ABSTRACT
Cystic fibrosis (CF), the most common lethal autosomal-recessive disease in Caucasians, is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene on the long arm of chromosome 7. Clinical disease results from mutations in CFTR that lead to defective transmembrane conductance of chloride ions and, thereby, to impaired transport of water. As a consequence, the viscosity of all exocrine fluids increases substantially, principally in the lungs and in the gastrointestinal tract, but also in the upper-respiratory tract, including the paranasal sinuses. Therapeutic advances over the past 35 years have enhanced the survival of patients with CF from a mean of 4 years in 1960 to a mean of 30 years in 1991, according to the CF Foundation patient registry. This chapter reviews the clinical manifestations of cystic fibrosis in the upper and lower airways, focusing on the interaction of paranasal sinus disease on lower-respiratory tract symptoms.
