ABSTRACT
By the example of 2 adult patients with cystic fibrosis (CF) problems of pulmonary manifestation of CF and its complications are discussed. In both patients disease manifested late. Life expectancy of CF-patients has markedly increased over the last decades. In the adult distinct complications appear more often such as exacerbation of bronchitis due to pseudomonas aeruginosa, colonisation of air ways by Burkholderia cepacia or by atypical mycobacteria, development of allergic bronchopulmonary aspergillosis, bronchiectases and spontaneous pneumothorax. Today, even in adults with chronic bronchitis of unknown origin the possibility of CF has to be considered. Optimized treatment under clinical conditions, in combination with a physical training program and a diet rich in calories will ameliorate subjective physical performance as well as objective pulmonary function and work capacity. Regular rehabilitation programs contribute to better compliance. In patients with CF direct transition from pediatric to pneumologic/internistic treatment for adults is mandatory in order to optimize expectancy and quality of life.
