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伴有血小板功能障碍的奥斯勒病中的结膜出血。病例报告。

Conjunctival bleeding in Osler's disease with associated platelet dysfunction. A case report.

作者信息

Pandolfi M, Ehinger B

出版信息

Acta Ophthalmol (Copenh). 1978 Feb;56(1):75-82. doi: 10.1111/j.1755-3768.1978.tb00469.x.

Abstract

A patient with Osler's disease (hereditary haemorrhagic telangiectasia) was admitted to hospital because of obstinate, profuse conjuctival bleeding occurring without any known preceding rrauma. Extensive examination of the haemostatic mechanism revealed an impaired platelet function reflected in defective platelet aggregation by ADP, collagen adrenalin and defective clot retraction. This platelet dysfunction, whose association with conjunctival telangiectasia was hitherto unknown, impaired the patient's already deficient primary haemostasis following the vascular anomaly and apparently contributed to the severity of the bleeding which could only be checked surgically. The findings seem to warrant investigation of the platelet function in patients with Osler's disease. In the event of platelet dysfunction drugs, such as acetylsalicylic acid (aspirin), indomethacin, dextrans as well as transfusions with bank blood are contraindicated.

摘要

一名患有奥斯勒病(遗传性出血性毛细血管扩张症)的患者因在无任何已知先前创伤的情况下发生顽固、大量的结膜出血而入院。对止血机制进行的广泛检查发现血小板功能受损,表现为对二磷酸腺苷、胶原、肾上腺素的血小板聚集缺陷以及血块回缩缺陷。这种血小板功能障碍与结膜毛细血管扩张症的关联此前尚不为人知,它损害了患者因血管异常而本就不足的初级止血功能,显然加剧了出血的严重程度,而出血只能通过手术控制。这些发现似乎值得对奥斯勒病患者的血小板功能进行研究。如果存在血小板功能障碍,诸如乙酰水杨酸(阿司匹林)、吲哚美辛、右旋糖酐等药物以及输注库血都是禁忌的。

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