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  • 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
    ESR-Untersuchungen an bestrahlten PhosphatgläsernProfessor Günther Rienäcker zum 70. Geburtstage am 13. Mai 1974 gewidmet. (1974)
    Weinheim : Wiley-Blackwell
    Zeitschrift für anorganische Chemie 405 (1974), S. 286-298 
    Details
    ISSN: 0044-2313
    Keywords: Chemistry ; Inorganic Chemistry
    Source: Wiley InterScience Backfile Collection 1832-2000
    Topics: Chemistry and Pharmacology
    Description / Table of Contents: ESR Studies of Radiation Processes in Phosphate GlassesRadiation processes in phosphate glasses are investigated by ESR and optical measurements. ESR shows a hole-centre in the systems K2O/P2O5 and K2O/MGO/P2O5 which depends on manner and concentration of the cations. Its nature is explained in somewhat a different way than given by BEEKENKAMP or by MIURA and HASEGAWA. Another hole-centre which is independent of cations is discussed in agreement with optical investigations. The electrons set free by radiation react with O2 solved in the glass or with cations. The particels Ag0, Ag2+, Ag+2 have been found and Agn+4 may be present too in the system K2O/MgO/Ag2O/P2O5. The system K2O/MgO/Tl2O/P2O5 shows analogous reactions, but full explanation of the lines could not yet be given.
    Notes: Durch ESR-Untersuchungen und Vergleiche mit optischen Messungen werden Aussagen über die durch Bestrahlung in Phosphatgläsern ablaufenden Vorgänge gemacht. In den Systemen K2O/P2O5 und K2O/MgO/P2O5 wird durch ESR-Messungen ein kationenabhängiges Lochzentrum gefunden, dessen Natur in Abweichung von den Modellen nach BEEKENKAMP und nach MIURA u. HASEGAWA erklärt wird. Ein weiteres kationenunabhängiges Lochzentrum wird in Übereinstimmung mit optischen Untersuchungen gedeutet. Die durch Bestrahlung freigesetzten Elektronen lagern sich an im Glas gelösten Sauerstoff (O2) an oder reagieren mit den Kationen. Im System K2O/MgO/Ag2O/P2O5 werden die Teilchen Ag0, Ag2+, Ag+2 gefunden und Agn+4 wahrscheinlich gemacht. Für das System K2O/MgO/Tl2O/P2O5 gelten analoge Verhältnisse. Eine ausführliche Zuordnung konnte aber dabei noch nicht vorgenommen werden.
    Additional Material: 6 Ill.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1002/zaac.19744050309
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  • 6
    Electronic Resource
    Electronic Resource
    2,4,6-Tris(tert.butyl)phenyl-substituierte Phosphine (1982)
    Weinheim : Wiley-Blackwell
    Zeitschrift für anorganische Chemie 488 (1982), S. 75-79 
    Details
    ISSN: 0044-2313
    Keywords: Chemistry ; Inorganic Chemistry
    Source: Wiley InterScience Backfile Collection 1832-2000
    Topics: Chemistry and Pharmacology
    Description / Table of Contents: 2,4,6-Tris(tert.butyl)phenyl Substituted PhosphinesTris(tert.butyl)phenyl-lithium reacts with PCl3 to give 2,4,6-tris(tert.butyl)phenyldihalogenophosphine which is reduced by LiAlH4 yielding 2,4,6-tris(tert.butyl)phenylphosphine. The same reaction by using (CH3)3Si—CH2—PCl2 leads to 2,4,6-tris(tert.butyl)phenyl-trimethylsilylmethylhalogenophosphine. Thermal treatment of this compound results under elimination of HCl and (CH3)3SiCl in the formation of . The nmr data of the compounds synthesized are discussed.
    Notes: Tris(tert.butyl)phenyl-lithium reagiert mit PCl3 oder (CH3)3Si—CH2—PCl2 zum 2,4,6-Tris(tert.butyl)phenyl-dihalogenphosphin bzw. entsprechenden -trimethylsilylmethylhalogenphosphin. Während sich ersteres mit LiAlH4 in das 2,4,6-Tris(tert.butyl)phenylphosphin überführen läßt, liefert letzteres durch thermische Beanspruchung nach Abspalten von HCl bzw. (CH3)3SiCl die Phosphine mit zweifach koordiniertem Phosphor. Die 1H- sowie 31P-NMR-Daten der dargestellten Verbindungen werden diskutiert.
    Additional Material: 1 Tab.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1002/zaac.19824880109
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  • 7
    Electronic Resource
    Electronic Resource
    Reaktion des aktivierten 1,2-Diphosphinobenzens mit Diphenylcarbodiimid (1985)
    Weinheim : Wiley-Blackwell
    Zeitschrift für anorganische Chemie 529 (1985), S. 216-221 
    Details
    ISSN: 0044-2313
    Keywords: Chemistry ; Inorganic Chemistry
    Source: Wiley InterScience Backfile Collection 1832-2000
    Topics: Chemistry and Pharmacology
    Description / Table of Contents: Reaction of Activated 1,2-Diphosphinobenzene with Diphenylcarbodiimide.The reaction behaviour of 1,2-(Ptms2)2C6H4 and 1,2-(PHLi)2C6H4 towards Ph—N=C=N—Ph leads to phosphaguanidino derivatives of 1,2-diphosphinobenzene and 2-(phenyltrimethylsilylamino)-3-trimethylsilyl-1,3-benzodiphosphole. The structure of the compounds are discussed on the basis of their n.m.r. spectra.
    Notes: Die Umsetzungen von Tetrakis(trimethylsilyl)-1,2-diphosphinobenzen sowie von 1,2-Di(lithiophosphino)benzen mit Diphenylcarbodiimid führen zu Phosphaguanidino-derivaten des 1,2-Diphosphinobenzens sowie zum 2-(Phenyl-trimethylsilylamino)-3-trimethylsilyl-1,3-Benzodiphosphol. Die Strukturen der dargestellten Verbindungen werden anhand der NMR-Daten diskutiert.
    Additional Material: 1 Tab.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1002/zaac.19855291027
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  • 8
    Electronic Resource
    Electronic Resource
    Über die Cycloaddition von Dithiocyan und Trithiocyan mit Hexafluoraceton und Folgeprodukte aus der Spaltung der Schwefel-Schwefel-Bindung mit elementarem Chlor im Dithiocyan-Hexafluoraceton-AdduktProfessor Gerhard Gattow zum 60. Geburtstage am 6. Februar 1986 gewidmet (1986)
    Weinheim : Wiley-Blackwell
    Zeitschrift für anorganische Chemie 532 (1986), S. 131-136 
    Details
    ISSN: 0044-2313
    Keywords: Chemistry ; Inorganic Chemistry
    Source: Wiley InterScience Backfile Collection 1832-2000
    Topics: Chemistry and Pharmacology
    Description / Table of Contents: Cycloaddition of Dithiocyanogen and Trithiocyanogen with Hexafluoroacetone and Products of the Cleavage of the Sulfur-Sulfur Bond in Dithiocyan-Hexafluoroacetone Adduct by Elemental ChlorineThe reaction of dithiocyanogen and trithiocyanogen with hexafluoroacetone (HFA) leads to the cycloaddition products (SCN)2 · 4 HFA 1 and S(SCN)2 · 4 HFA 2. These are the first reactions of (SCN)2 and S(SCN)2 without cleavage of the S - S bonds. Elemental chlorine cleaves in 1 the S—S bond and 3 is formed. In 3 the chlorine atom can be replaced by the rests  - CN 4, (C6H11)2N -  5, and n-C4H9- 6. 6 eliminates one molecule of HFA by warming up and the four-membered ring 7 is formed. The compounds were characterized on the basis of mass and nmr spectra.
    Notes: Die Reaktion von Dithiocyan und Trithiocyan mit Hexafluoraceton (HFA) führt zu den Cycloadditionsprodukten (SCN)2 · 4 HFA 1 und S(SCN)2 · 4 HFA 2. Es sind die ersten Reaktionen des (SCN)2 und S(SCN)2, die nicht unter Spaltung der S—S-Bindungen verlaufen. Durch elementares Chlor wird 1 an der S—S-Bindung gespalten und es entsteht 3. In 3 kann das Chloratom durch die Reste  - CN 4, (C6H11)2N -  5 und n-C4H9- 6 ersetzt werden. Beim Erwärmen verliert 6 ein Molekül HFA und es entsteht der viergliedrige Ring 7. Die Verbindungen wurden anhand ihrer Massen- und NMR-Spektren eindeutig charakterisiert.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1002/zaac.19865320118
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  • 9
    Electronic Resource
    Electronic Resource
    Carbosilylierte Phospha-alkeneProfessor Rolf Appel zum 60. Geburtstage am 25. Februar 1981 gewidmet (1981)
    Weinheim : Wiley-Blackwell
    Zeitschrift für anorganische Chemie 473 (1981), S. 85-90 
    Details
    ISSN: 0044-2313
    Keywords: Chemistry ; Inorganic Chemistry
    Source: Wiley InterScience Backfile Collection 1832-2000
    Topics: Chemistry and Pharmacology
    Description / Table of Contents: Carbosilylated phospha-alkenesLithium-tris-trimethylsilylmethanide, (Me3Si)3C-Li, reacts with various organodichlorophosphines to give tris-(trimethylsilyl)-methyl-organochlorophosphines, the termal treatment of which under elimination of trimethylchlorosilane results in formation of the title compounds of the general formula B.NMR-data as well as the synthesis of compounds A and B will be discussed.
    Notes: Lithium-tris-trimethylsilylmethanid, (Me3Si)3C-Li, reagiert mit verschieden substituierten Organyldichlorphosphinen zur Tris-Trimethylsilylmethyl-organylchlorphosphinen A, deren thermische Beanspruchung unter Abspaltung von Trimethylchlorsilan zu den Titelverbindungen der allgemeinen Formel B führt.Die NMR-spektroskopischen Daten der Verbindungen des Typs A und B sowie deren Synthese wer-den diskntiert.
    Additional Material: 2 Tab.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1002/zaac.19814730209
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  • 10
    Electronic Resource
    Electronic Resource
    Alkali-Phosphorverbindungen und ihr reaktives Verhalten. 79. NMR-Untersuchungen an Alkali-organophosphiden. I (1982)
    Weinheim : Wiley-Blackwell
    Zeitschrift für anorganische Chemie 495 (1982), S. 115-119 
    Details
    ISSN: 0044-2313
    Keywords: Chemistry ; Inorganic Chemistry
    Source: Wiley InterScience Backfile Collection 1832-2000
    Topics: Chemistry and Pharmacology
    Description / Table of Contents: Alkali Phosphorus Compounds and their Reactivity. 79. NMR Investigations of Alkali Organophosphides. IThe 31C and 31P NMR spectra display a further separation of ion-pairs of alkali-organophosphides in THF solution after addition of cryptandes . An exception presents LiP(Ph)2, T1—13C-NMR measurements suggest a tetrameric structure in THF.
    Notes: Anhand der 13C- und 31P-NMR-Daten läßt sich zeigen, daß sich die Ionenpaare der Alkali-organophosphide in THF durch Zugabe von Kryptanden entsprechend weiter trennen lassen. Eine Ausnahme bildet LiP(Ph)2, das aufgrund von T1—13C-NMR-Messungen eine tetramere Struktur in THF aufweist.
    Additional Material: 2 Tab.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1002/zaac.19824950111
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