Cystic fibrosis: clinical aspects

The disease primarily affects the bronchial system
and the gastrointestinal tract. Viscous
mucus formation leading to frequent, recurrent
bronchopulmonic infections and eventually
chronic oxygen deficiency characterize the
common, severe form of the disease. The average
life expectancy in typical CF is about 30
years. The diseasemay take a less severe, almost
mild course. Congenital bilateral absence of the
vas deferens (CBAVD) occurs in 95% of patients
with CF. It may be the only manifestation in individuals
with different mutant allelic combinations
at the CF locus.