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血小板无力症。来自印度南部的42例病例回顾与报告。

Glanzmann's thrombasthenia. A review and report of 42 cases from South India.

作者信息

Khanduri U, Pulimood R, Sudarsanam A, Carman R H, Jadhav M, Pereira S

出版信息

Thromb Haemost. 1981 Dec 23;46(4):717-21.

PMID:7330822
Abstract

In the 14 year period from June, 1966 to June, 1980, 42 cases of Glanzmann's thrombasthenia have been diagnosed, all fulfilling the criteria of prolonged bleeding time, with normal venous platelet count, defective clot retraction and decreased platelet aggregation, associated with a lifelong bleeding tendency. Few cases have been reported from India though it is the fourth most common congenital bleeding disorder among the patients seen at the Christian Medical College Hospital, Vellore. The large number of such cases found in South India as compared with reports from other parts of the world may be due to the high degree of consanguinity which is part of the accepted culture in this area. Reliable diagnosis of this condition is possible with fairly simple laboratory procedures.

摘要

在1966年6月至1980年6月的14年期间,共诊断出42例Glanzmann血小板无力症患者,所有患者均符合出血时间延长、静脉血小板计数正常、血块回缩缺陷和血小板聚集减少的标准,并伴有终生出血倾向。尽管Glanzmann血小板无力症是韦洛尔基督教医学院医院所见患者中第四常见的先天性出血性疾病,但印度报道的病例较少。与世界其他地区的报告相比,在南印度发现的此类病例数量较多,可能是由于该地区普遍接受的文化中近亲结婚程度较高。通过相当简单的实验室程序就可以对这种疾病进行可靠的诊断。

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