Paranjape, Shruti M. Eudowood Division of Pediatric Respiratory Sciences and Johns Hopkins Cystic Fibrosis Center, Johns Hopkins Medical Institutions, Baltimore, Maryland.
Last reviewed:May 2018
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A hereditary genetic disorder, resulting from mutations in the cystic fibrosis transmembrane conductance regulator gene, that severely damages the lungs, digestive system, pancreas, and liver. Cystic fibrosis is one of the most common life-shortening genetic diseases. First described in 1938, cystic fibrosis primarily affects the lungs (Fig. 1), as well as other organs and organ systems, particularly the pancreas, liver, and gastrointestinal system. For individuals with cystic fibrosis, the predicted median survival is 47.7 years, and this is related to early diagnosis and the use of therapies that treat chronic respiratory infection, optimize nutrition, and improve quality of life. Although there is presently no cure for cystic fibrosis, therapies that target the basic genetic defect hold significant promise for continued improvements in overall health and survival. See also: Digestive system; Gastrointestinal tract disorders; Genetics; Liver; Lung; Pancreas; Pancreas disorders; Pathology; Respiratory system disorders
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