Cystic fibrosis, pathophysiological and clinical aspects.

摘要: 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 genetic cellular abnormalities rapidly progressing, but therapy still symptomatic based on insufficiently controlled short-term studies. At present therapeutic approach aims combat respiratory infections by optimal antibiotic therapy, combined with techniques promote sputum evacuation. Additional measures attempt optimise both nutritional state physical condition. Median survival has imporved from approximately 1 year about 25 years during past 3 decades. This article summarises information disease mechanisms treatment.