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布加综合征的血小板超微结构研究。

Platelet ultra-structure study in Budd-Chiari syndrome.

作者信息

Dayal S, Pati H P, Pande G K, Sharma P, Saraya A K

机构信息

Department of Hematology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India.

出版信息

Eur J Haematol. 1995 Nov;55(5):294-301. doi: 10.1111/j.1600-0609.1995.tb00700.x.

Abstract

Morphologic characteristics of circulating platelets were studied in 20 patients with primary Budd-Chiari syndrome (BCS) without any known etiology, using transmission electron microscopy (TEM). Significant platelet ultrastructural changes were observed in all the patients (in 10-->90% platelets) as compared to 20 normal healthy controls (in up to 4% of their platelets). The prominent changes in the platelets were paucity or absence of alpha granules, hypertrophy of the open canalicular systems (OCS) and clumping and fusion of the granules and other organelles in the centre of platelets. Some other changes observed in platelets were dilated channels of OCS, pseudopodial protrusion of cytoplasm and presence of prominent masses of glycogen particles. Platelets from 20 normal controls processed along with the patients' platelets showed only a few such abnormalities. Most of these changes observed in patients' platelets were akin to the changes observed in platelets undergoing activation. Assay of plasma beta-thromboglobulin showed significantly higher levels in all the patients (p < 0.001) further confirming on-going in vivo platelet activation with morphologic changes most likely reflecting the thrombotic process present in BCS patients.

摘要

运用透射电子显微镜(TEM),对20例病因不明的原发性布加综合征(BCS)患者循环血小板的形态学特征进行了研究。与20名正常健康对照者(其血小板中此类情况发生率最高为4%)相比,所有患者均观察到显著的血小板超微结构变化(10% - 90%的血小板出现变化)。血小板的显著变化包括α颗粒缺乏或数量稀少、开放管道系统(OCS)肥大以及血小板中央颗粒与其他细胞器的聚集和融合。在血小板中观察到的其他一些变化包括OCS通道扩张、细胞质伪足样突出以及大量糖原颗粒的存在。与患者血小板一同处理的20名正常对照者的血小板仅显示出少数此类异常。在患者血小板中观察到的这些变化大多类似于活化血小板中观察到的变化。血浆β - 血小板球蛋白检测显示,所有患者的水平均显著升高(p < 0.001),进一步证实了体内血小板持续活化,形态学变化很可能反映了BCS患者存在的血栓形成过程。

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