ABSTRACT
I. Introduction 581
II. CF Genetics 582
III. CF Pathophysiology 582
IV. Predicting Natural History in CF 583
V. Radiography 584
A. Conventional Chest Radiography 584
B. Computed Tomography 585
C. Magnetic Resonance Imaging 589
VI. Conclusions 589
References 590
I. Introduction
Cystic fibrosis (CF) is the most common lethal genetic disease among
Caucasians, with an estimated incidence of 1:2500 live births in the United
States. Formally described in the 1930s, CF causes a spectrum of disease charac-
terized by rhinosinusitis, pancreatic insufficiency, malabsorption, and bron-
chiectasis (1). Presently, there are over 33,000 patients with CF in the United
States, and it is estimated that approximately 1:25 Caucasians are heterozygous
carriers of the autosomal recessive disease.