphate-eliminating enzyme ; 6-Pyruvoyl tetrahydropterin synthase ; Amniotic fluid ; Erythrocytes
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: 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.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00446058
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  • 3
    Electronic Resource
    Electronic Resource
    Continuation vs discontinuation of low-phenylalanine diet in PKU adolescents (1987)
    Springer
    European journal of pediatrics 146 (1987), S. A17 
    Details
    ISSN: 1432-1076
    Keywords: Phenylketonuria ; Adolescents ; Dietary control ; Intelligence ; Psychological problems
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00442050
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  • 4
    Electronic Resource
    Electronic Resource
    Neurological outcome in adult patients with early-treated phenylketonuria (1998)
    Springer
    European journal of pediatrics 157 (1998), S. 824-830 
    Details
    ISSN: 1432-1076
    Keywords: Key words Intelligence ; Neurology ; Neuropsychology ; Phenylketonuria
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: 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.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004310050945
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  • 5
    Electronic Resource
    Electronic Resource
    High temperature sulfidation of Fe-Mn alloys and Mn in H2-H2SDedicated to Professor Dr. Alfred Rahmel on the occasion of his 60th birthday (1987)
    Weinheim [u.a.] : Wiley-Blackwell
    Materials and Corrosion/Werkstoffe und Korrosion 38 (1987), S. 498-506 
    Details
    ISSN: 0947-5117
    Keywords: Chemistry ; Polymer and Materials Science
    Source: Wiley InterScience Backfile Collection 1832-2000
    Topics: Mechanical Engineering, Materials Science, Production Engineering, Mining and Metallurgy, Traffic Engineering, Precision Mechanics
    Description / Table of Contents: Sulfidierung von Fe-Mn-Legierungen und Mn bei hohen TemperaturenDie Sulfidierung von Fe-Mn-Legierungen mit 20-50% Mn und von reinem Mn wurde bei 973 K, 1073 K und 1173 K in H2-H2S Atmosphären bei konstantem pS2 oder konstantem pH2S/pH2 untersucht, unter Bedingungen, bei denen nur MnS gebildet wird. Die Sulfidierungskinetik ist vorwiegend parabolisch, aber bedingt durch zwei verschiedene Diffusionsvorgänge. Bei der Sulfidierung der Fe-Mn-Legierungen bei 973 K und 1073 K ist die Diffusion der Mn-Atome in der Legierung der geschwindigkeitsbestimmende Vorgang, und es wird eine verzahnte Grenzfläche Sulfidschicht/Legierung beobachtet. Bei 1173 K erfolgt ein Übergang zur Kontrolle der Sulfidierungsgeschwindigkeit durch Diffusion des Mn in der Sulfidschicht und ein Übergang von der verzahnten Grenzfläche zur ebenen Grenzfläche.
    Notes: The sulfidation behaviour of a range of Fe-Mn alloys and pure Mn were studied at 973 K, 1073 K, and 1173 K in H2-H2S atmospheres at constant pS2 or constant pH2S/pH2. The sulfidation kinetics were parabolic in most cases, but two different diffusion processes affect the kinetics. In the sulfidation of the Fe-Mn alloys at 973 K and 1073 K, the diffusion of manganese atoms in the alloy is the rate-determining step and an interlocked scale/alloy interface is observed. At 1173 K there is a transition to rate-control by the diffusion of manganese in the sulfide scale connected with transition from rugged to a planar scale/alloy interface.
    Additional Material: 13 Ill.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1002/maco.19870380907
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  • 6
    Electronic Resource
    Electronic Resource
    Einflüsse von HCl und N2 auf die Hochtemperaturkorrosion in Atmosphären mit niedrigem Sauerstoffdruck (1989)
    Weinheim [u.a.] : Wiley-Blackwell
    Materials and Corrosion/Werkstoffe und Korrosion 40 (1989), S. 642-650 
    Details
    ISSN: 0947-5117
    Keywords: Chemistry ; Polymer and Materials Science
    Source: Wiley InterScience Backfile Collection 1832-2000
    Topics: Mechanical Engineering, Materials Science, Production Engineering, Mining and Metallurgy, Traffic Engineering, Precision Mechanics
    Description / Table of Contents: Influence of HCl and N2 on high temperature corrosion in atmospheres with low oxygen pressuresThe effects of HCl and N2 on the high temperature corrosion in atmospheres with low oxygen pressures were investigated.Investigations of the alloy Fe-20Cr in He-H2-H2-O-HCl atmospheres at 1173 K under conditions, where no condensed chlorides could be formed, show the simultaneous growth of Cr2O3 and evaporation of CrCl2. The kinetics of the reactions can be described by growth of Cr2O3 according to the parabolic rate law and transport of CrCl2 in the gas flow following a linear rate law.During heating of the samples in He-H2-H2O-HCl condensed chlorides are formed which decrease the adherence of the oxide layers and cause oxide spallation during cooling. This can be avoided either by preoxidation in H2-H2O or heating in dry hydrogen. The porosity of Cr2O3 layers is not enhanced when HCl is added to the gas atmosphere.The oxidation and evaporation is enhanced in N2-H2-H2O-HCl atmospheres compared to He-H2-H2O-HCl atmospheres. The Cr2O3 scales are coarse grained and poros. The enhanced porosity is caused by the nucleation and growth of chromium nitrides in the transient state of oxidation.
    Notes: Der Einfluß von gasförmigem HCl und Stickstoff auf die Hochtemperatur-Korrosion in Atmosphären mit niedrigem Sauerstoffpartialdruck wurde untersucht.Untersuchungen mit der Legierung Fe-20Cr in He-H2-H2O-HCl-Atmosphären bei 1173 K unter Bedingungen, bei denen keine kondensierten Chloride gebildet werden, zeigten, daß gleichzeitig Wachstum von Cr2O3 und Abdampfen von CrCl2 erfolgt. Die Kinetik der Gesamtreaktion läßt sich durch Überlagerung eines parabolischen Zeitgesetzes für das Oxidwachstum mit einem linearen Zeitgesetz für die Abdiffusion des Chlorides in den Gasstrom beschreiben.Beim Aufheizen der Proben in He-H2-H2O-HCl bilden sich kondensierte Chloride, die die Haftung der Oxidschichten beeinträchtigen und Abplatzen beim Abkühlen verursachen. Voroxidation in H2-H2O oder Aufheizen in getrocknetem Wasserstoff verhindern dies. Die Porosität von Cr2O3-Schichten wird durch den Zusatz von HCl zur Gasatmosphäre nicht erhöht.In N2-H2-H2O-HCl-Atmosphären ist die Oxidation und das Abdampfen beschleunigt gegenüber He-H2-H2O-HCl-Atmosphären. Die gebildeten Cr2O3-Schichten sind grobkörnig und porös. Ursache der erhöhten Porosität ist die Keimbildung und das Wachstum von Chromnitriden im Anfangsstadium der Oxidation.
    Additional Material: 16 Ill.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1002/maco.19890401104
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  • 7
    Electronic Resource
    Electronic Resource
    Einfluß von HCl und Cl2 auf die Hochtemperaturkorrosion des 2 1/4 Cr 1 Mo-Stahls in Atmosphären mit hohen Sauerstoffdrücken (1990)
    Weinheim [u.a.] : Wiley-Blackwell
    Materials and Corrosion/Werkstoffe und Korrosion 41 (1990), S. 303-307 
    Details
    ISSN: 0947-5117
    Keywords: Chemistry ; Polymer and Materials Science
    Source: Wiley InterScience Backfile Collection 1832-2000
    Topics: Mechanical Engineering, Materials Science, Production Engineering, Mining and Metallurgy, Traffic Engineering, Precision Mechanics
    Description / Table of Contents: Influence of HCl and Cl2 on high temperature corrosion of 2 1/4Cr 1 Mo Steel in atmospheres with high oxygen pressuresThe oxidation of the 2 1/4 Cr 1 Mo steel was investigated at 773 K in oxidizing He-O2-HCl atmospheres. The addition of HCl to He-O2 atmospheres leads to accelerated oxidation rates. Below porous and cracked oxide scales condensed chlorides are formed. At low HCl pressures 0-1000 vppm the “active oxidation” is determining the corrosion process; i.e. oxidation of evaporating chlorides within the oxide scale. For higher HCl contents 1000-3000 vppm the corrosion behaviour changes to paralinear; i.e. simultaneous parabolic oxide growth and linear mass loss by chloride evaporation.
    Notes: Die Oxidation des 2 1/4Cr1Mo-Stahls in oxidierenden He-O2-HCl-Atmosphären bei 773 K wurde untersucht. Der Zusatz von HCl zu He-O2-Atmosphären führt zu stark beschleunigter Oxidation. Unter porösen und rissigen Oxidschichten bilden sich feste Chloride. Bei niedrigen HCl-Drücken 0-1000 vppm bestimmt „aktive Oxidation“ den Korrosionsprozeß; d.h. Oxidation flüchtiger Chloride innerhalb der Oxidschicht. Bei höheren HCl-Drücken 1000-3000 vppm erfolgt ein Übergang zum paralinearen Korrosionsverhalten, d.h. gleichzeitiges parabolisches Oxidwachstum und lineare Massenabnahme durch das Abdampfen von Chloriden.
    Additional Material: 4 Ill.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1002/maco.19900410602
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  • 8
    Electronic Resource
    Electronic Resource
    Untersuchungen zur radikalischen Copolymerisation von Acrylnitril mit α-Olefinen. 5. Mitt. Bestimmung der r-Werte am System Acrylnitril/Allylbenzol4. Mitt.: siche [8]Teil der Dissertation von H. S. Trang, Technische Universität Dresden, Sektion Chemie, 1978Herrn Professor Dr. Vasilij Vladimirovič Koršak zur Vollendung des 70. Lebensjahres gewidmet. (1979)
    Weinheim : Wiley-Blackwell
    Acta Polymerica 30 (1979), S. 98-102 
    Details
    ISSN: 0323-7648
    Keywords: Chemistry ; Polymer and Materials Science
    Source: Wiley InterScience Backfile Collection 1832-2000
    Topics: Chemistry and Pharmacology , Physics
    Description / Table of Contents: The r-values of the monomer pair acrylonitrile/allylbenzene in the radical bulk and solution polymerization, using hexamethylphosphoric acid triamide, dimethylsulfoxide, γ-butyrolactone, ethylene carbonate and nitromethane as solvents, have been determind. A copolymerization equation is derived, considering the degradative chain transfer, associate formation and solvent effect, by which the deviations from the Mayo-Lewis concept and the solvent effects can be explained.
    Notes: Die r-Werte des Monomerpaares Acrylnitril/Allylbenzol wurden bei der radikalischen Copolymerisation in Substanz bzw. in den Lösungsmitteln Hexamethylphosphorsäuretriamid, Dimethylsulfoxid, γ-Butyrolacton, Ethylencarbonat und Nitromethan bestimmt. Unter Berücksichtigung der degradativen Kettenübertragung, der Assoziatbildung und des Lösungsmitteleinflusses wurde eine Copolymerisationsgleichung abgeleitet, mit deren Hilfe die Abweichung vom Mayo-Lewis-Modell und Lösungsmitteleffekte interpretierbar sind.
    Additional Material: 1 Ill.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1002/actp.1979.010300206
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  • 9
    Electronic Resource
    Electronic Resource
    Untersuchungen zur radikalischen Copolymerisation von Acrylnitril mit α-Olefinen. 6. Mitt. Zur Assoziatbildung von Allybenzolsiehe 5. Mitt. [1] (1979)
    Weinheim : Wiley-Blackwell
    Acta Polymerica 30 (1979), S. 409-411 
    Details
    ISSN: 0323-7648
    Keywords: Chemistry ; Polymer and Materials Science
    Source: Wiley InterScience Backfile Collection 1832-2000
    Topics: Chemistry and Pharmacology , Physics
    Description / Table of Contents: In order to investigate the deviation of the copolymerization of acrylonitrile with allyl benzene from the Mayo-Lewis model caused by association the temperature-dependence of the r-values in different solvents and at different monomer ratios was determined. Independent comparison is achieved by 1H and 13C-NMR spectra of allyl benzene in tetrachloromethane and nitromethane.
    Notes: Zur Untersuchung der Abweichung vom MAYO-LEWIS-Modell bei der Acrylnitril-Allylbenzol-Copolymerisation durch Assoziatbildung wurde die Temperaturabhängigkeit der r-Werte in verschiedenen Lösungsmitteln und bei unterschiedlichen Monomermischungsverhältnissen bestimmt. Zum unabhängigen Vergleich dienten 1H- und 13C-NMR-Spektren des Allylbenzols in Tetrachlormethan und Nitromethan.
    Additional Material: 7 Ill.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1002/actp.1979.010300706
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  • 10
    Electronic Resource
    Electronic Resource
    Herstellung und Anwendung von neuen Nanokompositen (1995)
    Weinheim : Wiley-Blackwell
    Chemie Ingenieur Technik - CIT 67 (1995), S. 1157-1157 
    Details
    ISSN: 0009-286X
    Keywords: Chemistry ; Polymer and Materials Science
    Source: Wiley InterScience Backfile Collection 1832-2000
    Topics: Chemistry and Pharmacology , Process Engineering, Biotechnology, Nutrition Technology
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1002/cite.3306709107
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