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Activation of platelet function in Fabry's disease
Author(s) -
Igarashi Takashi,
Sakuraba Hitoshi,
Suzuki Yoshiyuki
Publication year - 1986
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.2830220110
Subject(s) - medicine , pathogenesis , platelet , platelet activation , platelet factor 4 , disease , fabry's disease , ceramide , cardiology , fabry disease , endocrinology , chemistry , biochemistry , apoptosis
Increased platelet aggregation and high plasma concentration of β‐thromboglobulin were observed in hemizygotes and heterozygotes of Fabry's disease. Carbamazepine and phenytoin administered for the treatment of pains in these patients showed no significant effect on platelet aggregation. No activation of platelets was observed after the addition of ceramide trihexoside, the storage lipid of this disease. Mitral valve prolapse was found in eight of 12 patients. Although the pathogenesis of platelet activation and mitral valve prolapse are not known, the platelet activation could be an early indicator and an accelerating factor of thromboembolic vascular change in this disease.