Cystic fibrosis

@article{Elborn2016CysticF,
  title={Cystic fibrosis},
  author={J. Elborn},
  journal={The Lancet},
  year={2016},
  volume={388},
  pages={2519-2531}
}
  • J. Elborn
  • Published 2016
  • Medicine
  • The Lancet
  • Cystic fibrosis is a common life-limiting autosomal recessive genetic disorder, with highest prevalence in Europe, North America, and Australia. The disease is caused by mutation of a gene that encodes a chloride-conducting transmembrane channel called the cystic fibrosis transmembrane conductance regulator (CFTR), which regulates anion transport and mucociliary clearance in the airways. Functional failure of CFTR results in mucus retention and chronic infection and subsequently in local airway… CONTINUE READING
    73 Citations

    Paper Mentions

    Interventional Clinical Trial
    Cystic Fibrosis (CF) is the most common autosomal recessive lethal disorders affecting 1:2.500 newborns among Caucasians. CF patients are peculiarly susceptible to infection and… Expand
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