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Previous Volume 357:2186-2188 November 22, 2007 Number 21 Next

	Since this article has no abstract, we have provided an extract of the first 100 words of the full text and any section headings.

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Cystic 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. . . . [Full Text of this Article]

Source Information

From the Division of Pulmonary Medicine and Cystic Fibrosis Center, Children's Hospital of Philadelphia, Philadelphia (J.A.); and the Division of Respiratory Diseases, Children's Hospital, Boston (G.V.).

Related Letters:

Lung Transplantation and Survival in Children with Cystic Fibrosis
Dawwas M. F., Watson C. J., Gimson A. E., Anbar R. D., Sweet S. C., Benden C., Elidemir O., Liou T. G., Adler F. R., Cox D. R.
Extract | Full Text | PDF  
N Engl J Med 2008; 358:1753-1755, Apr 17, 2008. Correspondence

This article has been cited by other articles:

• Dawwas, M. F., Watson, C. J., Gimson, A. E., Anbar, R. D., Sweet, S. C., Benden, C., Elidemir, O., Liou, T. G., Adler, F. R., Cox, D. R. (2008). Lung Transplantation and Survival in Children with Cystic Fibrosis. NEJM 358: 1753-1755 [Full Text]