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  • 2005-2009
  • 1980-1984  (2)
  • 1925-1929
  • 1910-1914
  • Phenylketonuria  (1)
  • SAXS  (1)
  • 1
    ISSN: 1432-1076
    Keywords: Neonatal screening ; Phenylketonuria ; Maple syrup urine disease ; Homocystinuria ; Galactosemia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A nationwide neonatal sreening program for phenylketonuria (PKU), maple syrup urine disease (MSUD), homocystinuria, histidinemia and galactosemia was started in Japan in 1977. The total number of infants screened had reached 6,311,754 by March, 1982. A follow-up study revealed the incidence of the disease in Japan: 1/108,823 for PKU; 1/450,840 for hyperphenylalaninemia (HPA); 1/1,577,939 for biopterin deficiency; 1/525,980 for MSUD; 1/1,051,959 for homocystinuria; 1/8,371 for histidinemia, and 1/788,969 for galactosemia type 1. The incidences of PKU, HPA, homocystinuria, and galactosemia (type 1) were found to be markedly low in Japan as compared with those in Caucasian countries. There was no great difference in the incidence of MSUD between both. On the other hand, the incidence of histidinemia was higher in Japan. It was found that most of the patients with PKU, HPA, MSUD, homocystinuria, or galactosemia are developing normally due to the early initiation of dietary treatment. These results clearly indicate that the neonatal mass screening program plays a great role in preventing the occurrence of handicapped children.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1435-1536
    Keywords: Mn(II) and Ni(II) salts of E-AA ; Ion-Clusters ; Dielectric Relaxation ; ESR ; SAXS
    Source: Springer Online Journal Archives 1860-2000
    Topics: Chemistry and Pharmacology , Mechanical Engineering, Materials Science, Production Engineering, Mining and Metallurgy, Traffic Engineering, Precision Mechanics
    Description / Table of Contents: Zusammenfassung Die Existenz von Ionen-Aggregaten in Äthylen-Acrylsäure-Mischpolymerisaten, die durch Mangan-acetat und Nickel-acetat neutralisiert wurden, wurden durch dielektrische Messungen im Temperaturbereich von -180 bis 80 °C und für einige Frequenzen zwischen 30 Hz und 100 kHz, studiert. Dieβ′- undγ-Relaxationsprozesse wurden beobachtet, die durch die Mikro-Brownschen Molekülbewegungen im höheren Temperaturbereich vonT g und durch lokale Molekülbewegungen im tieferen Temperaturbereich vonT g verursacht werden. Die Veränderung in Intensität und Relaxationstemperatur für die beiden Relaxationsprozesse mit dem Grad der Neutralisation deutet auf die Bildung von Ionen-Aggregaten in Bereichen höherer Neutralisation als einem Wert zwischen 3.3 und 14% für das Mn(II)-Salz und als 14% für das Ni(II)-Salz an. Diese Bildung von Ionen-Aggregaten wurde ebenso durch Elektronenspinresonanz (ESR) und durch Kleinwinkel-Röntgenstreuung (KWRS) nachgewiesen. Die ESR-Spektren in den Mn(II)-Salzen zeigen sechs Absorptionen bei 14% Neutralisation und eine Absorption bei 62%. Die KWRS deutet auf die Existenz von Ionen-Aggregaten hin, deren Durchmesser ungefähr 27 Å für das Mn(II)-Salz und 19–23 Å für das Ni(II)-Salz bei höherer Neutralisation als 14% ist.
    Notes: Summary The existence of ion-clusters in ethylene-acrylic acid copolymers (mole% of acrylic acid is 9.3) neutralized with manganese(II) and nickel(II) acetates, respectively, was studied by dielectric measurements in the temperature range of −180 to 80 °C at several frequencies between 30 Hz and 100 kHz. Theβ′ relaxation due to a micro-Brownian molecular motion aboveT g and theγ relaxation due to a local molecular motion belowT g were observed. Variation in the magnitude and the relaxation temperature of the two relaxations with degree of neutralization by manganese/nickel acetate suggest the formation of ion-clusters in the higher degrees of neutralization above a value between 3.3 and 14% for the Mn(II) salts and above about 14% for the Ni(II) salts. Furthermore, this formation of ion-clusters was evidenced by electron spin resonance and small angle X-ray scattering studies. The ESR spectra for the Mn(II) salts exhibited six peaks at 14% of degree of neutralization and one peak at 62%. This indicates the existence of ion-clusters at 62%. The small angle X-ray scatterings suggested the existence of ion-clusters of diameter of about 27 Å for the Mn(II) salts and 19–23 A for the Ni(II) salts, at the high degrees of neutralization above 14%.
    Type of Medium: Electronic Resource
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