Joseph M. Pilewski, MD; Darren B. Taichman, MD, PhD, Executive Deputy Editor
Disclosures: Disclosures can be viewed at www.acponline.org/authors/icmje/ConflictOfInterestForms.do?msNum=M14-1534.
Requests for Single Reprints: Joseph M. Pilewski, MD, University of Pittsburgh, Department of Medicine, PACCM Division, NW 628 MUH, 3459 Fifth Avenue, Pittsburgh, PA 15213.
Current Author Addresses: Dr. Pilewski: University of Pittsburgh, Department of Medicine, PACCM Division, NW 628 MUH, 3459 Fifth Avenue, Pittsburgh, PA 15213.
Dr. Taichman: American College of Physicians, 190 N. Independence Mall West, Philadelphia, PA 19106.
Pilewski J., Taichman D.; Cystic Fibrosis: Recent Successes Present New Challenges. Ann Intern Med. 2014;161:298-299. doi: 10.7326/M14-1534
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Published: Ann Intern Med. 2014;161(4):298-299.
Remarkable progress has been made in the care and survival of patients with cystic fibrosis (CF). First described in 1938 as a life-shortening disease characterized by cystic changes of the pancreas, CF is now known to be caused by any of many known mutations in a single gene, resulting in abnormal function of a chloride channel and multisystem disease (1). Before the development of pancreatic enzymes and nutritional supplements, most patients with CF died early from malnutrition related to pancreatic insufficiency. After this early threat became more controllable, progressive pulmonary disease characterized by chronic airway infection, inflammation, bronchiectasis, and respiratory failure emerged as the predominant cause of morbidity and mortality. Today, with supportive therapies targeting mucus obstruction and infections, most patients survive to adulthood and CF is no longer strictly a pediatric disease.
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Gastroenterology/Hepatology, Pulmonary/Critical Care, Pancreatic Disease.
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Print ISSN: 0003-4819 | Online ISSN: 1539-3704
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