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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    European journal of pediatrics 116 (1974), S. 95-114 
    ISSN: 1432-1076
    Keywords: Non-ketotic hyperglycinaemia ; Dietetic treatment ; Histologic changes of brain ; Liver
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung Es wird über eine Patientin mit einer nichtketotischen Hyperglycinämie berichtet, die 2 Jahre lang mit einer vollsynthetischen Kost aus einem Aminosäurengemisch, Mineralien, Glucose, Fett und Vitaminzusätzen behandelt wurde. Die diätetische Behandlung sowie Zusatz von Natrium-Benzoat zur Nahrung führten nur kurzfristig zu einem Absinken der Plasma-Glycin-Konzentrationen. Nach Anreicherung der Nahrung mit L-Methionin in einer Dosierung von 300 mg/kg KG/die zeigten sich zwar deutlich niedrigere Glycinspiegel, gleichzeitig kam es aber zu einer exzessiven Hypermethioninämie. L-Methionin-Gabe in Dosierungen von 150–200 mg/kg KG hatten keinen Einfluß auf die Höhe des Plasma-Glycin-Spiegels. Die körperliche Entwicklung des Kindes unter Diätbehandlung war altersentsprechend. Ein Einfluß auf die geistige Entwicklung wurde nicht bemerkt, es bestand aber bereits zu Beginn der Behandlung das Bild einer Decrebrierung. Pathologisch-histologisch zeigten sich Veränderungen des ZNS in Form von Markscheidenreifungshemmung und einer spongiösen Degeneration der weißen Substanz in Großhirn, Kleinhirn und Rückenmark sowie Einlagerungen von doppeltbrechenden Kristallen. In der grauen Substanz waren keine groben Alterationen nachweisbar. Im Bereich des peripheren Nervensystems fanden sich keine Markscheidenausfälle und keine doppeltbrechenden Kristalle. Die Leber zeigte eine leichte Vacuolisierung der Leberzellen und eine starke Einlagerung von doppeltbrechenden Kristallen in Parenchymzellen und Makrophagen.
    Notes: Abstract A girl with non-ketotic hyperglycinaemia was treated for 2 years with a synthetic diet consisting of a glycine- and serine-free amino-acid mixture, glucose, fat, minerals, and vitamins. The addition of sodium benzoate to this diet resulted in only a temporary decrease of the plasma glycine concentration. Addition of 300 mg methionine/kg/day led to a marked reduction of glycine in the plasma but also to an excessive increase of the methionine plasma concentration. L-methionine at a lower dose did not influence the plasma glycine level. During the dietary treatment an almost normal gain of body weight was observed, though the psychomotor development was not influenced. Post mortem the following changes were noted: 1. reduced myelination of the white matter; 2. spongiform alterations of the white matter of the cerebrum, the cerebellum, and the spinal cord; 3. inclusions of birefringent crystals in liver and brain. The grey matter of the cerebrum did not show any marked alterations. In peripheral nerves no areas of demyelination or inclusions of crystals were found.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-1076
    Keywords: Key words Protein quality ; Dietetic treatment ; Phenylketonuria
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The protein quality of the diets of phenylketonuria (PKU) children of different ages (3 months, 10 months, 3 years, 8 years, 12 years, 16 years) with low or high phenylalanine (Phe) tolerance was assessed according to the recommendations of the FAO/WHO consultation group [13]. The amount of each essential amino acid (AA) per gram dietary protein was calculated and compared to the reference. The resultant amino acid score (AAS) indicated a limited to inadequate biological protein quality of the diets in 3-month-old infants (2.2 g protein/kg body weight/day) and 10-month-old infants (2.0 g protein/kg body weight/day) with a “high” Phe tolerance. In all other age groups the AAS was 〉 100%. However remarkable imbalances in the AA pattern were apparent. Beginning with the age of 3 years (1.7 g protein/kg body weight/day) the intake of the AA lysine and isoleucine was three or two times higher than recommended. At the age of 8 years (1.4 g protein/kg body weight/day) the intake of three AA (valine, isoleucine, lysine) was – related to the WHO recommendations – 217%, 229% and 291%. Similar results could be found in the age groups of 12 years (1.1 g protein/kg body weight/day) and 16 years (0.9 g protein/kg body weight/day), respectively. These calculations might help to reconsider the composition of the AA mixtures used in the dietetic treatment of PKU patients.
    Type of Medium: Electronic Resource
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