| Literature DB >> 9061568 |
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.Entities:
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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