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Cystic fibrosis

Ratjen, Felix, Döring, Gerd·2003·The Lancet
DOI 10.1016/s0140-6736(03)12567-6
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Abstract

Cystic fibrosis is the most common autosomal recessive disorder in white people, with a frequency of about 1 in 2500 livebirths. Discovery of the mutated gene encoding a defective chloride channel in epithelial cells--named cystic fibrosis transmembrane conductance regulator (CFTR)--has improved our understanding of the disorder's pathophysiology and has aided diagnosis, but has shown the disease's complexity. Gene replacement therapy is still far from being used in patients with cystic fibrosis, mostly because of difficulties of targeting the appropriate cells. Life expectancy of patients with the disorder has been greatly increased over past decades because of better notions of symptomatic treatment strategies. Here, we summarise advances in understanding and treatment of cystic fibrosis, focusing on pulmonary disease, which accounts for most morbidity and deaths.

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Metadata

Authors
Ratjen, F., & Döring, G.
Venue
The Lancet
Publisher
Elsevier BV
Published
2003-02
Volume
361
Issue
9358
Pages
681-689
ISSN
0140-6736
DOI
10.1016/s0140-6736(03)12567-6
Cited by
885
References
0
Language
en
Licence
www.elsevier.com/tdm/userlicense/1.0/ (tdm)

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