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Seeing is believing: imaging early lung disease in cystic fibrosis

Stamatia Alexiou1, Ronald C Rubenstein

  • 11 Division of Pulmonary Medicine and Cystic Fibrosis Center The Children's Hospital of Philadelphia Philadelphia, Pennsylvania and Department of Pediatrics Perelman School of Medicine at the University of Pennsylvania Philadelphia, Pennsylvania.

American Journal of Respiratory and Critical Care Medicine
|April 17, 2014
PubMed
Abstract

No abstract available in PubMed .

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Cystic fibrosis (CF), an autosomal recessive disorder, significantly affects the function of exocrine glands. This genetically inherited disease is characterized by the production of thick and sticky mucus, which can severely affect various organs and systems in the body.
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Cystic fibrosis (CF) is an autosomal recessive disorder that predominantly affects individuals of Northern European descent, occurring at a rate of 1 in 3500. It is caused by a genetic mutation in a gene on chromosome 7, most commonly the ΔF508 mutation, that codes for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. This results in thicker mucus secretions and obstruction pathologies in multiple organs, including the lungs and sinuses.
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