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[遗传性出血性毛细血管扩张症。十例病例报告]

[Rendu-Osler-Weber disease. Contribution of ten cases].

作者信息

Rodríguez Cuartero A, González Martínez F, Rodríguez Cuartero F, Ceballos Torres A

机构信息

Departamento de Medicina Interna, Hospital Clínico San Cecilio, Facultad de Medicina, Universidad de Granada.

出版信息

An Med Interna. 1992 Mar;9(3):131-3.

PMID:1567950
Abstract

Ten new cases of hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber's disease) are presented. Five of them were detected among 30,000 patients attending a Service of Internal Medicine; the remainder five, were detected while conducting the family study. Epistaxis (5 cases) and hidden (2 cases) and evident (1 case) digestive hemorrhage were the most frequent clinical signs. All patients presented telangiectasia affecting lips. We observed edemas in nasal fossas (5 cases), hand fingers (6 cases), tongue (2 cases) and face (1 case), as well as in gastroduodenal mucosa in the three patients undergoing endoscopic study. We did not observed hemostasis disorders, except for moderate thrombocytosis in two patients and microcytic anemia in three patients. The relevance of clinical evaluation is stressed, because all patients presented oral-facial telangiectasia.

摘要

本文报告了10例遗传性出血性毛细血管扩张症(即Rendu-Osler-Weber病)的新病例。其中5例是在30000名内科门诊患者中发现的;其余5例是在进行家族研究时发现的。鼻出血(5例)以及隐匿性(2例)和显性(1例)消化道出血是最常见的临床症状。所有患者均出现累及唇部的毛细血管扩张。我们观察到鼻窝(5例)、手指(6例)、舌部(2例)和面部(1例)出现水肿,在内镜检查的3例患者的胃十二指肠黏膜中也观察到水肿。除2例患者有中度血小板增多症和3例患者有小细胞贫血外,未观察到止血障碍。强调了临床评估的重要性,因为所有患者均出现口腔面部毛细血管扩张。

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