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囊性纤维化:巴西耳鼻喉科经验

Cystic Fibrosis: Brazilian ENT Experience.

作者信息

Sih Tania, Godinho Ricardo, Franco Leticia Paiva, Piltcher Otávio

机构信息

Faculty of Medicine, Laboratório de Investigações Médicas (LIM), Number 40, Universidade de São Paulo, São Paulo, Rua Mato Grosso, 306/1511, 01239-040 São Paulo, SP, Brazil.

出版信息

Int J Otolaryngol. 2012;2012:204696. doi: 10.1155/2012/204696. Epub 2012 May 6.

Abstract

Most published studies about Cystic Fibrosis (CF) are European or North American. There are still few publications about the characteristics of fibrocystic populations in developing countries. The incidence of cystic fibrosis (CF) in Brazil varies among different regions (1 : 10,000 in Minas Gerais, 1 : 9,500 in Paraná, 1 : 8,700 in Santa Catarina, and 1 : 1600 in Rio Grande do Sul). The prevalence of the DF508 mutation also varies according to population: 33% in Sao Paulo, 49% in Rio Grande do Sul, 27% in Santa Catarina, and 52% in Minas Gerais. Cough and nasal obstruction are the most common symptoms. The variation in nasal polyposis prevalence may be explained by population genotypic characteristics in a country that spans a continent. Findings on nasal endoscopy and computed tomography (CT) have better correlation than do this information compared with surgical and clinical history. Microbiologic studies suggest a high level of early contamination of the airways. Sensorineural hearing loss (SNHL) occurs in these patients as a result of ototoxic antibiotics. The data compiled in this paper is useful, but also lead to the general agreement that more research would be welcome due to the unique characteristics of this country.

摘要

大多数已发表的关于囊性纤维化(CF)的研究来自欧洲或北美。关于发展中国家纤维囊性人群特征的出版物仍然很少。巴西不同地区的囊性纤维化(CF)发病率各不相同(米纳斯吉拉斯州为1:10000,巴拉那州为1:9500,圣卡塔琳娜州为1:8700,南里奥格兰德州为1:1600)。DF508突变的患病率也因人群而异:圣保罗为33%,南里奥格兰德州为49%,圣卡塔琳娜州为27%,米纳斯吉拉斯州为52%。咳嗽和鼻塞是最常见的症状。在一个跨越大陆的国家,鼻息肉患病率的差异可能由人群基因型特征来解释。与手术和临床病史相比,鼻内镜检查和计算机断层扫描(CT)的结果具有更好的相关性。微生物学研究表明气道早期污染程度较高。这些患者因耳毒性抗生素而出现感音神经性听力损失(SNHL)。本文汇编的数据很有用,但也导致人们普遍认为,鉴于该国的独特特征,欢迎开展更多研究。

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