Literature DB >> 9061568

Decreased platelet membrane anisotropy in patients with adrenoleukodystrophy treated with erucic acid (22:1)-rich triglycerides.

S Stöckler1, C Opper, A Greinacher, D H Hunneman, G C Korenke, C J Unkrig, F Hanefeld.   

Abstract

Low platelet count and bleeding diathesis have been observed in patients with adrenoleukodystrophy (ALD) treated with erucic acid (22:1)-rich triglycerides ("Lorenzo's oil'). To investigate possible alterations of biophysical membrane properties, we measured platelet membrane anisotropy, which is inversely related to membrane fluidity, in 16 patients with and in 3 patients without treatment. In patients on treatment, platelet membrane anisotropy was significantly decreased. Additionally, we found increased platelet concentrations of 22:1 and compromised in vitro platelet aggregation response. The decrease of platelet membrane anisotropy is probably a main cause of bleeding diathesis. Long-term haematological side-effects must be considered in ALD patients treated with Lorenzo's oil.

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Year:  1997        PMID: 9061568     DOI: 10.1023/a:1005309623452

Source DB:  PubMed          Journal:  J Inherit Metab Dis        ISSN: 0141-8955            Impact factor:   4.982


  19 in total

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Authors:  P Sedlmayr; B Plecko; E Paschke; W Zenz; H Ramschak; H Toplak; T C Wascher; M Wilders-Truschnig; S Stöckler
Journal:  J Inherit Metab Dis       Date:  1995       Impact factor: 4.982

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Authors:  C Opper; C Fett; B Capito; S Raha; W Wesemann
Journal:  Thromb Res       Date:  1993-10-01       Impact factor: 3.944

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Authors:  S Zierz; R Schröder; C J Unkrig
Journal:  Clin Investig       Date:  1993-10

9.  Glyceroltrioleate/glyceroltrierucate therapy in 16 patients with X-chromosomal adrenoleukodystrophy/adrenomyeloneuropathy: effect on clinical, biochemical and neurophysiological parameters.

Authors:  G C Korenke; D H Hunneman; J Kohler; S Stöckler; K Landmark; F Hanefeld
Journal:  Eur J Pediatr       Date:  1995-01       Impact factor: 3.183

10.  A two-year trial of oleic and erucic acids ("Lorenzo's oil") as treatment for adrenomyeloneuropathy.

Authors:  P Aubourg; C Adamsbaum; M C Lavallard-Rousseau; F Rocchiccioli; N Cartier; I Jambaqué; C Jakobezak; A Lemaitre; F Boureau; C Wolf
Journal:  N Engl J Med       Date:  1993-09-09       Impact factor: 91.245

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  2 in total

1.  Enhanced lymphocyte proliferation in patients with adrenoleukodystrophy treated with erucic acid (22:1)-rich triglycerides.

Authors:  R B Pour; S Stöckler-Ipsiroglu; D H Hunneman; M Gahr; G C Korenke; W Pabst; F Hanefeld; A Peters
Journal:  J Inherit Metab Dis       Date:  2000-03       Impact factor: 4.982

2.  Effects of smoking and sun-drying on proximate, fatty and amino acids compositions of Southern pink shrimp (Penaeus notialis).

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  2 in total

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