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  • 1985-1989  (7)
  • Inorganic Chemistry  (4)
  • Phenylketonuria  (3)
  • 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
    New Prophosphatranes: Novel intermediates to five-coordinate phosphatranesDedicated to Professor Kurt Issleib on his 70th Birthday (1989)
    Weinheim : Wiley-Blackwell
    Zeitschrift für anorganische Chemie 578 (1989), S. 75-80 
    Details
    ISSN: 0044-2313
    Keywords: Chemistry ; Inorganic Chemistry
    Source: Wiley InterScience Backfile Collection 1832-2000
    Topics: Chemistry and Pharmacology
    Description / Table of Contents: Neue Prophosphatrane: Neuartige Zwischenstufen auf dem Wege zu pentakoordinierten PhosphatranenDie Reaktion von N(CH2CH2NHMe)3 mit P(NMe2)3 oder P(NMe2)2Cl ergibt die Phosphatranvorstufe N(CH2CH2NMe)3P. Dessen Umsetzung mit Oxydationsmitteln ode Elektrophilen führt zu nicht-transannular-stabilisierten, tetrakoordinierten Derivaten N(CH2CH2NMe)3P—X, (X: O, S, Se, N3Ph, NPh, CS2, 1/2 PtCl2). Die Strukturen der beschriebenen Verbindungen werden anhand ihrer 1H-, 13C- und 31P-NMR-Daten diskutiert.
    Notes: The reaction of N(CH2CH2NHMe)3 with P(NMe2)3 or P(NMe2)2Cl yields the phosphatrane precursor N(CH2CH2NMe)2P,4. With oxydizing agents or electrophiles 4 is converted to the untransannulated four-coordinated derivatives N(CH2CH2NMe)3P—X, (X: O, S, Se, N3Ph, NPh, I CS2, 1/2 PtCl2). The structures of the compounds described are discussed by 1H, 13C, and 31P n.m.r. data.
    Additional Material: 1 Tab.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1002/zaac.19895780109
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  • 5
    Electronic Resource
    Electronic Resource
    C-metallierte Aminophosphine 1. α-lithiierte DimethylaminophosphineProfessor Marianne Baudler zum 65. Geburtstage am 27. April 1986 gewidmet (1986)
    Weinheim : Wiley-Blackwell
    Zeitschrift für anorganische Chemie 535 (1986), S. 47-55 
    Details
    ISSN: 0044-2313
    Keywords: Chemistry ; Inorganic Chemistry
    Source: Wiley InterScience Backfile Collection 1832-2000
    Topics: Chemistry and Pharmacology
    Description / Table of Contents: C-metalated Aminophosphines. 1. α-lithiated DimethylaminophosphinesThe preparation of dimethylaminophosphines of the general formula Z—CH2—Pdma2 and (Z—CH2)2Pdma, respectively (Z = (CH3)3Si, C6H5), their monometalation and the reactions of these metalated species with chlorosilanes, bis-(dimethylamino)-chlorophosphine and trimethylsilylmethyl-dichlorophosphine are described. The n.m.r. spectra of the prepared compounds are discussed.
    Notes: Es wird die Darstellung von Dimethylaminophosphinen der allgemeinen Formulierung Z—CH2—Pdma2 bzw. (Z—CH2)2Pdma, deren Monolithiierung zu Z—CH(Li)—Pdma2 bzw. Z—CH(Li)—P(dma)—CH2—Z (Z = (CH3)3Si, C6H5) und die Reaktionen dieser lithiierten Spezies mit Chlorsilanen sowie Bis-(dimethylamino)-chlorphosphin und Trimethylsilylmethyldichlorphosphin beschrieben. Die NMR-Daten der dargestellten Verbindungen werden diskutiert.
    Additional Material: 1 Ill.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1002/zaac.19865350407
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  • 6
    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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  • 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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