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[血友病和血管性血友病-于尔根斯综合征的诊断。3. 血管性血友病-于尔根斯综合征患者、血管性血友病-于尔根斯综合征杂合子患者及Glanzmann-Naegeli血小板无力症患者的研究结果]

[Diagnosis of hemophilia and von Willebrand-Jürgens syndrome. 3. Results of studies on patients with vWJS, heterozygotes of vWJS and patients with Glanzmann-Naegeli thrombasthenia].

作者信息

Lutze G, Schlote A

出版信息

Z Gesamte Inn Med. 1985 Sep 15;40(18):532-9.

PMID:3878049
Abstract

By means of genealogical and laboratory-diagnostic examinations in the district of Magdeburg 59 patients with a von-Willebrand-Jürgens-syndrome and 2 patients with thrombasthenia were detected. On the basis of the laboratory data and the severity of the inclination to haemorrhage in patients with von-Willebrand-Jürgens-syndrome a subdivision into type I (severe from), type I (easy form) and type II was made. The diagnosis was rendered difficult by the appearance of clinically asymptomatic carriers of signs. The experiences hitherto made in the dispensary care and the use of the laboratory programme are estimated.

摘要

通过在马格德堡地区进行的系谱和实验室诊断检查,发现了59例患有冯威勒布兰德-于尔根斯综合征的患者和2例血小板无力症患者。根据实验室数据以及冯威勒布兰德-于尔根斯综合征患者出血倾向的严重程度,将其分为I型(重型)、I型(轻型)和II型。具有体征的临床无症状携带者的出现使诊断变得困难。对迄今为止在门诊护理和实验室检查项目使用方面的经验进行了评估。

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