The utilization of diagnostic and therapeutic programs has considerably improved survival of patients with cystic fibrosis (FC). Nevertheless, we still do not know at all which factors really affect survival in FC. The frequent identification of patients with mild expression of the disease has further contributed to improved survival rates, but the great variability of clinical forms in FC makes prognostic evaluation of individuals very difficult. From 1978 through 1987, 155 patients with FC were admitted to our Department for a therapeutic program. Ten years later, the effect on survival of some factors such as sex, age at diagnosis, age of presentation of symptoms, diagnostic delay, symptoms at diagnosis was evaluated. Survival analysis was performed by the Kaplan-Meyer method and by the LeeDesu non parametric statistic test and by the Logrank test. In our survey, survival rate is 77.2% at the ages of 10 years (Tables I, II). Age at diagnosis less than 1 year and the presence of gastrointestinal symptoms at the first visit are the factors associated with a lower survival rate in our patients (Fig. 1, 5). The diagnostic delay shows a more complex result. In the group of patients whose diagnosis was delayed within 16 months since the presentation of symptoms, a delay longer than 4 months was associated with a poorer survival rate; when the delay was longer than 16 months, other factors probably contribute to prolong the survival. The small number of patients and the short mean follow-up period do not permit to evaluate the effects of the considered variables on the outcome of our patients. Nevertheless, the results of our study have given to us precious informations for the organization of the management of FC patients in our Department. We have developed programs in order to emphasize the physicians' awareness towards cystic fibrosis and in order to start a prompt and comprehensive treatment of the disease as early as possible.
