Course of cystic fibrosis in 95 patients.

The course of 95 patients with cystic fibrosis is presented. Survivors have 
a mean follow-up period of over 14 years (minimum: 13 years). 
Of 45 patients diagnosed prior to extensive irreversible pulmonary involvement, only 
one has died and none is disabled. In contrast, of the 
other 50 patients diagnosed after substantial irreversible pulmonary disease was present, 
26 have died. Mortality and morbidity has been greater in females. 
Possible factors contributing to the improving prognosis include early diagnosis, aggressive 
management with comprehensive care, easy access to specialized care, and improved 
antimicrobial therapy. 