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  • 1
    Electronic Resource
    Electronic Resource
    Glyceroltrioleate/glyceroltrierucate therapy in 16 patients with X-chromosomal adrenoleukodystrophy/ adrenomyeloneuropathy: effect on clinical, biochemical and neurophysiological parameters (1994)
    Springer
    European journal of pediatrics 154 (1994), S. 64-70 
    Details
    ISSN: 1432-1076
    Keywords: Key words     X-chromosomal adrenoleukodystrophy ; Adrenomyeloneuropathy ; Very long-chain fatty acids ; Therapy ; Peroxisomal disorder
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract      We have investigated the effect of glyceroltrioleate/glyceroltrierucate (GTO/GTE) therapy on X-chromosomal adrenoleukodystrophy in 16 patients with adrenoleukodystrophy (n = 6), adrenomyeloneuropathy (n = 3), Addison disease without neurological involvement (n = 2), and neurologically and endocrinologically asymptomatic patients (n = 5). Therapy was carried out for 19.4 ± 10 months. All patients showed a normalization of C 26:0 plasma fatty acid concentrations. None of the seven neurologically asymptomatic patients developed neurological symptoms. Somatosensory evoked potentials of the tibialis nerve was the most sensitive electrophysiological parameter, showing a slight improvement in neurologically asymptomatic patients during therapy. In none of the patients with normal cranial MRI at start of therapy (n = 6) has MRI deterioration been observed whilst on therapy. Follow up of the neurologically asymptomatic children supports the hypothesis that GTO/GTE therapy might prevent the development of neurological symptoms. Six of the nine neurologically symptomatic patients deteriorated to varying degrees whilst on therapy. MRI alterations have worsened in all patients with clinical deterioration. Conclusion     GTO/GTE treatment should be initiated in all neurological asymptomatic boys before first neurological symptoms develop. To discover these patients very long-chain fatty acid determination should be performed in all family members at risk when adrenoleukodystrophy or adrenomyeloneuropathy is diagnosed.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01972976
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    Paper (German National Licenses)
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  • 2
    Electronic Resource
    Electronic Resource
    Glyceroltrioleate/glyceroltrierucate therapy in 16 patients with X-chromosomal adrenoleukodystrophy/adrenomyeloneuropathy: Effect on clinical, biochemical and neurophysiological parameters (1995)
    Springer
    European journal of pediatrics 154 (1995), S. 64-70 
    Details
    ISSN: 1432-1076
    Keywords: X-chromosomal adrenoleukodystrophy ; Adrenomyeloneuropathy ; Very long-chain fatty acids Therapy ; Peroxisomal disorder
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We have investigated the effect of glyceroltrioleate/glyceroltrierucate (GTO/GTE) therapy on X-chromosomal adrenoleukodystrophy in 16 patients with adrenoleukodystrophy (n=6), adrenomyeloneuropathy (n=3), Addison disease without neurological involvement (n=2), and neurologically and endocrinologically asymptomatic patients (n=5). Therapy was carried out for 19.4±10 months. All patients showed a normalization of C 26:0 plasma fatty acid concentrations. None of the seven neurologically asymptomatic patients developed neurological symptoms. Somatosensory evoked potentials of the tibialis nerve was the most sensitive electrophysiological parameter, showing a slight improvement in neurologically asymptomatic patients during therapy. In none of the patients with normal cranial MRI at start of therapy (n=6) has MRI deterioration been observed whilst on therapy. Follow up of the neurologically asymptomatic children supports the hypothesis that GTO/GTE therapy might prevent the development of neurological symptoms. Six of the nine neurologically symptomatic patients deteriorated to varying degrees whilst on therapy. MRI alterations have worsened in all patients with clinical deterioration. Conclusion GTO/GTE treatment should be initiated in all neurological asymptomatic boys before first neurological symptoms develop. To discover these patients very long-chain fatty acid determination should be performed in all family members at risk when adrenoleukodystrophy or adrenomyeloneuropathy is diagnosed.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF01972976
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
    Paper (German National Licenses)
    Fulltext
  • 3
    Electronic Resource
    Electronic Resource
    The value of determination of homovanillic and vanillylmandelic acids in plasma for the diagnosis and follow-up of neuroblastoma in children (1987)
    Springer
    European journal of pediatrics 146 (1987), S. 489-493 
    Details
    ISSN: 1432-1076
    Keywords: Neuroblastoma ; Catecholamine metabolites ; Homovanillic acid ; Vanillylmandelic acid
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The plasma levels of homovanillic and vanillylmandelic acids were determined in 84 children with neuroblastoma prior to treatment to evaluate the usefulness of these parameters for the diagnosis of this disease. Elevated levels of one or both of these metabolites were observed in 50% of children with Stage I or II disease, in 88% of those with Stage III, in 93% of those with Stage IV and in 100% of children with Stage IV-S for a total of 88% of all patients. The clinical course of the disease can be followed readily by determination of these metabolites, successful therapy or surgery being accompanied by a corresponding decrease in the plasma levels and relapse by a corresponding increase. Determination of homovanillic and vanillylmandelic acids in plasma can obviate many of the problems associated with the collection of urine, especially in young children or ambulant patients, and thus facilitates a more frequent control with consequent advantages for the management of this disease.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00441600
    Library Location Call Number Volume/Issue/Year Availability
    BibTip Others were also interested in ...
    Paper (German National Licenses)
    Fulltext
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