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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Keywords Cystic fibrosis, Gastro-intestinal aspects, Hepatic aspects, Pathophysiology, Respiratory aspects
Persistent URL dx.doi.org/10.1007/BF01957271, hdl.handle.net/1765/73834
Journal European Journal of Pediatrics
Neijens, H.J, Sinaasappel, M, de Groot, R, de Jongste, J.C, & Overbeek, S.E. (1990). Cystic fibrosis, pathophysiological and clinical aspects. European Journal of Pediatrics (Vol. 149, pp. 742–751). doi:10.1007/BF01957271