Origins of Cystic Fibrosis Lung Disease  

Oleh:

Stoltz, David A. ; Meyerholz, David K. ; Welsh, Michael J.

Jenis: Article from Journal - ilmiah internasional
Dalam koleksi: The New England Journal of Medicine (keterangan: ada di Proquest) vol. 372 no. 04 (Jan. 2015), page 351-362.

Ketersediaan

Perpustakaan FK Nomor Panggil: N08.K Non-tandon: 1 (dapat dipinjam: 0) Tandon: tidak ada

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Isi artikel

At the basic level, we know the genetic cause of cystic fibrosis: it is an autosomal recessive disease caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR).1,2 At the clinical level, we know that chronic bacterial airway infection, prominent neutrophilic inflammation and mucus in airways, and progressive bronchiectasis characterize advanced cystic fibrosis lung disease, which causes most morbidity and death in people with cystic fibrosis.2 Between those two extremes, the way in which loss of CFTR-mediated chloride and bicarbonate transport leads to chronic airway infection has remained uncertain.

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