囊性纤维性变(Cystic fibrosis,CF)是最常见的致死性遗传性疾病。本病以累及外分泌腺为特点,故常表现为支气管与肺、胰腺和汗腺等功能异常。其中又以肺部病变最为常见,也最为严重。本病虽多见于婴幼儿,但近年来随着诊治方法的改善,延长了CF患者的存活时间,青年和成年CF患者逐年增多。【发病机理】迄今尚未清楚,据认为与下列因素有关。 1.遗传学特点(2,6,10):CF系一常染色体隐性遗传性疾病。当父母均为CF基因 Cystic fibrosis (CF) is the most common fatal genetic disease. The disease is characterized by the involvement of exocrine glands, often manifested as bronchial and lung, pancreas and sweat glands and other dysfunction. One of the most common lung disease, but also the most serious. Although the disease is more common in infants and young children, but with the improvement of diagnosis and treatment methods in recent years, extending the survival time of CF patients, young and adult CF patients increased year by year. 【Pathogenesis】 So far not yet clear, it is considered with the following factors. 1. Genetics (2,6,10): CF Department of an autosomal recessive disease. When both parents are CF genes