[Cystic fibrosis in the Western health region].

78 patients with cystic fibrosis have been diagnosed in western Norway (Health Region 3) since 1960. 31 were born before 1971 (40%). 44 patients were born in the county of Hordaland (62%), including the city of Bergen where the western Cystic Fibrosis Centre is located. 24 (31%) patients were born in Rogaland, and five (7%) in Sogn & Fjordane. 12 patients have died, 9 of them born before 1971 (survival in this cohort of patients is 71%). During two decades from 1971 to 1990, 3 out of 33 patients have died (survival 91%). All patients born after 1990 are alive. The median age at death was 18.5 years. Among patients alive in 1999, 32 (41.5%) are more than 20 years and 19 (24.7%) are more than 30 years old. Median age among all patients is 19.5 years (range 3-51). Chronic lung infection with Pseudomonas aeruginosa has been diagnosed in 20.8% of all patients, which is lower than the average of Norwegian cystic fibrosis patients (35%). Survival among cystic fibrosis patients in Western Norway is good compared to data from other countries. It seems realistic to expect that our patients should achieve a median survival to more than 30 years of age.