[Mucoviscidosis--cystic fibrosis. Diagnosis--therapy--prognosis]

Abstract

In 1989, the sequence of the cystic fibrosis gen (CFTR) was analyzed. Since that time, prenatal diagnosis as well as genetic counseling is possible in all CF-families. During the last decades, the prognosis of CF-patients is still increasing. In 1943, 33.5% of 2447 patients undergoing regular care in 53 CF-centers in Germany were adults. The prognosis of CF-patients depends upon early diagnosis and regular care in a specialized CF-center. Intensive physiotherapy, optimal nutrition and aggressive antibiotic treatment are the most important factors for the increased life expectancy. Today, the majority of medical care for CF-children and young adults including antibiotic therapy is organized on outpatient basis. Only severe pulmonary exacerbations or specific complications of cystic fibrosis need clinical treatment. One to the increased life expectancy, the development of experienced centers for adult patients is extremely important. Some of these centers should provide the possibility of lung transplantation for terminal ill CF-patients in cooperation with thoracic surgeons.