作者
Tiina Kahre
发表日期
2004
出版商
Tartu University Press
简介
Cystic fibrosis (CF) is the most common life-shortening autosomal recessively inherited disorder among Caucasians (1 in 2500), but is found in all racial and ethnic groups (Welsh et al., 1995). The first comprehensive description of the symptoms of cystic fibrosis was provided in 1938 by Dorothy Andersen who called it “cystic fibrosis of the pancreas”(Andersen, 1938). Cloning of the cystic fibrosis transmembrane conductance regulator (CFTR) gene in 1989 (Rommens et al., 1989; Riordan et al., 1989) significantly advanced our understanding of the biology and pathophysiology of the CF cell thus enabling more precise genetic testing and novel treatment. CF is still an important medical problem. Although the life span of CF patients has increased significantly, from around 5 years in the 1950s (Davis et al., 1996) to about 31 years in 2002 (Cystic Fibrosis Foundation, 2003), due to its cost CF still has a major impact on …
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