多发性外生性疾病:三个塞内加尔家庭的研究

N. Moustapha, Kane Baïdy Sy, Salissou Garba Mahaman, Sarr Lamine, D. Coumba, Akpo L. Geraud, D. A. Badara, Diallo Saidou, Ndongo Souhaibou, Pouye Abdoulaye
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引用次数: 1

摘要

外生瘤是一种良性肿瘤,对应于分化良好的骨赘,在生长过程中由生发软骨帽产生[1,2]。它通常是散发性的,但它也可以是常染色体显性的多发性外生疾病的一部分,称为贝塞尔-哈根病[1]。外生瘤,尤其是单发外生瘤,是最常见的良性肿瘤(占良性骨肿瘤的20% ~ 50%,占所有骨肿瘤的10% ~ 15%)。多发性外生骨病是一种罕见的疾病,由Boyer于1814年首次描述[3-5]。它主要在西方文献中报道,其患病率估计为1/50,000[3,5],在北非的程度较小[6,7]。这种流行率在非洲人口中尚未确定。然而,已经在北非和撒哈拉以南非洲开展了有关该病的工作[3-12]。评估该病家族型的研究主要在西方文献中进行[12-18]。在撒哈拉以南非洲,报告的病例显然是零星的。本研究的目的是研究家族型多发性外生骨病的流行病学、诊断和治疗方面。
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Multiple Exostosis Disease: Study of Three Senegalese Families
Exostosis is a benign tumour corresponding to a well-differentiated bone excrescence, produced by a germinal cartilage cap during growth [1,2]. It is most often sporadic, but it can also be part of an autosomal dominant, multiple exostosis disease known as Bessel-Hagen disease [1]. Exostosis, especially in its solitary form, represents the most frequent benign tumour (20 to 50% of benign bone tumours and 10 to 15% of all bone tumours) [1]. The multiple exostosis disease is a rare disease first described by Boyer in 1814 [3-5]. It is mainly reported in Western literature where its prevalence is estimated at 1/50,000 [3,5] and, to a lesser extent, in North Africa [6,7]. This prevalence has not been determined in the African population. However, work has been carried out on the disease in North Africa and sub-Saharan Africa [3-12]. Studies evaluating family forms of the disease were mainly carried out in the Western literature [12-18]. In sub-Saharan Africa, reported cases are apparently sporadic. The objective of this work was to study the epidemiological, diagnostic and therapeutic aspects of familial forms of multiple exostosis disease.
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