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  • 1
    Electronic Resource
    Electronic Resource
    Clinical and biochemical consequences of copper-histidine therapy in Menkes disease (1993)
    Springer
    European journal of pediatrics 152 (1993), S. 828-832 
    Details
    ISSN: 1432-1076
    Keywords: Menkes disease ; Copper treatment ; d-Penicillamine ; Dopamine-β-hydroxylase
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Menkes disease (MD) is an X-linked recessively inherited neurodegenerative disorder of copper (Cu) metabolism leading to death in early childhood. Symptoms are attributed to deficient activity of Cu-dependent enzymes. Limited experience has been reported concerning clinical and biochemical consequences of parenteral treatment with copper-(histidine)2-complex (Cu-His) in MD. Cu-His was administered in a 13-week-old boy with MD by daily intramuscular injections. After 6 weeks of therapy, Cu and caeruloplasmin in serum and Cu in CSF were normalized. The excessive dopamine level in CSF was corrected after 3 months of treatment. After 6 weeks of Cu supplementation, complete reduction of epileptic discharges, improved muscular tone and increased motor activities were observed. Developmental regression stopped and was replaced by a slight progression. Death at the age of 19 months was caused by septicaemia due to a fulminant urinary tract infection; there was no evidence of chronic Cu toxicity. These findings suggest that Cu-His supplementation may be a promising palliative treatment in MD.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF02073380
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    Paper (German National Licenses)
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  • 2
    Electronic Resource
    Electronic Resource
    Serum magnesium level and sleep behavior of newborn infants (1980)
    Springer
    European journal of pediatrics 134 (1980), S. 239-243 
    Details
    ISSN: 1432-1076
    Keywords: Serum Mg ; Full-term newborn infants ; Active sleep ; Quiet sleep
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Polygraphic studies were performed in 14 fullterm newborns. The sleep behavior of the infants was correlated to the serum Mg level. Correlations could be demonstrated between serum Mg and active sleep, as well as between serum Mg and quiet sleep. With increasing serum Mg quiet sleep increased, whereas active sleep decreased. After Mg injection quiet sleep increased and active sleep decreased even more. There were also corresponding correlations between serum Mg and rapid eye movements, submental muscle tone, and gross body movements.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00441479
    Library Location Call Number Volume/Issue/Year Availability
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    Paper (German National Licenses)
    Fulltext
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