| Abstract: | In this study the clinical data and treatment concept in 197 patients with cystic fibrosis, seen during the past 20 years at the University Children's Hospital of Berne (Switzerland), are presented. The results showed that during recent years the median age of survival, the mean age at death and the cumulative survival rates have improved. Furthermore, this study demonstrates that by forming groups of patients with similar age at diagnosis the cumulative survival rate declined in the group with early diagnosis much more markedly than in the group of patients with later diagnosis. Further evaluation of these data revealed that most patients of the former group had a more severe form of cystic fibrosis than the latter group. Since there are different patterns of severity in this disease, it will be necessary to develop staging for the purpose of statistical comparisons. |
