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[囊性纤维化及其相关感染的病理生理学]

[Physiopathology of cystic fibrosis and of infections associated to this disease].

作者信息

Aramă Stefan Sorin, Aramă Victoria, Cercel Adrian Streinu, Ion Daniela Adriana, Hristea Adriana, Sotcan Mihai

机构信息

Catedra de Patologie Generală şi Oromaxilofacială, Facultatea de Medicină Dentară, UMF Carol Davila Bucureşti.

出版信息

Pneumologia. 2007 Oct-Dec;56(4):202-7.

PMID:18320796
Abstract

Cystic fibrosis (CF) is one of the most common lethal inherited diseases in the caucasian population. The hope of understanding the physiopathology of CF appeared after identification of the gene which encodes CF. The alteration in CFTR protein breaks the hydric balance of the mucosal secretions, producing mucus with increased viscosity. Broncho-pulmonary infections are constant encountered because of the stasis of the mucus. The most common bacterial pathogens in the sputum of patients with MV are Haemophilus influenzae, Staphylococcus aureus, Pseudomonas aeruginosa, Burkholderia cepacia. MV remains a severe, incurable disease, but the evolution is getting better because of the improvements of care methods.

摘要

囊性纤维化(CF)是白种人群中最常见的致死性遗传病之一。在编码CF的基因被鉴定出来后,人们看到了理解CF生理病理学的希望。CFTR蛋白的改变破坏了粘膜分泌物的水平衡,产生了粘度增加的黏液。由于黏液的淤滞,支气管肺部感染经常发生。机械通气患者痰液中最常见的细菌病原体是流感嗜血杆菌、金黄色葡萄球菌、铜绿假单胞菌、洋葱伯克霍尔德菌。机械通气仍然是一种严重的、无法治愈的疾病,但由于护理方法的改进,病情发展正在好转。

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