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[蓝色橡皮疱痣综合征。关于2例病例的诊断与处理]

[Blue rubber bleb naevus syndrome. Diagnosis and management apropos of 2 cases].

作者信息

Pinlong E, Frydman E, Dieckmann K, Faure N, Rolland J C, Lorette G, Ployet M J

机构信息

Service ORL, CHRU Clocheville, Tours.

出版信息

Ann Otolaryngol Chir Cervicofac. 1994;111(4):228-31.

PMID:7726481
Abstract

Blue Rubber Bleb Naevus Syndrome is a rare pathology: 70 cases have been noted since the first description, by Gascoyen, in 1860. It is an acquired disease which usually appears before the age of ten. It is marked by angiomatic cutaneo-digestive tumours combined with chronic anaemia and iron deficiency. Its many and various complications, particularly cataclysmic haemorrhages, car jeopardize the vital prognosis throughout. This little known syndrome is rarely diagnosed at the time of its first manifestations. We recall, from two cases and a review of their literature, the clinical characteristics of the syndrome, its complications and its essentially symptomatic treatment. We therefore suggest an approach to its diagnosis.

摘要

蓝色橡皮疱痣综合征是一种罕见的病症

自1860年加斯库安首次描述以来,已记录到70例。它是一种后天性疾病,通常在10岁之前出现。其特征为皮肤和消化道血管性肿瘤,伴有慢性贫血和缺铁。它有许多各种各样的并发症,尤其是灾难性出血,可能会危及整个生命预后。这种鲜为人知的综合征在首次出现症状时很少被诊断出来。我们通过两个病例及其文献回顾,回忆了该综合征的临床特征、并发症及其主要的对症治疗方法。因此,我们提出了一种诊断方法。

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