1990-08-01
Cystic fibrosis, pathophysiological and clinical aspects
Publication
Publication
European Journal of Pediatrics , Volume 149 - Issue 11 p. 742- 751
Abstract
Cystic fibrosis is a lethal, hereditary, until recently little understood disease, which leads to progressive functional disturbances in various organs, including the lungs, liver and pancreas. Knowledge of the genetic and cellular abnormalities is rapidly progressing, but therapy is still symptomatic and based on insufficiently controlled and short-term studies. At present the therapeutic approach aims to combat respiratory infections by optimal antibiotic therapy, combined with techniques to promote sputum evacuation. Additional measures attempt to optimise both nutritional state and physical condition. Median survival has imporved from approximately 1 year to about 25 years during the past 3 decades. This article summarises present information on disease mechanisms and treatment.
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doi.org/10.1007/BF01957271, hdl.handle.net/1765/73834 | |
European Journal of Pediatrics | |
Organisation | Department of Pediatrics |
Neijens, H., Sinaasappel, M., de Groot, R., de Jongste, J., & Overbeek, S. (1990). Cystic fibrosis, pathophysiological and clinical aspects. European Journal of Pediatrics (Vol. 149, pp. 742–751). doi:10.1007/BF01957271 |