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  • 1
    Electronic Resource
    Electronic Resource
    Decreased vigilance and neurotransmitter synthesis after discontinuation of dietary treatment for phenylketonuria in adolescents (1985)
    Springer
    European journal of pediatrics 144 (1985), S. 17-20 
    Details
    ISSN: 1432-1076
    Keywords: Attention ; Serotonin ; Dopamine ; PKU
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Four adolescent or young adult patients with phenylketonuria were examined before and after discontinuation of dietary treatment. Plasma and CSF phenylalanine concentrations increased about two-fold in three patients. In these patients the CSF concentration of the dopamine and serotonin metabolites homovanillic acid (HVA) and 5-hydroxyindoleacetic acid (5-HIAA) decreased markedly; 5-HIAA to extremely low values. The reaction time variability increased in these patients. In the fourth case plasma phenylalanine levels, CSF HVA and 5-HIAA levels, and reaction time variability were essentially unchanged. The relationship between reaction time variability and the CSF 5-HIAA level for all four patients could be presented as a linear function. However, a causal relationship is still unproven. These preliminary findings demonstrate that there may be hazards in the discontinuation of dietary treatment, even in adolescents or young adults, for neurotransmitter metabolism and mental function.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00491918
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    Paper (German National Licenses)
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  • 2
    Electronic Resource
    Electronic Resource
    Increased neurotransmitter biosynthesis in phenylketonuria induced by phenylalanine restriction or by supplementation of unrestricted diet with large amounts of tyrosine (1988)
    Springer
    European journal of pediatrics 148 (1988), S. 238-245 
    Details
    ISSN: 1432-1076
    Keywords: Attention ; Dopamine ; PKU ; Serotonin ; Tyrosine
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Seven phenylketonuria (PKU) patients aged 15–24 years were allowed unrestricted diet for 3 weeks. Three of these patients performed well on unrestricted diet according to visual reaction time variability (RTv 50–100 ms) and did not show significant changes when returning to the phenylalanine-restricted diet (RTv 70–100 ms). Neither did the concentrations of homovanillic acid (HVA) and 5-hydroxyindoleacetic acid (5-HIAA) in cerebrospinal fluid (CSF) change significantly. Four of the patients, however, performed rather poorly (RTv 120–220 ms) on unrestricted diet and improved significantly (P〈0.03) when the diet was restored (RTv 70–150 ms). The improvements were accompanied by significant (P〈0.01 and P〈0.02) increases (mean 52% and 109%) in CSF levels of HVA and 5-HIAA. Five PKU patients aged 15–23 years were allowed unrestricted diet or unrestricted diet supplemented with various amounts of tyrosine (106–194 mg/kg per 24 h). Two of these patients performed very well on unrestricted diet (RTv 60 ms) and showed little change when the unrestricted diet was supplemented with tyrosine (RTv 70 ms and 80 ms). The three other patients, who performed rather poorly (RTv 120–220 ms), improved significantly (P〈0.03) when the unrestricted diet was supplemented with tyrosine (RTv 70–140 ms). HVA in CSF increased significantly (P〈0.01) with the tyrosine supplement when the amount exceeded a threshold of approximately 80 mg/kg per 24 h. The simultaneous increase in CSF level of 5-HIAA showed a positive correlation (r=0.90; P〈0.02) with the increase in HVA concentration suggesting a functional interrelation between the dopaminergic and the serotoninergic nervous systems.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00441411
    Library Location Call Number Volume/Issue/Year Availability
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    Paper (German National Licenses)
    Fulltext
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