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.