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[智利第九大区的血管性血友病]

[Von Willebrand's disease in the IX Region of Chile].

作者信息

Cabrera M E, Artigas C G, Páez E, Monsalve V, Zolezzi P, Arauco G, Espinoza R, Hevia C, Villegas J

出版信息

Rev Med Chil. 1989 Apr;117(4):423-30.

PMID:2519397
Abstract

Bleeding disorders are frequent indications for reference to Hematologic Clinics. Won Willebrand's disease (vWD) is one of the commonest of inheritable hemorrhagic diathesis. In order to evaluate the frequency and clinical characteristics of vWD in the IX Region of Chile, we performed a clinical and laboratory investigation in 132 patients, children and adults, over a period of 3 years: 1983-1985. Within this group there was 26 close relatives. The laboratory tests used for the diagnosis were: bleeding time, factor VIII, von Willebrand factor and ristocetin cofactor. Diagnosis of vWD was made in 83 cases, most of them presenting the mild form of the disease. Two children had the severe form. The estimated prevalence of vWD in symptomatic patients in the IX Region of Chile is at least 113 per million, which makes it 3 or 4 times more frequent than hemophilia. vWD is observed at a much lower rate in mapuche than in caucasian population. The symptoms and their frequency were: post partum bleeding 75%, dental extraction bleeding 65%, surgical bleeding 65%, epistaxis 63%, menorrhagia 54%, easy bruising 45%, deep hematomas 8% and petechiae 5%. Five cases were asymptomatic. The bleeding time was the test most frequently abnormal: 72/83 cases (87%).

摘要

出血性疾病是血液科门诊常见的就诊指征。血管性血友病(vWD)是最常见的遗传性出血性疾病之一。为评估智利第九大区vWD的发病率及临床特征,我们在1983年至1985年的3年间,对132例儿童及成人患者进行了临床和实验室调查。该组中有26名近亲。用于诊断的实验室检查包括:出血时间、凝血因子VIII、血管性血友病因子和瑞斯托霉素辅因子。83例患者确诊为vWD,其中大多数为疾病的轻型。两名儿童为重型。智利第九大区有症状患者中vWD的估计患病率至少为百万分之113,这使其发病率比血友病高3至4倍。在马普切人中观察到的vWD发病率远低于高加索人群。症状及其出现频率如下:产后出血75%、拔牙后出血65%、手术出血65%、鼻出血63%、月经过多54%、易瘀斑45%、深部血肿8%、瘀点5%。5例患者无症状。出血时间是最常出现异常的检查:83例中有72例(87%)异常。

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