The DOI matches its Crossref record; 147 of the 147 references it cites were found in a registry or an index.
Managing Cystic Fibrosis: Strategies That Increase Life Expectancy and Improve Quality of Life
Abstract
The survival of patients with cystic fibrosis (CF) continues to improve. The discovery and cloning of the CFTR gene more than 21 years ago led to the identification of the structure and function of the CFTR chloride channel. New therapies based on the understanding of the function of CFTR are currently under development. The better clinical status and improved survival of patients with CF is not only a result of understanding of the molecular mechanisms of CF but also a result of the development of therapeutic strategies that are based on insights into the natural course of the disease. Current CF treatments that target respiratory infections, inflammation, mucociliary clearance, and nutritional status are associated with improved pulmonary function and reduced exacerbations. Patients benefit from treatment at a specialized CF center by a multidisciplinary dedicated team with emphasis being placed on frequent visits, periodic testing, and monitoring adherence to therapy. The purpose of this review is to survey recent developments in CF care that are responsible for the improved survival and quality of life of patients with CF.
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Cites
Identification of the Cystic Fibrosis Gene: Cloning and Characterization of Complementary DNA
Riordan, J. R., Rommens, J. M., Kerem, B.-S., Alon, N., Rozmahel, R., Grzelczak, Z., Zielenski, J., Lok, S., Plavsic, N., Chou, J.-L., et al. (1989) · Science · doi:10.1126/science.2475911 · Cited by 7,418
Identification of the Cystic Fibrosis Gene: Genetic Analysis
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Identification of the Cystic Fibrosis Gene: Chromosome Walking and Jumping
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Intermittent Administration of Inhaled Tobramycin in Patients with Cystic Fibrosis
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CYSTIC FIBROSIS OF THE PANCREAS AND ITS RELATION TO CELIAC DISEASE: A CLINICAL AND PATHOLOGIC STUDY
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Cystic Fibrosis Pulmonary Guidelines: Chronic Medications for Maintenance of Lung Health
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Self-citation
Cited by
Elborn, J. S. (2016) · The Lancet · doi:10.1016/s0140-6736(16)00576-6 · Cited by 1,805
Yen, E. H., Quinton, H., & Borowitz, D. (2013) · The Journal of Pediatrics · doi:10.1016/j.jpeds.2012.08.040 · Cited by 306
Cystic Fibrosis Lung Disease: An Overview
Turcios, N. L. (2020) · Respiratory Care · doi:10.4187/respcare.06697 · Cited by 217
Prognostic value of six minute walk test in cystic fibrosis adults
Martin, C., Chapron, J., Hubert, D., Kanaan, R., Honoré, I., Paillasseur, J.-L., Aubourg, F., Dinh-Xuan, A.-T., Dusser, D., Fajac, I., et al. (2013) · Respiratory Medicine · doi:10.1016/j.rmed.2013.10.001 · Cited by 63
“Structural abnormalities characterized by airway remodeling and mucus plugging lead to progressive airflow limitation [1], often resulting in respiratory failure and in the need for lung transplant [2].”
BackgroundHealth-related quality of life in cystic fibrosis
Royce, F. H., & Carl, J. C. (2011) · Current Opinion in Pediatrics · doi:10.1097/mop.0b013e32834a7829 · Cited by 27
Metadata
- Authors
- Cohen-Cymberknoh, M., Shoseyov, D., & Kerem, E.
- Venue
- American Journal of Respiratory and Critical Care Medicine
- Publisher
- Oxford University Press (OUP)
- Published
- 2011-06-01
- Volume
- 183
- Issue
- 11
- Pages
- 1463-1471
- ISSN
- 1073-449X (ISSN-L)
- 1535-4970
- OpenAlex
- W2134224630
- Cited by
- 287
- References
- 147
- FWCI
- 18.06
- Type
- journal article
- Language
- en
- Open access
- closed
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Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
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