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Titolo:
DECREASED PLATELET MEMBRANE ANISOTROPY IN PATIENTS WITH ADRENOLEUKODYSTROPHY TREATED WITH ERUCIC-ACID (22 1)-RICH TRIGLYCERIDES/
Autore:
STOCKLER S; OPPER C; GREINACHER A; HUNNEMAN DH; KORENKE GC; UNKRIG CJ; HANEFELD F;
Indirizzi:
UNIV VIENNA,CHILDRENS HOSP,WAEHRINGER GUERTEL 18-20 A-1090 VIENNA AUSTRIA UNIV GOTTINGEN,CHILDRENS HOSP,DEPT NEUROPEDIAT D-3400 GOTTINGEN GERMANY UNIV MARBURG,DEPT NEUROCHEM,INST PHYSIOL CHEM D-35032 MARBURG GERMANY UNIV GIESSEN,INST CLIN IMMUNOL & TRANSFUS MED D-35390 GIESSEN GERMANY UNIV BONN,INST EXPT HEMATOL & TRANSFUS MED D-5300 BONN GERMANY
Titolo Testata:
Journal of inherited metabolic disease
fascicolo: 1, volume: 20, anno: 1997,
pagine: 54 - 58
SICI:
0141-8955(1997)20:1<54:DPMAIP>2.0.ZU;2-O
Fonte:
ISI
Lingua:
ENG
Soggetto:
X-LINKED ADRENOLEUKODYSTROPHY; LORENZOS OIL; THERAPY; ADRENOMYELONEUROPATHY; THROMBOCYTOPENIA; FLUIDITY;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Science Citation Index Expanded
Citazioni:
22
Recensione:
Indirizzi per estratti:
Citazione:
S. Stockler et al., "DECREASED PLATELET MEMBRANE ANISOTROPY IN PATIENTS WITH ADRENOLEUKODYSTROPHY TREATED WITH ERUCIC-ACID (22 1)-RICH TRIGLYCERIDES/", Journal of inherited metabolic disease, 20(1), 1997, pp. 54-58

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 compromisedin 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.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 01/10/20 alle ore 03:36:19