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ArtikelLung Transplantation in Cystic Fibrosis — Primum Non Nocere?  
Oleh: Allen, Julian ; Visner, Gary
Jenis: Article from Journal - ilmiah internasional
Dalam koleksi: The New England Journal of Medicine (keterangan: ada di Proquest) vol. 357 no. 21 (Nov. 2007), page 2186.
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  • Perpustakaan FK
    • Nomor Panggil: N08.K.2007.06
    • Non-tandon: 1 (dapat dipinjam: 0)
    • Tandon: tidak ada
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Isi artikelCystic fibrosis is one of the most common autosomal recessive diseases. It causes intestinal, pancreatic, and progressive lung dysfunction, which is ultimately lethal. In 1989, the first specific cystic fibrosis gene defect on chromosome 7 was described, and its function was determined shortly thereafter. The cystic fibrosis gene codes for the cystic fibrosis transmembrane regulator (CFTR) protein, a chloride channel that controls chloride and, indirectly, sodium transport across epithelial cells. When CFTR function is lost, excess sodium and chloride are resorbed from the airway lumen, leading to dehydration of the luminal contents and increased fluid viscosity of the airway lining. . . .
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