Cystic fibrosis (CF) is a monogenetic disorder that presents as a multisystem disease. The first signs and symptoms typically occur in childhood, but nearly 3 percent of patients are diagnosed as adults. Due to improvements in therapy, more than 25 percent of patients reach adulthood and more than 9 percent live past the age of 30.
The disease is characterized by
chronic airways infection that ultimately leads to
bronchiectasis and bronchiolectasis,
exocrine pancreatic insufficiency,
abnormal sweat gland function, and urogenital dysfunction.