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[加德纳综合征:临床与流行病学最新进展]

[Gardner syndrome: clinical and epidemiologic up to date].

作者信息

Losacco T, Punzo C, Santacroce L

机构信息

D.O.C., Cattedra di Chirurgia Generale, Università degli Studi di Bari, Italia.

出版信息

Clin Ter. 2005 Nov-Dec;156(6):267-71.

Abstract

In 1950 EJ Gardner first described a new syndrome characterized by (1) familial colonic polyposis, (2) multiple osteomas, (3) soft tissues cysts and (4) fibrous lesions. Thereafter, in 1975 Watne and coll. have demonstrated the occurence, in patients affected by Gardner syndrome, of the early onset of osteomas and dental inclusions in maxillary bones. Gardner syndrome is actually considered a severe life treathening condition due to the poor quality of life and the evolutive pattern of colonic polyps to colon cancer in 100% of cases. The aim of this paper is the review of the pathophysiologic and clinical aspects of Gardner syndrome, with report of institutional clinical data about epidemiology and clinical presentation of such condition, attempting to elaborate a clinical protocol for early detection of that.

摘要

1950年,EJ·加德纳首次描述了一种新综合征,其特征为:(1)家族性结肠息肉病,(2)多发性骨瘤,(3)软组织囊肿,(4)纤维性病变。此后,1975年瓦特内及其同事证明,在患有加德纳综合征的患者中,上颌骨骨瘤和牙内陷会早期出现。由于生活质量差以及结肠息肉在100%的病例中会演变成结肠癌,加德纳综合征实际上被认为是一种严重危及生命的疾病。本文旨在综述加德纳综合征的病理生理和临床方面,报告有关该疾病流行病学和临床表现的机构临床数据,试图制定一种早期检测的临床方案。

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