COURSE OF CYSTIC-FIBROSIS IN 95 PATIENTS

The course of 95 patients with cystic fibrosis is presented. Survivors had a mean follow-up period of over 14 yr (minimum: 13 yr). Of 45 patients diagnosed prior to extensive irreversible pulmonary involvement, only 1 died and none were disabled. In contrast, of the other 50 patients diagnosed after substantial irreversible pulmonary disease was present, 26 died. Mortality and morbidty was greater in females. Possible factors contributing to the improving prognosis included early diagnosis, aggressive management with comprehensive care, easy access to specialized care and improved antimicrobial therapy.